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PubMed - Adenoid Cystic Carcinoma (100 unread)

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Presentation and Outcomes of Non-Squamous Cell Carcinoma Sinonasal Malignancies: A National Perspective

Fetched: June 18th, 2021, 5:00am GMT by Zaid Al-Qurayshi

Ann Otol Rhinol Laryngol. 2021 Jun 17:34894211024783. doi: 10.1177/00034894211024783. Online ahead of print.

ABSTRACT

BACKGROUND: Non-squamous cell carcinoma sinonasal malignancies (NSCCSM) are relatively rare. Neoadjuvant radiotherapy and/or chemotherapy (NTx) have been proposed to improve outcomes compared to surgery alone. In this study, we aim to examine the prevalence of NTx utilization and associated outcomes.

METHODS: A retrospective study utilizing the National Cancer Database, 2004 to 2015. The study population included adult patients diagnosed with primary NSCCSM.

RESULTS: A total of 574 patients were included. The mean age of the study population was 61.7 ± 16.5 years. The median follow-up time was 40.4 months (interquartile range: 15.3-81.3 months). The histopathological diagnoses identified included: (i) 37.0% adenocarcinoma, (ii) 22.8% adenoid cystic carcinoma, (iii) 20.0% mucosal melanoma, (iv) 11.9% esthesioneuroblastoma, and (v) 8.2% sinonasal undifferentiated carcinoma (SNUC). NTx was utilized in 70 (12.20%) of the study population. Patients who received NTx were more likely to have SNUC or esthesioneuroblastoma (P < .01 each) and to have stage III or IV disease (P < .01 each). NTx was most likely to be administrated in a high-volume center [OR: 3.94, 95%CI: (1.47, 10.53), P = .006]. Patients who received NTx had a significantly lower prevalence of positive margin postoperatively [OR: 0.48, 95%CI: (0.26, 0.87), P = .016]. In patients with NSCCSM, negative margin was associated with improved overall survival [HR: 0.55, 95%CI: (0.36, 0.82), P = .004].

CONCLUSIONS: This study provides an epidemiological perspective regarding NSCCSM and related practice patterns and survival outcomes. Neoadjuvant radiotherapy and/or chemotherapy is likely to decrease the risk of positive margin which ultimately could improve survival in this population.

PMID:34137285 | DOI:10.1177/00034894211024783

Identification and validation of selective deubiquitinase inhibitors

Fetched: June 17th, 2021, 5:00am GMT by Anthony C Varca

Cell Chem Biol. 2021 Jun 2:S2451-9456(21)00257-9. doi: 10.1016/j.chembiol.2021.05.012. Online ahead of print.

ABSTRACT

Deubiquitinating enzymes (DUBs) are a class of isopeptidases that regulate ubiquitin dynamics through catalytic cleavage of ubiquitin from protein substrates and ubiquitin precursors. Despite growing interest in DUB biological function and potential as therapeutic targets, few selective small-molecule inhibitors and no approved drugs currently exist. To identify chemical scaffolds targeting specific DUBs and establish a broader framework for future inhibitor development across the gene family, we performed high-throughput screening of a chemically diverse small-molecule library against eight different DUBs, spanning three well-characterized DUB families. Promising hit compounds were validated in a series of counter-screens and orthogonal assays, as well as further assessed for selectivity across expanded panels of DUBs. Through these efforts, we have identified multiple highly selective DUB inhibitors and developed a roadmap for rapidly identifying and validating selective inhibitors of related enzymes.

PMID:34129829 | DOI:10.1016/j.chembiol.2021.05.012

Permalink for 'Cervical carcinoma with histologic components of sarcomatoid carcinoma and multiple basaloid variants: A case report and review of the literature'

Cervical carcinoma with histologic components of sarcomatoid carcinoma and multiple basaloid variants: A case report and review of the literature

Fetched: June 15th, 2021, 5:00am GMT by Paulina J Haight

Gynecol Oncol Rep. 2021 May 27;37:100797. doi: 10.1016/j.gore.2021.100797. eCollection 2021 Aug.

ABSTRACT

Cervical cancer with co-existing pathologic components of squamous cell carcinoma, basaloid morphology and sarcomatoid carcinoma is rare, with limited reports in the literature. Here we present a patient who underwent a modified radical hysterectomy for cervical cancer, with final pathology specimen demonstrating multiple histologic variants including basal carcinoma, adenoid cystic-like areas, basaloid squamous cell carcinoma and areas of high-grade transformation to sarcomatoid carcinoma.

PMID:34124329 | PMC:PMC8173268 | DOI:10.1016/j.gore.2021.100797

Notch inhibitors induce diarrhea, hypercrinia and secretory cell metaplasia in the human colon

Fetched: June 15th, 2021, 5:00am GMT by Michael Collins

EXCLI J. 2021 Apr 26;20:819-827. doi: 10.17179/excli2021-3572. eCollection 2021.

ABSTRACT

In humans, inhibition of Notch oncogenic signaling leads to tumor regression. Preclinical studies indicate that Notch signaling contributes to the maintenance of intestinal homeostasis. Here, we sought to describe the intestinal effects of a first-in-human Notch inhibitor in an indication of refractory cancer. Between 2014 and 2017, adult patients treated for refractory cancer with the novel Notch inhibitor LY3039478 and who had grade ≥ 2 diarrhea were referred to the gastroenterology department of a tertiary hospital in the Paris region of France. Eleven patients (median (range) age: 72 (29-83)) were included in the study. All patients had advanced cancer: adenoid cystic carcinoma (n=3, 27 %), sarcoma (n=3, 27 %), and other types (n=5, 46 %). In all cases, digestive tract endoscopy revealed abundant mucus in the intestinal lumen, and digestive tract biopsies showed an abnormally low proportion of enterocytes and marked elevation of the proportion of pseudostratified goblet cells. Microscopic inflammation was seen in colon biopsies from 2 of the 11 patients (18 %). The clinical, endoscopic and histological abnormalities were dependent on the dose of Notch inhibitor. All patients resolved their digestive signs or symptoms after discontinuing the dose and the median (range) time interval between discontinuation of the Notch inhibitor and resolution of all the gastrointestinal signs and symptoms was 7 days (4-24). Likewise, the median time interval between discontinuation and resolution of the histological abnormalities was 7 days (1-10). Blocking Notch signaling induces secretory cell metaplasia of the intestinal epithelium, which in turn leads to transient diarrhea. Our results confirm the role of Notch signaling in intestinal homeostasis in humans.

PMID:34121974 | PMC:PMC8192874 | DOI:10.17179/excli2021-3572

CXCR5 induces perineural invasion of salivary adenoid cystic carcinoma by inhibiting microRNA-187

Fetched: June 12th, 2021, 5:00am GMT by Mei Zhang

Aging (Albany NY). 2021 Jun 10;13. doi: 10.18632/aging.203097. Online ahead of print.

ABSTRACT

CXCR5 played critical roles in tumorigenesis and metastasis. Nevertheless, little was known about the involvement of CXCR5 in perineural invasion (PNI) of salivary adenoid cystic carcinoma (SACC). Here, we confirmed upregulation of CXCR5 in SACC specimens and cells and identified that CXCR5 exhibited a significant positive correlation with PNI. Functionally, knockdown of CXCR5 suppressed SACC cells migration, invasion and PNI ability, whereas CXCR5 overexpression displayed the opposite effects. Moreover, CXCR5 downregulated microRNA (miR)-187, which could competitively sponge S100A4. The PNI-inhibitory effect of CXCR5 knockdown or miR-187 overexpression could be reversed by elevated expression of S100A4. Conjointly, our data revealed that CXCR5 facilitated PNI through downregulating miR-187 to disinhibit S100A4 expression in SACC.

PMID:34114971 | DOI:10.18632/aging.203097

Whole-Genome Sequencing of Common Salivary Gland Carcinomas: Subtype-Restricted and Shared Genetic Alterations

Fetched: June 12th, 2021, 5:00am GMT by Tatiana V Karpinets

Clin Cancer Res. 2021 May 19. doi: 10.1158/1078-0432.CCR-20-4071. Online ahead of print.

ABSTRACT

PURPOSE: Salivary gland carcinomas (SGCs) are pathologically classified into several widely diverse subtypes, of which adenoid cystic carcinoma (ACC), mucoepidermoid carcinoma (MEC), and salivary duct carcinoma (SDC) are the most commonly encountered. A comparative genetic analysis of these subtypes provides detailed information on the genetic alterations that are associated with their tumorigenesis and may lead to the identification of biomarkers to guide tumor-specific clinical trials.

EXPERIMENTAL DESIGN: Whole-genome sequencing of 58 common SGCs (20 ACCs, 20 SDCs, and 18 MECs) was performed to catalog structural variations, copy number, rearrangements, and driver mutations. Data were bioinformatically analyzed and correlated with clinicopathologic parameters, and selected targets were validated.

RESULTS: Novel and recurrent type-specific and shared genetic alterations were identified within and among 3 subtypes. Mutually exclusive canonical fusion and nonfusion genomic alterations were identified in both ACC and MEC. In ACCs, loss of chromosome 12q was dominant in MYB or MYBL1 fusion-positive tumors and mutations of NOTCH pathway were more common in these fusion negatives. In MECs, CRTC1-MAML2 fusion-positive tumors showed frequent BAP1 mutation, and tumors lacking this fusion were enriched with LRFN1 mutation. SDCs displayed considerable genetic instability, lacked recurrent chromosomal rearrangements, and demonstrated nonoverlapping TP53 mutation and ERBB2 amplification in a subset of tumors. Limited genetic alterations, including focal amplifications of 8q21-q23, were shared by all subtypes and were associated with poor survival.

CONCLUSIONS: This study delineates type-specific and shared genetic alterations that are associated with early phenotypic commitment and the biologic progression of common SGCs. These alterations, upon validation, could serve as biomarkers in tumor-specific clinical trials.

PMID:34011559 | DOI:10.1158/1078-0432.CCR-20-4071

Permalink for 'Intraorbital and intracranial extension of adenoid cystic carcinoma without clinical or radiological lacrimal gland involvement'

Intraorbital and intracranial extension of adenoid cystic carcinoma without clinical or radiological lacrimal gland involvement

Fetched: June 11th, 2021, 5:00am GMT by Carmelo Macri

Orbit. 2021 Jun 9:1-5. doi: 10.1080/01676830.2021.1939731. Online ahead of print.

ABSTRACT

Adenoid cystic carcinoma (AdCC) is a rare epithelial neoplasm of the head and neck, most commonly found in the salivary glands. Orbital AdCC is an uncommon clinical entity arising from the lacrimal glands, however primary orbital AdCC has been previously described in a small number of case reports. The exact origin of the neoplasm with uninvolved lacrimal gland in the orbit is unknown, however it may arise from ectopic lacrimal or salivary gland tissue, or extension from nearby epithelial structures. We describe the clinical characteristics, investigations and management of a 55-year-old man presenting with vertical diplopia, found to have left posterior orbital AdCC invading the skull base with intracranial extension involving the inferotemporal fossa, pterygopalatine fossa, left carotid artery, cavernous sinus and temporal lobe dura, without clinical or radiological lacrimal gland involvement or systemic metastases.

PMID:34107855 | DOI:10.1080/01676830.2021.1939731

A Combined Neutron and Proton Regimen for Advanced Salivary Tumors: Early Clinical Experience

Fetched: June 10th, 2021, 5:00am GMT by Saif Aljabab

Cureus. 2021 May 4;13(5):e14844. doi: 10.7759/cureus.14844.

ABSTRACT

Background and objective Fast neutron radiotherapy (NRT) is a high linear energy transfer (LET) particle therapy that offers a local control (LC) advantage over low-LET X-rays in the treatment of advanced and unresectable salivary gland malignancies. However, in tumors approximating the base of skull (BOS), target volumes may be underdosed to minimize toxicity to the central nervous system (CNS). In this setting, a proton beam boost to the underdosed part of the tumor may improve LC. We report our early experience with a hybrid neutron-proton approach in patients with BOS involvement. Materials and methods We retrospectively reviewed 29 patients with locally advanced and unresectable salivary gland tumors involving the BOS between 2014-2018. The median age of the patients was 56 years, with the majority of them having adenoid cystic carcinomas (ACC) (79%) with advanced T4a/b disease (86%), pathologic perineural invasion (PNI) (55.2%), and orbital invasion (34.5%). Five patients (17.2%) were cases of re-irradiation. Surgical resection was attempted in 15 patients (51.7%), of which none achieved negative margins. The median neutron dose was 18.4 neutron Gray (nGy) with a sequential proton boost (PB) with a median dose of 25 Gy [relative biological effectiveness (RBE)] (range: 16-45 Gy). Toxicity was graded per the Common Terminology Criteria for Adverse Events (CTCAE) version 4.03. Descriptive statistics and the Kaplan-Meier method were used. Results At a median follow-up of 18.9 months [interquartile range (IQR): 6.1-32.5], the entire cohort's overall survival (OS) was 93.1%, progression-free survival (PFS) was 79.3%, and LC was 89.7%. Among patients who were not re-irradiated (n=24), the most commonly recorded acute grade 3 toxicities were mucositis (50%) and dermatitis (37.5%). There was no documented acute grade 4/5 events. Late grade 3/4 events included trismus (n=1), hearing loss (n=2), visual loss (n=6), and bone necrosis (n=1). There were no reported late grade 5 events in de novo patients. Conclusion In this challenging cohort with a poor prognosis, early outcomes for a hybrid neutron-proton approach were found to be promising. Further studies involving longer follow-ups with a larger cohort of patients are required to validate our findings.

PMID:34104589 | PMC:PMC8175057 | DOI:10.7759/cureus.14844

Surgical treatment of liver and kidney metastasis from an adenoid cystic carcinoma of the submandibular gland: case report

Fetched: June 8th, 2021, 5:00am GMT by Ferrari Cecilia

Tumori. 2021 Jun 7:3008916211021664. doi: 10.1177/03008916211021664. Online ahead of print.

ABSTRACT

INTRODUCTION: Adenoid cystic carcinomas (ACCs) account for 10% of all malignant salivary tumours. They are slow-growing but locally aggressive. Reports of liver and renal metastases are rare.

CASE DESCRIPTION: A 58-year-old woman who had undergone resection of a left submandibular ACC in 1995 was referred to our centre for follow-up in 2018. A computed tomography scan revealed two lesions: one on segment six of the liver and the other on a kidney. A hepatic wedge resection and right nephrectomy were performed. The postoperative course was uneventful. At 2-year follow-up, the patient was alive and well with no evidence of disease.

CONCLUSION: Management of ACC is a therapeutic challenge because of its tendency for distant metastases. The literature recommends regular follow-up imaging and radical surgical treatment but specific guidelines for the approach to recurrence are lacking.

PMID:34097534 | DOI:10.1177/03008916211021664

A phase II trial of all-trans retinoic acid (ATRA) in advanced adenoid cystic carcinoma

Fetched: June 7th, 2021, 5:00am GMT by Glenn J Hanna

Oral Oncol. 2021 Jun 3;119:105366. doi: 10.1016/j.oraloncology.2021.105366. Online ahead of print.

ABSTRACT

BACKGROUND: Effective therapies are lacking for recurrent, metastatic adenoid cystic carcinoma (R/M ACC) and preclinical models suggest retinoic acid agonists inhibit ACC growth. This phase II trial evaluated all-trans retinoic acid (ATRA) as a novel therapy for ACC.

METHODS: Patients with R/M ACC (any site) with clinical and/or radiographic progression ≤12 months prior to study entry were eligible. Cohort 1 (CH1) received ATRA 45 mg/m² split oral daily dosing on days 1-14 of a 28-day cycle; Cohort 2 (CH2) received the same dosing continuously. Primary endpoint was best overall response rate (CR + PR) (RECIST v1.1). Secondary endpoints: safety and progression-free survival (PFS). Exploratory analyses: ATRA impact on MYB expression and genomic predictors of response.

RESULTS: Eighteen patients enrolled. There were no responses, but 61% (11/18) had stable disease (SD) and 28% (5/18) progression as best response; 11% (2/18) unevaluable. Median duration of stability: 3.7 months (95%CI, 1.9-3.9). One patient (CH1) remains on drug with SD approaching 1 year. Half of those who received prior VEGFR therapy achieved SD (4/8). At median follow up of 7.9 months, median PFS was 3.2 months (95%CI, 1.8-3.9). N = 1 required dose adjustment; N = 1 came off drug for toxicity. There were no grade 3-4 adverse events. NOTCH1 and PI3K pathway alterations were most frequent. Low MYB protein expression was associated with longer duration of stability on ATRA (P < 0.01).

CONCLUSION(S): While the trial did not meet its prespecified response endpoint, ATRA alone or in combination may be a low toxicity treatment for disease growth stabilization in R/M ACC.

PMID:34091189 | DOI:10.1016/j.oraloncology.2021.105366

Zebrafish, an In Vivo Platform to Screen Drugs and Proteins for Biomedical Use

Fetched: June 3rd, 2021, 5:00am GMT by Hung-Chieh Lee

Pharmaceuticals (Basel). 2021 May 24;14(6):500. doi: 10.3390/ph14060500.

ABSTRACT

The nearly simultaneous convergence of human genetics and advanced molecular technologies has led to an improved understanding of human diseases. At the same time, the demand for drug screening and gene function identification has also increased, albeit time- and labor-intensive. However, bridging the gap between in vitro evidence from cell lines and in vivo evidence, the lower vertebrate zebrafish possesses many advantages over higher vertebrates, such as low maintenance, high fecundity, light-induced spawning, transparent embryos, short generation interval, rapid embryonic development, fully sequenced genome, and some phenotypes similar to human diseases. Such merits have popularized the zebrafish as a model system for biomedical and pharmaceutical studies, including drug screening. Here, we reviewed the various ways in which zebrafish serve as an in vivo platform to perform drug and protein screening in the fields of rare human diseases, social behavior and cancer studies. Since zebrafish mutations faithfully phenocopy many human disorders, many compounds identified from zebrafish screening systems have advanced to early clinical trials, such as those for Adenoid cystic carcinoma, Dravet syndrome and Diamond-Blackfan anemia. We also reviewed and described how zebrafish are used to carry out environmental pollutant detection and assessment of nanoparticle biosafety and QT prolongation.

PMID:34073947 | DOI:10.3390/ph14060500

Antizyme inhibitor 2 (AZIN2) associates with better prognosis of head and neck minor salivary gland adenoid cystic carcinoma

Fetched: May 29th, 2021, 5:00am GMT by Hanna Hämetoja

APMIS. 2021 May 28. doi: 10.1111/apm.13158. Online ahead of print.

ABSTRACT

AIM: The key regulator of the polyamine biosynthetic pathway is ornithine decarboxylase (ODC). ODC is activated by antizyme inhibitor 1 (AZIN1) and 2 (AZIN2). AZIN1 and recently AZIN2 have been related to cancer, however, their functions in adenoid cystic carcinoma (ACC) have not been studied. We evaluated the immunoexpression of AZIN1 and 2 in tumor tissue from ACC patients, and related the results to clinical and clinicopathological features and patient survival.

STUDY DESIGN: We performed immunohistochemical study on minor salivary and mucous gland ACC tissue samples of patients treated at the Helsinki University Hospital (Helsinki, Finland) during 1974-2012. We scored AZIN1 and 2 immunoexpression in 42 and 45 tumor tissue samples, respectively, and correlated them with clinicopathological factors and survival.

RESULTS: Enhanced AZIN2 expression was associated with better survival. In addition, both AZINs were seen more commonly in cribriform and tubular than in solid growth patterns. AZIN1 expression did not correlate with the studied clinicopathological factors.

CONCLUSIONS: It seems that AZIN2 expression is higher in cancer tissue with secretory functions. In ACC tissue, high AZIN2 expression could be related to well-differentiated histological type which still has a functioning vesicle transportation system. Thus, AZIN2 could be a prognostic factor for better survival of ACC patients.

PMID:34046926 | DOI:10.1111/apm.13158

Thyroid metastasis from adenoid cystic carcinoma of the lung: a case report and literature review

Fetched: May 28th, 2021, 5:00am GMT by Giulio Montecamozzo

J Surg Case Rep. 2021 May 20;2021(5):rjab178. doi: 10.1093/jscr/rjab178. eCollection 2021 May.

ABSTRACT

Both adenoid cystic carcinoma (ACC) and thyroid metastasis are quite peculiar clinical presentations. ACC is a malignant salivary gland-type tumour mostly found in the head and neck district, but that can arise from different organs. Due to its rarity, it can pose problems in the diagnostic and therapeutic management. A 72-year-old woman presented for a persistent non-productive cough. A computed tomography showed a lung mass. She underwent lung surgery, and the lesion was an ACC primitive of the lung. She developed hoarseness and ultrasound and cytology confirmed metastatic involvement of left thyroid lobe from ACC. A total thyroidectomy was performed, followed by radiotherapy. The present case highlights the need to be aware of possible metastatic thyroid localization of ACC originating in lower airways. This is a very rare event, and clinical and cytological findings must be carefully examined. It represents an opportunity to consider the current knowledge about ACC metastasis to thyroid.

PMID:34040752 | PMC:PMC8136885 | DOI:10.1093/jscr/rjab178

A Review Leveraging a Rare and Unusual Case of Basal Cell Carcinoma of the Prostate

Fetched: May 27th, 2021, 5:00am GMT by Lin He

Case Rep Pathol. 2021 May 4;2021:5520581. doi: 10.1155/2021/5520581. eCollection 2021.

ABSTRACT

Basal cell carcinoma (BCC) is a rare nonacinar variant of prostatic carcinoma. In spite of prostatic acinar adenocarcinoma being one of the most common carcinomas in prostate, <100 prostatic BCC cases have been reported to date. Adenoid cystic/cribriform histology has been described in varying proportions to occur in prostatic BCC and is reported to be associated with aggressive behavior and high risk of metastasis. Herein, we present a case of prostatic BCC with adenoid cystic morphology, comprehensively describe its immunohistochemical and MYB/MYBL1 gene rearrangement findings, discuss its differential diagnosis, and review the literature of this rare entity.

PMID:34035971 | PMC:PMC8116143 | DOI:10.1155/2021/5520581

The Evaluation and Management of Carcinoma of the Minor Salivary Glands

Fetched: May 25th, 2021, 5:00am GMT by Rohan Walvekar

Otolaryngol Clin North Am. 2021 Jun;54(3):629-639. doi: 10.1016/j.otc.2021.02.011.

ABSTRACT

There are several hundred minor salivary glands throughout the upper aerodigestive tract, aiding in lubrication and protection of the system. Compared with all tumors of the head and neck and those of the six major glands, neoplasms of the minor glands are rare. However, more than half are found to be malignant, prompting a low threshold for further work-up. This review discusses the evaluation of patients who present with masses of the minor salivary glands, including strategies for tissue diagnosis and staging. Management options for and long-term survival outcomes of the most common malignancies affecting these glands are also discussed.

PMID:34024489 | DOI:10.1016/j.otc.2021.02.011

Permalink for 'Intensity modulated radiation therapy may improve survival for tracheal-bronchial adenoid cystic carcinoma: A retrospective study of 133 cases'

Intensity modulated radiation therapy may improve survival for tracheal-bronchial adenoid cystic carcinoma: A retrospective study of 133 cases

Fetched: May 23rd, 2021, 5:00am GMT by Yufan Yang

Lung Cancer. 2021 Jul;157:116-123. doi: 10.1016/j.lungcan.2021.05.006. Epub 2021 May 8.

ABSTRACT

PURPOSES: This study aimed to evaluate the role of radiotherapy (RT) and intensity modulated radiation therapy (IMRT) in adjuvant and definitive settings of tracheal-bronchial adenoid cystic carcinoma (TACC) treatment.

MATERIALS/METHODS: TACC patients (n = 133) treated with surgery and/or RT curatively in our institution between January 1^st, 1984 and December 31^st, 2017 were analyzed retrospectively.

RESULTS: Among the 116 patients undergoing surgery, 50 (43.1 %) achieved complete resections and 66 (56.9 %) had positive surgical margins. For patients with positive margins, overall adjuvant RT was correlated with no significantly improved OS (10-year: 58.0 % vs. 47.9 %; P = 0.340) and a slight LRFS benefit (5-year: 81.9 % vs.75.6 %; P = 0.056), but adjuvant IMRT showed significant superiority in both OS (10-year: 82.9 % vs. 47.9 %; P = 0.031) and LRFS (5-year: 100.0 % vs. 75.6 %; P = 0.001) in comparison with no postoperative RT. Multivariate analysis also identified adjuvant IMRT as a significant favorable factor with OS (HR = 0.186, 95 %CI: 0.039-0.883; P = 0.034). For 17 patients receiving definitive RT, IMRT achieved promising 5-year OS of 88.9 % and LRFS of 64.3 %, yet no significant difference from non-IMRT group was reached (P = 0.447 and 0.706). Different therapies presented no significantly different impact on DMFS, whilst DMFS explained more of the OS variances (P < 0.001, R² = 0.480) than LRFS (P < 0.001, R² = 0.323).

CONCLUSION: IMRT could confer greatly improved OS and LRFS in postoperative setting for TACC patients with positive surgical margins. IMRT was also a good therapeutic option for definitive TACC with promising survival and local control.

PMID:34020823 | DOI:10.1016/j.lungcan.2021.05.006

Permalink for 'Surgical techniques and outcome analysis of uniportal video-assisted thoracic surgery complex sleeve lung resection: a 20 case-series study'

Surgical techniques and outcome analysis of uniportal video-assisted thoracic surgery complex sleeve lung resection: a 20 case-series study

Fetched: May 21st, 2021, 5:00am GMT by Ji-Chen Qu

J Thorac Dis. 2021 Apr;13(4):2255-2263. doi: 10.21037/jtd-20-3002.

ABSTRACT

BACKGROUND: Our study aims to explore the feasibility of uniportal video-assisted complex sleeve lung resection and summarize the surgical techniques and clinical outcomes.

METHODS: From June 2016 to April 2020, a total of 20 complex sleeve pulmonary and distal tracheal resections were performed by the single surgical team at the Thoracic Surgery Department of the Shanghai Pulmonary Hospital. We defined cases as complex sleeve pulmonary resections if they required pulmonary segment sleeve resection, extended sleeve resection (lobectomy plus segmentectomy of the remaining lobe), sleeve pneumonectomy, lobectomy plus carinoplasty or neo-carina construction, pulmonary-sparing main bronchus resection plus carina reconstruction, and distal trachea resection with end to end anastomosis.

RESULTS: The 20 cases comprised lung squamous cell carcinoma (n=11), lung adenocarcinoma (n=2), hamartoma (n=3), adenoid cystic carcinoma (n=2), carcinoid tumor (n=1), and pleomorphic carcinoma (n=1). The average blood loss during the operation was 250±126.17 mL (50-800 mL). The average operation time was 192.0±61.1 minutes. The average number of lymph node stations removed was 5.82±1.33, including station seven in all cases, and the median number of lymph nodes removed was 4.18±5.89. On the day of surgery, the drainage volume was 266±192.01 mL. The mean postoperative hospital stay was 5.37±1.86 days. Twelve of the 16 patients diagnosed with malignancy received postoperative chemotherapy. Granuloma formation at the anastomosis level led to stenosis in one case, and tumor recurrence occurred in one case. Broncho-esophageal fistula occurred in one patient after radiochemotherapy. The postoperative follow-up time was 15.6±10.7 months. The 30-day mortality was zero, and at one-year follow-up, only one patient had died of metastatic disease after the operation.

CONCLUSIONS: Uniportal video-assisted complex sleeve pulmonary resections are feasible when conducted by experienced teams.

PMID:34012576 | PMC:PMC8107526 | DOI:10.21037/jtd-20-3002

Permalink for 'Clinical application of combined infratemporal fossa approaches for internal carotid artery in petrous bone reconstruction for large lateral skull base tumors'

Clinical application of combined infratemporal fossa approaches for internal carotid artery in petrous bone reconstruction for large lateral skull base tumors

Fetched: May 20th, 2021, 5:00am GMT by G D Feng

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2021 May 7;56(5):417-423. doi: 10.3760/cma.j.cn115330-20200613-00500.

ABSTRACT

Objective: By summarizing the technical points and therapeutic outcomes of combing infratemporal fossa approach (IFA) and internal carotid artery (ICA) reconstruction for the colossal skull base tumor invading ICA in petrous bone, the clinical application value was discussed. Methods: Five patients (2 males, 3 females,aging from 27 to 55 years old) who received surgeries between July 2015 and May 2017 for lateral skull base pathology involved petrous ICA using technique combined IFA and pre-reconstruction, were reviewed. Results: Among the five patients, three were paraganglioma of head and neck, one was carotid aneurysms, and one was recurrent adenoid cystic carcinoma (ACC). The median tumor size in the largest cross-section was 60 mm × 51 mm (range, 28 mm × 22 mm-72 mm × 58 mm). Complete excision was achieved with IFA and ICA reconstruction. The median blood loss volume was 1 000 ml (range, 600-2 500 ml). Four cases showed no new long-term neurologic sequelae, while one showed hemiplegia due to graft vessel occlusion. Except for the one with ACC having facial nerve cut, others achieved good facial nerve function of HB grade Ⅰ to Ⅱ during 3 to 12 months, follow-up. No tumor recurrence was observed over the median duration of follow-up for above 36 months (range, 36-58 months). Conclusion: For lesions involved superior part of ICA, which is unable to separate from ICA, IFA and ICA reconstruction can achieve complete excision.

PMID:34010992 | DOI:10.3760/cma.j.cn115330-20200613-00500

Permalink for 'High-grade Transformation/Dedifferentiation in Salivary Gland Carcinomas: Occurrence Across Subtypes and Clinical Significance'

High-grade Transformation/Dedifferentiation in Salivary Gland Carcinomas: Occurrence Across Subtypes and Clinical Significance

Fetched: May 20th, 2021, 5:00am GMT by Alena Skalova

Adv Anat Pathol. 2021 Mar 17. doi: 10.1097/PAP.0000000000000298. Online ahead of print.

ABSTRACT

High-grade transformation (HGT) or dedifferentiation has been described in a variety of salivary gland carcinomas, including acinic cell carcinoma, secretory carcinoma, adenoid cystic carcinoma, epithelial-myoepithelial carcinoma, polymorphous adenocarcinoma, low-grade mucoepidermoid carcinoma, and hyalinizing clear cell carcinoma. High-grade (HG) transformed tumors are composed of a conventional low-grade component characterized by specific microscopic and immunohistochemical features for the given entity, intermingled with or juxtaposed to areas of HG morphology. This is usually either poorly differentiated adenocarcinoma, carcinoma not otherwise specified, or undifferentiated carcinoma, in which the original line of differentiation is lost. The HG component is composed of solid nests of anaplastic cells with large vesicular pleomorphic nuclei, prominent nucleoli, and abundant cytoplasm. Frequent mitoses and extensive necrosis may be present. The Ki-67 labeling index is consistently higher in the HG component. The molecular genetic mechanisms responsible for HGT of salivary gland carcinomas are largely unknown, though p53 inactivation and human epidermal growth factor receptor 2 overexpression and/or gene amplification have been demonstrated in the HG component in a few examples, the frequency varies for each histologic type. Salivary gland carcinomas with HGT are more aggressive than conventional carcinomas, with a higher local recurrence rate and a poorer prognosis. They have a high propensity for cervical lymph node metastasis suggesting a need for a wider resection and neck dissection. HGT of salivary gland carcinoma can occur either at initial presentation or less commonly at the time of recurrence, sometimes following postoperative radiotherapy. The potential for HGT in almost any type of salivary gland carcinoma warrants a thorough sampling of all salivary gland malignancies to prevent oversight of a HG component.

PMID:34009780 | DOI:10.1097/PAP.0000000000000298

Colonic Metastasis of Adenoid Cystic Carcinoma 19 Years after the Primary Tumor Resection

Fetched: May 20th, 2021, 5:00am GMT by Subramanya Sakaleshpura Mallikarjunappa

Case Rep Pathol. 2021 Apr 27;2021:5511935. doi: 10.1155/2021/5511935. eCollection 2021.

ABSTRACT

Adenoid cystic carcinoma (ACC) is a tumor characterized by slow growth and late distant metastasis. The lung and breast are the most common sites for metastasis. Colonic metastasis of such a tumor is rare, with few case reports available. Here, we report a case of ACC arising from minor salivary gland that metastasized to the colon 19 years after the primary tumor resection, with literature review of the clinical, histological, and molecular features of ACC. This case raises our awareness of such tumors as a differential diagnosis of colorectal cancer.

PMID:34007502 | PMC:PMC8100383 | DOI:10.1155/2021/5511935

The expression of the GATA6 gene in oral carcinoma cell lines

Fetched: May 20th, 2021, 5:00am GMT by Cheng-Lin Xu

World J Surg Oncol. 2021 May 18;19(1):153. doi: 10.1186/s12957-021-02245-y.

ABSTRACT

BACKGROUND: This study aimed to investigate the expression level of the GATA6 gene in different oral cancer cells.

METHODS: In this study, we sub-cultured normal oral epithelial cell lines HOK, human tongue squamous cell carcinoma cell lines CAL-27 and SCC-4, and human salivary gland adenoid cystic carcinoma cell lines SACC-LM and SACC-83. Subsequently, we used reverse transcription-polymerase chain reaction RT-PCR and Western blot methods to detect the mRNA and the protein expressions of GATA6 in normal oral epithelial cells, human tongue squamous cell carcinoma cells, and human salivary gland adenoid cystic carcinoma cells.

RESULTS: The results of this study showed that the mRNA expression levels of GATA6 in CAL-27, SCC-4, and SACC-LM cells were significantly increased when compared with the HOK cells. However, the mRNA expression level of GATA6 in the SACC-83 cells had no significant difference compared with the HOK cells. The protein expression levels of GATA6 in the SCC-4 and SACC-LM cells were, however, significantly increased whereas the protein expression levels of GATA6 in the CAL-27 and SACC-83 cells had no significant difference when compared with the HOK cells.

CONCLUSION: The GATA6 gene may be related to the occurrence and progression of certain oral cancers.

PMID:34006300 | PMC:PMC8132435 | DOI:10.1186/s12957-021-02245-y

Permalink for 'Apatinib in patients with recurrent or metastatic adenoid cystic carcinoma of the head and neck: a single-arm, phase II prospective study'

Apatinib in patients with recurrent or metastatic adenoid cystic carcinoma of the head and neck: a single-arm, phase II prospective study

Fetched: May 18th, 2021, 5:00am GMT by Guopei Zhu

Ther Adv Med Oncol. 2021 May 8;13:17588359211013626. doi: 10.1177/17588359211013626. eCollection 2021.

ABSTRACT

BACKGROUND: Apatinib, a vascular endothelial growth factor receptor (VEGFR) blocker, has demonstrated encouraging antitumor activities and tolerable toxicities in various cancer types. Recurrent or metastatic adenoid cystic carcinoma of the head and neck (R/MACCHN) carries a poor prognosis, and treatment options are currently limited. This study was conducted to explore the antitumor activity and safety of apatinib in patients with R/MACCHN.

METHODS: In this phase II single-arm, prospective study, patients aged 15-75 years with incurable R/MACCHN received apatinib at a 500 mg dose once daily until intolerance or progression occurred. The primary endpoint was the 6-month progression-free survival (PFS) rate based on RECIST version 1.1. The secondary endpoints included response rate, overall survival (OS), and safety. Efficacy was assessed in all dosed patients with at least one post-baseline tumor assessment.

RESULTS: Among 68 patients treated with apatinib, 65 were evaluable for efficacy analysis, with a median follow-up time of 25.8 months. The 6-month, 12-month, and 24-month PFS rates were 92.3% [95% confidence interval (CI): 83-97.5%], 75.2% (95% CI: 61.5-84.0%) and 44.7% (95% CI: 32.3-57.5%), respectively. The objective response rate (ORR) and disease control rate (DCR), as assessed by investigators, were 46.2% (95% CI: 33.7-59.0%) and 98.5% (95% CI: 91.7-100.0%), respectively. The median duration of response was 17.7 months [interquartile range (IQR) 14.0-20.9]. The 12-month and 24-month OS rates were 92.3% (95% CI: 83.0-97.5%) and 82.3% (95% CI: 70-90.4%), respectively. The most common adverse events of grades 3-4 were hypertension (5.9%), proteinuria (9.2%), and hemorrhage (5.9%). One patient developed a fatal hemorrhage.

CONCLUSION: An encouraging PFS, a high ORR, and a manageable safety profile were observed in this study. It seems that the administration of apatinib in R/MACCHN is likely to have a clinically meaningful therapeutic benefit and warrants further investigation.This study was prospectively registered in ClinicalTrials.gov (NCT02775370; date of registration: 17 May 2016; date of first patient enrollment: 25 May 2016).

PMID:33995600 | PMC:PMC8111556 | DOI:10.1177/17588359211013626

Permalink for 'Prolonged response to treatment based on cell-free DNA analysis and molecular profiling in three patients with metastatic cancer: a case series'

Prolonged response to treatment based on cell-free DNA analysis and molecular profiling in three patients with metastatic cancer: a case series

Fetched: May 18th, 2021, 5:00am GMT by Mohammad Faraz Naqvi

Ther Adv Med Oncol. 2021 Mar 24;13:17588359211001538. doi: 10.1177/17588359211001538. eCollection 2021.

ABSTRACT

BACKGROUND: Patients with advanced and/or metastatic solid tumors have limited treatment options. Mutations that serve as biomarkers of carcinogenesis can be found in cell-free DNA of patients' plasma. Analysis of circulating tumor DNA (ctDNA) was developed as a non-invasive, cost-effective alternative to tumor biopsy when such biopsy is not technically feasible or it is associated with high risk for complications. The role of ctDNA in precision oncology is promising but its clinical significance across tumor types remains to be validated. We report a case series of three heavily pretreated patients with advanced solid tumors who received matched targeted therapy based on ctDNA analysis and/or tumor molecular profiling.

CASE PRESENTATION: Three patients with advanced, metastatic cancer and the following characteristics are presented: a 71-year-old woman with ovarian cancer and BRCA2 mutation identified in ctDNA and tumor tissue was treated with a PARP inhibitor and achieved partial response by RECIST (Response Evaluation Criteria in Solid Tumors) for 22.6+ months; a 40-year-old woman with adenoid cystic carcinoma of the parotid gland was treated with a MEK/RAF pathway inhibitor on the basis of RAF1 amplification on ctDNA analysis and had stable disease for 20.2 months; and a 56-year-old woman with breast cancer and a BRCA1 mutation identified by ctDNA analysis was treated with a PARP inhibitor and achieved stable disease for 9.1 months. All three patients are alive at the time of this report.

CONCLUSIONS: These results suggest that ctDNA analysis can contribute to selection of targeted therapy in patients with advanced, metastatic cancer. Prospective clinical trials to evaluate and optimize ctDNA biomarkers, as well as the integration of novel and/or alternative targeted therapies, are warranted to fully assess the role of ctDNA analysis in cancer therapy.

TRIAL REGISTRATION: www.clinicaltrials.gov (NCT02152254). Registered May 28, 2014. [https:] MD Anderson protocol # PA12-1161 (approval ID IRB1 FWA00000121) and # PA11-0377 (approval ID IRB4 FWA00005015).

PMID:33995588 | PMC:PMC8107674 | DOI:10.1177/17588359211001538

Predicting Histology of Tracheobronchial Neoplasms: A CT Based Differentiation Model

Fetched: May 18th, 2021, 5:00am GMT by Deeksha Bhalla

Curr Probl Diagn Radiol. 2021 Apr 18:S0363-0188(21)00071-2. doi: 10.1067/j.cpradiol.2021.03.018. Online ahead of print.

ABSTRACT

BACKGROUND: Tracheobronchial (TB) tumors follow same pathological classification as lung neoplasms; however, some entities are known to favor airways. Distinction of pathological types is necessary for suggesting appropriate management strategy.

PURPOSE: To evaluate utility of multidetector CT (MDCT) in differentiation of primary TB tumors; and assess validity of a scoring system based on imaging biomarkers to differentiate tumor types.

METHODS: MDCT features of 45 patients were analyzed for location, shape, calcification, attenuation, parenchymal changes, bronchoceles, extraluminal extension, lymphadenopathy, metastases. The two largest groups were compared with each other and remaining entities using Chi square tests. Six-point scoring system combining the differentiating features was devised and receiver operating characteristic curve analysis performed.

RESULTS: The most frequent type was neuroendocrine tumors (NET) (51.1%), followed by salivary gland tumors (SGT) (20%); including adenoid cystic carcinoma (ACC) (13.3%) and mucoepidermoid carcinoma (MEC) (6.7%). Comparing NETs with other entities as a whole, and independently with SGTs, significant difference was found among location (p = 0.05 and 0.001 respectively), shape (p < 0.001), calcification (p = 0.038 and 0.041 respectively), attenuation (p = 0.001 and 0.019 respectively), bronchoceles (p = 0.013 and 0.010 respectively). Significant difference was found among ACC and MEC in location (p = 0.01) and morphology (p < 0.001). On receiver operating characteristic (ROC) curve analysis of the score, areas under curve for NET, SGT and ACC were 0.913, 0.872 and 0.962 respectively. Suggested cut-off values were >3.5 for carcinoid (sensitivity 70%, specificity 91%), <2.5 for SGT (sensitivity 78%, specificity 75%), <1.5 for ACC (sensitivity 83%, specificity 92%).

CONCLUSION: Use of a scoring system enables maximum diagnostic accuracy in MDCT differentiation of TB tumors.

PMID:33994228 | DOI:10.1067/j.cpradiol.2021.03.018

Veno-venous extra-corporeal membrane oxygenation-assisted right tracheal-sleeve pneumonectomy

Fetched: May 18th, 2021, 5:00am GMT by Antonio Mazzella

Interact Cardiovasc Thorac Surg. 2021 May 16:ivab124. doi: 10.1093/icvts/ivab124. Online ahead of print.

ABSTRACT

Tracheal sleeve pneumonectomy for lung cancer is an old technique, and it is reserved for exceptional cases with tracheal carina involvement. Intra-operative airways management of this operation is incredibly complex, involving thoracic surgeons, anaesthesiologists and pulmonologists. We report a case of a 38-year-old male with no clinical history, referred to our department for an adenoid-cystic carcinoma involving distal trachea, carina and main right bronchus. Tracheal sleeve pneumonectomy was performed using extra-corporeal membrane oxygenation (ECMO). A veno-venous ECMO circuit was established through a heparin-coated percutaneous cannula in the right femoral vein and a heparin-coated percutaneous cannula in the internal right jugular vein by ultrasound assistance. No major complications occurred, and the patient was discharged after 30-day bronchoscopic control, showing the absence of fistula and negativity of the methylene blue test. ECMO-assisted surgery ensures adequate respiratory support, haemodynamic stability, lower risk of bleeding complications with a clean operating field and better brain and myocardial oxygenation.

PMID:33993258 | DOI:10.1093/icvts/ivab124

CT diagnosis and prognosis prediction of tracheal adenoid cystic carcinoma

Fetched: May 18th, 2021, 5:00am GMT by Shu Chao Wang

Eur J Radiol. 2021 Jul;140:109746. doi: 10.1016/j.ejrad.2021.109746. Epub 2021 Apr 30.

ABSTRACT

PURPOSE: To evaluate computed tomography (CT) features and establish a predictive model for the clinical diagnosis and prognosis of tracheal adenoid cystic carcinoma (ACC).

METHOD: From January 2010 to December 2018, 82 patients with tracheal tumors, including 46 patients with ACC confirmed by surgery and histopathology, were enrolled in this study. These patients' clinicopathologic information, CT features and survival outcomes were recorded and analyzed. Independent predictors of diagnosis and prognosis of tracheal ACC were determined by both univariate and multivariate analyses.

RESULTS: Compared with tracheal non-ACC patients, univariate analysis showed that ACC patients were more likely to have extensive longitudinal length (p < 0.001) and to appear as annular wall thickening (p = 0.001), transmural growth (p = 0.036), poorly defined border (p = 0.003) and mild enhancement (p = 0.001). Multivariate logistic analysis showed that longitudinal length and enhancement degree were independent predictors of tracheal ACC. The 3-year and 5-year disease-free survival (DFS) were 75.7 % and 64.5 %, respectively. Longitudinal length (≥ 34 mm), transverse length (≥ 20 mm) and transmural growth were associated with poor DFS in univariate analysis. After multivariate adjustment, only transverse length (≥ 20 mm) was an adverse prognostic factor for DFS (hazard ratio = 4.594, 95 % confidence interval = 1.240-17.017; p = 0.022).

CONCLUSIONS: CT longitudinal length and enhancement degree of tumors showed satisfactory discrimination for tracheal ACC. Excessive CT transverse length might be an unfavorable indicator for ACC recurrence and could be helpful for predicting the survival outcomes of ACC at the initial diagnosis.

PMID:33992979 | DOI:10.1016/j.ejrad.2021.109746

Inhibition of DEC2 is necessary for exiting cell dormancy in salivary adenoid cystic carcinoma

Fetched: May 16th, 2021, 5:00am GMT by Xiao Yang

J Exp Clin Cancer Res. 2021 May 14;40(1):169. doi: 10.1186/s13046-021-01956-0.

ABSTRACT

BACKGROUND: Patients were prone to have poor prognosis once dormant tumor cells being reactivated. However, the molecular mechanism of tumor cell dormancy remains poorly understood. This study aimed to investigate the function of DEC2 in the dormancy of salivary adenoid cystic carcinoma (SACC) in vitro and vivo.

METHODS: The function of DEC2 in tumor dormancy of SACC was investigated in nude mice by establishing primary and lung metastasis model. Meanwhile, the interaction between hypoxia and SACC dormancy and the role of DEC2 were demonstrated through CoCl2 induced hypoxia-mimicking microenvironments. Furthermore, the expression of DEC2 was detected by immunohistochemical staining in primary SACC samples with and without recurrence.

RESULTS: In the primary SACC, DEC2 overexpression inhibited cell proliferation, increased cell population arrested in G0/G1 phase, and participated in dormancy regulation, which limited tumor growth. Intriguingly, in the model of lung metastasis, the level of DEC2 was reduced significantly and resulted in dormancy exit and growth resumption of SACC cells. Then, we found that DEC2 may associate with hypoxia in contributing to tumor dormancy, which might provide a possible cue to explain the different roles of DEC2 in primary and metastasis lesions. And overexpression of DEC2 induced dormancy and promoted migration and invasion through activating EMT program. Finally, DEC2 positive expression was shown to be significantly correlated with recurrence and dormancy of SACC patients.

CONCLUSIONS: These findings provide a novel insight into the role of DEC2 gene in tumor dormancy and metastasis.

PMID:33990215 | PMC:PMC8120837 | DOI:10.1186/s13046-021-01956-0

CD56 expression in basaloid anal squamous cell carcinoma - A potential diagnostic pitfall

Fetched: May 16th, 2021, 5:00am GMT by Sepideh Madahian

Ann Diagn Pathol. 2021 May 8;53:151758. doi: 10.1016/j.anndiagpath.2021.151758. Online ahead of print.

ABSTRACT

Anal squamous cell carcinoma (SqCC) is a morphologically heterogeneous entity. Basaloid and non-keratinizing anal SqCC may be confused with other tumors including neuroendocrine carcinoma due to morphologic overlap, and expression of neuroendocrine markers is not well-studied in anal SqCC. Prompted by a case of anal SqCC that was initially misdiagnosed as neuroendocrine carcinoma on the basis of morphology and CD56 expression, we retrospectively examined the expression of neuroendocrine markers CD56, synaptophysin, and chromogranin in 48 cases of basaloid anal SqCC, with clinicopathologic correlation. HPV16 was identified in 46 cases, HPV33 in one case, and one case was HPV-negative. Three (6.3%) cases demonstrated CD56 expression, including two with diffuse and one with focal expression. Two CD56-positive cases demonstrated basaloid morphology with peripheral palisading and the other demonstrated adenoid cystic/cylindroma-like morphology. None of the cases showed significant synaptophysin or chromogranin expression. The three cases expressing CD56 were HPV16-positive, and one demonstrated a CTNNB1 mutation. There was no difference in clinicopathologic features including stage, outcome, or HPV status, between CD56-positive and negative groups. Our findings support that CD56 expression is infrequently expressed in anal SqCC and is not indicative of neuroendocrine differentiation in the absence of expression of more specific neuroendocrine markers such as synaptophysin and chromogranin. Pathologists should be aware that CD56 expression may occur in basaloid anal SqCC and is a diagnostic pitfall due to morphologic overlap with neuroendocrine carcinoma and other tumors including basal cell carcinoma.

PMID:33989959 | DOI:10.1016/j.anndiagpath.2021.151758

Basal Cell Carcinoma of Prostate With MSMB-NCOA4 Fusion and a Probable Basal Cell Carcinoma In Situ: Case Report

Fetched: May 13th, 2021, 5:00am GMT by Vilde Pedersen

Int J Surg Pathol. 2021 May 12:10668969211017321. doi: 10.1177/10668969211017321. Online ahead of print.

ABSTRACT

Basal cell carcinomas of prostate (BCCP) are very rare. Most arise in the transition zone and thus are associated with lower urinary tract symptoms and rarely associated with elevated prostate-specific antigen (PSA). These features make diagnosis/early diagnosis difficult because of the routine protocols followed. Basal cell carcinomas have distinctive histopathological, immunohistochemical, and to some extent also different molecular characteristics. Basal cell carcinoma in situ (BCCIS) is a nonexistent histological lesion as per the current literature, but here is an attempt to describe it through this case.A 74-year-old man presented with hematuria and previous diagnosis of prostatic hyperplasia. Based on this history, he underwent a prostatectomy ad modum Freyer. Pathological examination surprisingly revealed a diffusely infiltrative tumor with nonacinar adenocarcinoma morphology and many glandular structures probably representing BCCIS. Tumor was diagnosed as BCCP. Patient presented with metastasis to the abdominal wall 8 months postprostatectomy.BCCP is an aggressive type of prostate cancer, which might be challenging to diagnose based on routine protocols. This results in delayed diagnosis and treatment and thus poor prognosis. Furthermore, patients with this subtype of prostate cancer need appropriately designed, and maybe a totally different follow-up regimen as PSA is of no use for BCCP patients. Finally, diagnosis of BCCIS, if agreed upon its existence needs to be studied in larger cohorts as a precursor lesion.

PMID:33978524 | DOI:10.1177/10668969211017321

Expanding role of radiotherapy in adenoid cystic carcinoma of the tracheobronchial tree: a new horizon

Fetched: May 13th, 2021, 5:00am GMT by ChinnaBabu Dracham

Tumori. 2021 May 12:3008916211012461. doi: 10.1177/03008916211012461. Online ahead of print.

ABSTRACT

BACKGROUND: Primary adenoid cystic carcinomas (ACCs) of central trachea-bronchi system are rare and heterogeneous tumors. Definitive radiotherapy (RT) is the recommended treatment in surgically unresectable or incomplete resection or in the presence of severe comorbidities.

OBJECTIVE: To evaluate the clinical features and outcomes of patients with ACC of trachea-bronchi treated with radiotherapy.

METHODS: Retrospective medical records review was done in all patients with histologically confirmed ACC of trachea-bronchi between January 2010 and December 2019. Patient disease and treatment characteristics and toxicity data were analyzed. Overall survival (OS), local recurrence-free survival (LRFS), and distant metastasis-free survival (DMFS) were computed using Kaplan-Meier method (log-rank test).

RESULTS: Nineteen patients (10 women and 9 men) were included in this analysis with median age of 40 years (range, 14-70). Of these patients, 63.2% (n = 12) presented in stage IV disease. Twelve and three patients received definitive (median dose 67.8 Gy) and adjuvant (median dose 50 Gy) RT, respectively. The median follow-up was 42.5 months (range, 4-120); 15 patients were alive and 4 were dead at that time. Local recurrence or progression was observed in 52.6% and distant metastasis found in 47.3% of patients. The 5-year OS, LRFS, and DMFS for all patients were 81.2%, 52.8%, and 39.6%, respectively. Baseline lymph node involvement showed significant impact on OS (56.3% vs 100%, p = 0.011). Among patients receiving definitive RT, patients with higher RT dose (⩾66 Gy) had significantly better survival outcomes (5-year LRFS: 75% vs 16.7%, p = 0.013).

CONCLUSION: Definitive RT is an exemplary treatment for unresectable disease. Higher dose is recommended to improve long-term outcomes.

PMID:33977780 | DOI:10.1177/03008916211012461

Permalink for 'The Chimeric Scapulodorsal Vascularized Latissimus Dorsi Nerve Flap for Immediate Reconstruction of Total Parotidectomy Defects With Facial Nerve Sacrifice: Building a New Program and Preliminary Results From 25 Cases'

The Chimeric Scapulodorsal Vascularized Latissimus Dorsi Nerve Flap for Immediate Reconstruction of Total Parotidectomy Defects With Facial Nerve Sacrifice: Building a New Program and Preliminary Results From 25 Cases

Fetched: May 13th, 2021, 5:00am GMT by Sean S Li

Ann Plast Surg. 2021 May 1;86(5S Suppl 3):S379-S383. doi: 10.1097/SAP.0000000000002746.

ABSTRACT

BACKGROUND: Total parotidectomy with facial nerve sacrifice creates 2 challenging reconstructive problems: restoration of facial contour and facial nerve rehabilitation. Strong evidence suggesting that vascularized nerve grafts are superior to nonvascularized nerve grafts motivated our team to develop a chimeric scapulodorsal flap combining the usual harvestable local tissues with the vascularized latissimus dorsi motor nerve (SD-LDVxN). We present our experiences developing a new program at University of California, San Diego, highlighting our first case here, and present preliminary retrospective results focusing on the functional outcomes of facial nerve reanimation.

MATERIALS AND METHODS: The first case performed in the United States was a 57-year-old woman with stage IVA left parotid adenoid cystic carcinoma and House-Brackmann grade 6 facial palsy. She underwent total parotidectomy with facial nerve sacrifice and a free chimeric SD-LDVxN flap reconstruction. She had an unremarkable postoperative course, and 3- and 6-month follow-up functional results are reported. Preliminary functional results from our total series of 25 patients were reported.

RESULTS: At her 3-month follow-up, she was a House-Brackmann 5 with a static eFACE score of 37, dynamic eFACE score of 31, and smile eFACE score of 48. At her 6-month follow-up, she was a House-Brackmann 5 with a static eFACE score of 50, dynamic eFACE score of 27, and smile eFACE score of 53. Preliminary results from our total series of 25 patients with an average of 5 years of follow-up were a House-Brackmann 2.5 and eFACE scores of 83.1 for static facial symmetry, 67.5 for dynamic facial symmetry, and 77.7 for smile score. Twenty of the 25 patients had postoperative radiotherapy. No local tumor recurrence had been reported. The average reinnervation time was 9 months and ranged from 3 to 15 months.

CONCLUSIONS: The SD-LDVxN flap is a highly resourceful solution to reconstruct complex parotid defects, especially those that sacrifice the facial nerve. The vascularized nerve graft allows for primary facial reanimation. Nerve recovery may be superior to what could be expected with a conventional nerve graft.

PMID:33976066 | DOI:10.1097/SAP.0000000000002746

Intramedullary spinal cord metastasis from an adenoid cystic carcinoma of the external auditory canal: case report

Fetched: May 11th, 2021, 5:00am GMT by N Marchesini

Br J Neurosurg. 2021 May 10:1-5. doi: 10.1080/02688697.2021.1923653. Online ahead of print.

ABSTRACT

BACKGROUND AND IMPORTANCE: Intramedullary spinal cord metastases (ISCMs) are rare and no cases of ISCM from an adenoid cystic carcinoma (ACC) of the external auditory canal (EAC) have been reported.

CLINICAL PRESENTATION: We report a 54-year old man complaining backpain and worsening myelopathy. He had an ACC of the EAC resected years prior. A spinal MRI demonstrated a contrast-enhancing intramedullary lesion within the conus medullaris. The histopathological diagnosis of the patient was consistent with the patient's primary cancer. At 3 months follow-up, the neurological condition of the patient was unchanged.

CONCLUSIONS: This is the first reported case of ISCM from a primary ACC of the EAC.

PMID:33970733 | DOI:10.1080/02688697.2021.1923653

Utilization of Nivolumab in Adenoid Cystic Carcinoma After Progression on Platinum-Based Chemotherapy

Fetched: May 11th, 2021, 5:00am GMT by Diana V Maslov

Perm J. 2021 May;25. doi: 10.7812/TPP/20.229.

ABSTRACT

INTRODUCTION: Adenoid cystic carcinoma (ACC) is a rare malignant neoplasm within the secretory glands of the head and neck. Clinical findings include a lump on the palate, tongue, or bottom of the mouth. Because symptoms can be mild, patients go for long periods of time without investigation. ACC is diagnosed using histology. Treatment is by surgical resection because there is no effective chemotherapy. Radiation can be effective adjuvant therapy, and proton therapy and stereotactic irradiation can be used for those who are ineligible for surgery. Immunotherapy has clinical activity for those with metastatic head and neck cancers who progress on proton therapy. This case reviews the use of immunotherapy in a patient with ACC.

CASE PRESENTATION: A man in his 20s presented with a 6-month history of nasal congestion, epistaxis, and sinus tenderness. Noncontrast computed tomography of the sinuses revealed a mass of the lateral wall of the nasal cavity, lateral wall of the maxillary sinus, and pterygoid plates. Positron emission tomography confirmed metastatic disease in the right iliac crest and right cervical lymph node; biopsy of the nasopharynx confirmed ACC. The patient received proton therapy and intensity-modulated radiotherapy and completed 2 Phase 1 trails but continued to have progressive disease. The patient started nivolumab and died 12 weeks later.

CONCLUSION: The patient recently received proton therapy, intensity-modulated radiotherapy, and completed 2 Phase 1 trials but continued to have progressive disease.

PMID:33970093 | DOI:10.7812/TPP/20.229

Impact of anatomic site of distant metastasis on survival in salivary gland cancers

Fetched: May 8th, 2021, 5:00am GMT by William Tyler Turchan

Head Neck. 2021 May 7. doi: 10.1002/hed.26727. Online ahead of print.

ABSTRACT

BACKGROUND: As exemplified in patients with adenoid cystic carcinoma (ACC), metastatic salivary gland cancers display heterogenous behavior. Although anatomic site of metastasis has been suggested to be prognostic for survival in this population, this is not adequately characterized in the current literature.

METHODS: Using the National Cancer Database (NCDB), patients with newly diagnosed metastatic salivary gland cancers with distant metastasis to a single organ were identified.

RESULTS: Eight hundred and fifty-eight patients (n = 284 bone-only, n = 322 lung-only, n = 252 other-site-only) were identified. Anatomic site of distant metastasis was not associated with survival in the cohort as a whole; however, on pre-planned subgroup analysis, lung-only metastasis, relative to bone-only metastasis, was the only factor associated with improved survival in patients with ACC (HR: 0.52, 95%CI: 0.30-0.93, p = 0.029).

CONCLUSIONS: Anatomic site of metastasis is strongly associated with survival in patients with metastatic ACC and should be considered in future studies aiming to optimize therapy in this population.

PMID:33961718 | DOI:10.1002/hed.26727

$Permalink for 'Preclinical development and first in human study of KA2507, a selective and potent inhibitor of histone deacetylase 6, for patients with refractory solid tumors'$

Preclinical development and first in human study of KA2507, a selective and potent inhibitor of histone deacetylase 6, for patients with refractory solid tumors

Fetched: May 6th, 2021, 5:00am GMT by Apostolia M Tsimberidou

Clin Cancer Res. 2021 May 4. doi: 10.1158/1078-0432.CCR-21-0238. Online ahead of print.

ABSTRACT

PURPOSE: Inhibition of histone deacetylase 6 (HDAC6) is predicted to deliver both direct antitumor activity and modulation of the antitumor immune response. This study describes the development of a novel HDAC6 inhibitor.

PATIENTS AND METHODS: KA2507 was characterized in HDAC biochemical and cellular target engagement assays and in preclinical efficacy models of melanoma and colorectal cancer. In a phase I study, KA2507 was administered orally using a 3+3 dose-escalation design (NCT03008018).

RESULTS: KA2507 is a potent and selective inhibitor of HDAC6 (biochemical IC50 = 2.5 nmol/L). Preclinical models demonstrated antitumor efficacy in syngeneic tumor-bearing mice, with translational studies highlighting modulation of the antitumor immune response. Twenty patients were treated in a phase I study. KA2507 was well tolerated; dose-limiting toxicity was not observed up to the maximum dose administered. Pharmacokinetic profiling supported twice-daily oral dosing. Pharmacodynamic analysis demonstrated selective HDAC6 target engagement in peripheral blood cells, free from off-target class I HDAC activity. Stable disease was the best clinical response (7 patients). Three of these patients (adenoid cystic carcinoma, n = 2; rectal adenocarcinoma, n = 1) had prolonged disease stabilization that lasted for 16.4, 12.6, and 9.0 months, respectively.

CONCLUSIONS: KA2507 is a potent and selective inhibitor of HDAC6 showing antitumor efficacy and immune modulatory effects in preclinical models. In a phase I study, KA2507 showed selective target engagement, no significant toxicities, and prolonged disease stabilization in a subset of patients. Further clinical studies of KA2507 are warranted, as a single agent or, preferably, combined with other immuno-oncology drugs.

PMID:33947698 | DOI:10.1158/1078-0432.CCR-21-0238

Permalink for 'Use of three-dimensional printing to guide fibreoptic intubation in a patient with a difficult airway caused by massive adenoid cystic carcinoma of the maxillary bone: A case report'

Use of three-dimensional printing to guide fibreoptic intubation in a patient with a difficult airway caused by massive adenoid cystic carcinoma of the maxillary bone: A case report

Fetched: May 5th, 2021, 5:00am GMT by Xiao Zhang

Anaesth Intensive Care. 2021 May 3:310057X20971648. doi: 10.1177/0310057X20971648. Online ahead of print.

NO ABSTRACT

PMID:33940941 | DOI:10.1177/0310057X20971648

Permalink for '(68)Ga-FAPI-PET/CT improves diagnostic staging and radiotherapy planning of Adenoid Cystic Carcinomas - imaging analysis and histological validation'

(68)Ga-FAPI-PET/CT improves diagnostic staging and radiotherapy planning of Adenoid Cystic Carcinomas - imaging analysis and histological validation

Fetched: May 5th, 2021, 5:00am GMT by Manuel Röhrich

Radiother Oncol. 2021 May 1;160:192-201. doi: 10.1016/j.radonc.2021.04.016. Online ahead of print.

ABSTRACT

BACKGROUND: Adenoid cystic carcinomas (ACCs) are rare epithelial tumors mostly situated in the head and neck region and characterized by infiltrative growth. The tumor stroma of ACCs includes cancer-associated fibroblasts (CAFs) expressing Fibroblast Activation Protein (FAP), a new target for positron emission tomography (PET) imaging. Here we describe the value of PET/ computed tomography (PET/CT) imaging using ⁶⁸Ga-labelled FAP-Inhibitors (⁶⁸Ga-FAPI-PET/CT) and their clinical potential for staging and radiotherapy planning in 12 ACC patients (7 primary, 5 recurrent).

PATIENTS AND METHODS: Patients underwent contrast enhanced staging CT (ceCT) and magnetic resonance imaging (ceMRI) before ⁶⁸Ga-FAPI - PET/CT. PET-scans were acquired 10, 60 and 180 minutes after administration of 150-250 MBq of ⁶⁸Ga-labelled FAPI tracers. SUVmax and SUVmean values of ACCs and healthy organs were obtained using a 60% of maximum iso-contour. FAP and alpha smooth muscle actin (α-SMA) immunohistochemistry was performed in 13 cases (3 with and 10 without ⁶⁸Ga FAPI-PET/CT). Staging and radiotherapy planning based on ⁶⁸Ga-FAPI-PET/CT versus ceCT/MRI alone were compared.

RESULTS: We observed elevated tracer uptake in all ACCs. Immunohistochemistry showed FAP-expressing CAFs in the tumor. Compared to conventional staging, ⁶⁸Ga-FAPI-PET/CT led to upstaging in 2/12 patients and to detection of additional metastases in 3 patients, thus in total 42% of patients had their staging altered. Moreover, ⁶⁸Ga-FAPI PET improved the accuracy of target volume delineation for radiotherapy, as compared to CT and MRI.

CONCLUSION: ⁶⁸Ga-FAPI-PET/CT is a promising imaging modality for ACC, increasing the accuracy of staging exams and radiotherapy planning volumes, as compared conventional to CT and MRI.

PMID:33940087 | DOI:10.1016/j.radonc.2021.04.016

Permalink for 'Identification and characterization of primary cilia-positive salivary gland tumours exhibiting basaloid/myoepithelial differentiation'

Identification and characterization of primary cilia-positive salivary gland tumours exhibiting basaloid/myoepithelial differentiation

Fetched: May 2nd, 2021, 5:00am GMT by Kazuya Shinmura

J Pathol. 2021 Apr 30. doi: 10.1002/path.5688. Online ahead of print.

ABSTRACT

Primary cilia (PC) are non-motile, antenna-like structures on the cell surface. Many types of neoplasms exhibit PC loss, whereas in some neoplasms PC are retained and involved in tumourigenesis. To elucidate the PC status and characteristics of major salivary gland tumours (SGTs), we examined 100 major SGTs encompassing eight histopathological types by immunohistochemical analysis. PC were present in all (100%) of the pleomorphic adenomas (PAs), basal cell adenomas (BCAs), adenoid cystic carcinomas (AdCCs), and basal cell adenocarcinomas (BCAcs) examined, but absent in all (0%) of the Warthin tumours, salivary duct carcinomas, mucoepidermoid carcinomas, and acinic cell carcinomas examined. PC were also detected by electron-microscopic analysis using the NanoSuit method. It is worthy of note that the former category and latter category of tumours contained and did not contain a basaloid/myoepithelial differentiation component, respectively. The four types of PC-positive SGTs showed longer PC than normal and exhibited a characteristic distribution pattern of the PC in the ductal and basaloid/neoplastic myoepithelial components. Two PC-positive carcinomas (AdCC and BCAc) still possessed PC in their recurrent/metastatic sites. Interestingly, activation of the Hedgehog signalling pathway, shown by predominantly nuclear GLI1 expression, was significantly more frequently observed in PC-positive SGTs. Finally, we identified tau tubulin kinase 2 (TTBK2) as being possibly involved in the production of PC in SGTs. Taken together, our findings indicate that SGTs that exhibit basaloid/myoepithelial differentiation (PA, BCA, AdCC, and BCAc) are ciliated, and their PC exhibit tumour-specific characteristics, are involved in activation of the Hedgehog pathway, and are associated with TTBK2 upregulation, providing a significant and important link between SGT tumourigenesis and PC. © 2021 The Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

PMID:33931860 | DOI:10.1002/path.5688

Permalink for 'Epidemiology of Primary Epithelial Salivary Gland Tumors in Southern Poland-A 26-Year, Clinicopathologic, Retrospective Analysis'

Epidemiology of Primary Epithelial Salivary Gland Tumors in Southern Poland-A 26-Year, Clinicopathologic, Retrospective Analysis

Fetched: May 1st, 2021, 5:00am GMT by Michał Gontarz

J Clin Med. 2021 Apr 13;10(8):1663. doi: 10.3390/jcm10081663.

ABSTRACT

(1) Background: Epidemiological studies of epithelial salivary gland neoplasms are difficult to conduct effectively due to tumor rarity, histological heterogeneity, tumor location diversity, and a lack of national registries collecting data. This study presents 26 years of epidemiological data from a single institution in southern Poland that estimates incidence rates of primary epithelial salivary gland tumors. (2) Methods: The charts of 805 patients with epithelial salivary gland tumors were retrospectively reviewed. (3) Results: Pleomorphic adenomas occurred less frequently in elderly patients; however, Warthin tumors were more common (p < 0.001). Pediatric patients mainly suffered from mucoepidermoid carcinoma. The estimated crude and European age-standardized incidence rates of all primary epithelial salivary gland tumors were 6.7 and 6.02 per 100,000 population, respectively. The incidence rates of salivary gland tumors increased in recent years; however, this is attributed to an increase in benign tumors (p < 0.001). (4) Conclusions: The incidence of primary epithelial salivary gland tumors in southern Poland has increased over the past 26 years. This increase is attributed to a rise in the number of patients with benign tumors, particularly Warthin tumors in elderly patients. Moreover, the incidence of malignant salivary gland tumors appears to be higher in pediatric patients.

PMID:33924553 | PMC:PMC8070067 | DOI:10.3390/jcm10081663

Adenoid cystic carcinoma of the male breast: report of a case

Fetched: April 30th, 2021, 5:00am GMT by L N Zhao

Zhonghua Bing Li Xue Za Zhi. 2021 May 8;50(5):535-537. doi: 10.3760/cma.j.cn112151-20210121-00064.

NO ABSTRACT

PMID:33915669 | DOI:10.3760/cma.j.cn112151-20210121-00064

Management of Salivary Gland Malignancy: ASCO Guideline

Fetched: April 27th, 2021, 5:00am GMT by Jessica L Geiger

J Clin Oncol. 2021 Jun 10;39(17):1909-1941. doi: 10.1200/JCO.21.00449. Epub 2021 Apr 26.

ABSTRACT

PURPOSE: To provide evidence-based recommendations for practicing physicians and other healthcare providers on the management of salivary gland malignancy.

METHODS: ASCO convened an Expert Panel of medical oncology, surgical oncology, radiation oncology, neuroradiology, pathology, and patient advocacy experts to conduct a literature search, which included systematic reviews, meta-analyses, randomized controlled trials, and prospective and retrospective comparative observational studies published from 2000 through 2020. Outcomes of interest included survival, diagnostic accuracy, disease recurrence, and quality of life. Expert Panel members used available evidence and informal consensus to develop evidence-based guideline recommendations.

RESULTS: The literature search identified 293 relevant studies to inform the evidence base for this guideline. Six main clinical questions were addressed, which included subquestions on preoperative evaluations, surgical diagnostic and therapeutic procedures, appropriate radiotherapy techniques, the role of systemic therapy, and follow-up evaluations.

RECOMMENDATIONS: When possible, evidence-based recommendations were developed to address the diagnosis and appropriate preoperative evaluations for patients with a salivary gland malignancy, therapeutic procedures, and appropriate treatment options in various salivary gland histologies.Additional information is available at www.asco.org/head-neck-cancer-guidelines.

PMID:33900808 | DOI:10.1200/JCO.21.00449

Pan-TRK Immunohistochemistry Is Highly Correlated With NTRK3 Gene Rearrangements in Salivary Gland Tumors

Fetched: April 27th, 2021, 5:00am GMT by Marie Csanyi-Bastien

Am J Surg Pathol. 2021 Apr 23. doi: 10.1097/PAS.0000000000001718. Online ahead of print.

ABSTRACT

Secretory carcinoma (SC) is characterized by ETV6 rearrangements, most often ETV6-NTRK3 fusion. Given its histologic overlap with other salivary gland tumors (SGTs), SCs can be difficult to diagnose without genetic confirmation. A recently developed pan-TRK (tropomyosin receptor kinase) antibody shows promise for identifying tumors with NTRK (neurotrophic tyrosine kinase receptor 3) fusions. The aim of this study was to evaluate the utility of pan-TRK immunohistochemistry in distinguishing SCs from mimics and selecting patients eligible for TRK inhibitor clinical trials. We examined whole-tissue sections from 111 SGTs with molecular characterization, including 26 SCs (23 with ETV6-NTRK3 fusion and 3 with ETV6-RET fusion detected by ligation-dependent reverse transcription-polymerase chain reaction, next-generation sequencing and 85 non-SC SGTs (no ETV6-NTRK3 fusion). Immunohistochemistry was performed with a pan-TRK rabbit monoclonal antibody. When any pan-TRK staining (nuclear or cytoplasmic with any staining intensity) was considered to indicate positivity, 22 of 23 SCs with ETV6-NTRK3 fusion (95.7%) and 33 of 85 non-SC (38.8%) salivary neoplasms were positive, mainly basal cell adenoma, pleomorphic adenomas, adenoid cystic carcinomas, and epithelial-myoepithelial carcinomas. All SCs with ETV6-RET fusion were entirely negative. When only nuclear pan-TRK staining with any staining intensity was considered positive, 18 of 23 SCs with ETV6-NTRK3 fusion (78.3%) were positive, 11 among them with diffuse staining (>30% of cells). All non-SCs and SCs with ETV6-RET fusion were entirely negative. In comparison to molecular analysis (ligation-dependent reverse transcription-polymerase chain reaction, next-generation sequencing), nuclear pan-TRK IHC has a sensitivity of 78.3% and a specificity of 100% for diagnosing SCs with ETV6-NTRK3 fusion, 69% and 100% for SCs (all fusions). Pan-TRK is a reasonable screening test for diagnosing SCs among SGTs when taking only nuclear staining into account. Although pan-TRK expression is not entirely sensitive for SCs, nuclear staining is highly specific for SCs with ETV6-NTRK3 fusion. The lack of pan-TRK immunoreactivity in a subset of SCs is suggestive of atypical exons 4 to 14 or exons 5 to 14 ETV6-NTRK3 fusion or non-NTRK alternative fusion partners such as ETV6-RET. Pan-TRK staining can serve as a strong diagnostic marker to distinguish SC from it mimics and to select patients eligible for TRK inhibitor clinical trials.

PMID:33899788 | DOI:10.1097/PAS.0000000000001718

Permalink for 'Exploration of High-Grade Transformation and Postoperative Radiotherapy on Prognostic Analysis for Primary Adenoid Cystic Carcinoma of the Head and Neck'

Exploration of High-Grade Transformation and Postoperative Radiotherapy on Prognostic Analysis for Primary Adenoid Cystic Carcinoma of the Head and Neck

Fetched: April 27th, 2021, 5:00am GMT by Yuelu Zhu

Front Oncol. 2021 Apr 9;11:647172. doi: 10.3389/fonc.2021.647172. eCollection 2021.

ABSTRACT

BACKGROUND: Despite Adenoid cystic carcinoma (ACC) with cribriform or tubular components being recognized as a potentially indolent malignancy, ACC displaying solid or, more rarely, high-grade transformation (HGT) components is considered a more aggressive variant of the disease. As it is difficult to measure the proportion of the solid component objectively, and the role of HGT in the current grading system remains unclear, the prognostic influence of tumor grading remains controversial. In addition, postoperative radiotherapy (PORT) has been proven to be effective in local control of ACC of the head and neck (ACCHN) with a high rate of nerve invasion and close surgical margin. However it remains to be explored that whether PORT could improve the survival of patients with ACC, particularly those with HGT.

METHODS: A series of 73 surgically treated primary ACCHN cases were retrospectively accessed. Immunohistochemical staining was performed to observe the biphasic ductal-myoepithelial differentiation and to identify the HGT components of ACC for tumor grading. The correlation between tumor grading and clinicopathological characteristics was analyzed. Univariate and multivariate prognostic analysis were performed for progression-free survival (PFS) and overall survival (OS).

RESULTS: Of the 73 included cases, 47 were grade I-II ACC and 26 were grade III ACC. Among the grade III cases, 14 with loss of biphasic ductal-myoepithelial differentiation identified by immunostaining were classified as HGT, and could be distinguished from conventional grade III cases. These HGT cases were correlated with a high propensity of lymph node metastases and more advanced stage. Univariate analysis demonstrated that tumor grading, perineural invasion, T stage, stage groups, and PORT were predictors for PFS, whereas tumor grading, margin status, and PORT were predictors for OS. However, only tumor grading and PORT were independent predictors for PFS and OS. The patients with HGT had significantly worse prognosis than those with conventional ACC. Moreover, disease progression tended to occur more frequently in younger patients. Among the patients with HGT, those who received PORT had a longer median survival time than those who did not.

CONCLUSION: HGT ACC identified by loss of biphasic differentiation should be considered in tumor grading. Tumor grading and PORT were independent predictors for disease progression and OS in surgically treated ACCHN patients.

PMID:33898317 | PMC:PMC8063729 | DOI:10.3389/fonc.2021.647172

Pulmonary Cylindromas in CYLD Cutaneous Syndrome: A Rare Differential Diagnosis of Pulmonary Adenoid Cystic Carcinoma

Fetched: April 27th, 2021, 5:00am GMT by Judith E Bülau

Clin Lung Cancer. 2021 Mar 27:S1525-7304(21)00070-X. doi: 10.1016/j.cllc.2021.03.016. Online ahead of print.

NO ABSTRACT

PMID:33896728 | DOI:10.1016/j.cllc.2021.03.016

Permalink for 'Diagnostic discrepancy in second opinion reviews of primary epithelial neoplasms involving salivary gland: An 11-year experience from a tertiary referral center focusing on useful pathologic approaches and potential clinical impacts'

Diagnostic discrepancy in second opinion reviews of primary epithelial neoplasms involving salivary gland: An 11-year experience from a tertiary referral center focusing on useful pathologic approaches and potential clinical impacts

Fetched: April 25th, 2021, 5:00am GMT by Bin Xu

Head Neck. 2021 Apr 24. doi: 10.1002/hed.26719. Online ahead of print.

ABSTRACT

AIMS: In the era of precision medicine, accurate pathologic diagnoses are crucial for appropriate management.

METHODS: We herein described the histologic features and clinical impacts of 66 salivary gland epithelial neoplasms in which the diagnosis was altered after expert review.

RESULTS: The most common revised diagnosis was that of salivary duct carcinoma (SDC, n = 12), adenoid cystic carcinoma (n = 12), and myoepithelial carcinoma (n = 10). The most common initial diagnosis was mucoepidermoid carcinoma (n = 19) with SDC being the most common revised diagnosis (7/19). Thirteen salivary gland carcinomas were initially diagnosed as benign entities, whereas five benign tumors were initially interpreted as carcinoma. The change in diagnosis was considered to be clinically significant in 65 (97%) cases.

CONCLUSIONS: Given their rarity, salivary gland neoplasms are prone to diagnostic inaccuracy and discrepancy. A constellation of histologic features and ancillary studies are useful in reaching the correct diagnosis, which can have significant clinical impacts.

PMID:33893750 | DOI:10.1002/hed.26719

Permalink for 'SalvGlandDx - a comprehensive salivary gland neoplasm specific next generation sequencing panel to facilitate diagnosis and identify therapeutic targets'

SalvGlandDx - a comprehensive salivary gland neoplasm specific next generation sequencing panel to facilitate diagnosis and identify therapeutic targets

Fetched: April 21st, 2021, 5:00am GMT by Sandra N Freiberger

Neoplasia. 2021 May;23(5):473-487. doi: 10.1016/j.neo.2021.03.008. Epub 2021 Apr 18.

ABSTRACT

Diagnosis of salivary gland neoplasms is often challenging due to their high morphological diversity and overlaps. Several recurrent molecular alterations have been described recently, which can serve as powerful diagnostic tools and potential therapeutic targets (e.g. NTRK or RET fusions). However, current sequential molecular testing can be expensive and time consuming. In order to facilitate the diagnosis of salivary gland neoplasms, we designed an all-in-one RNA-based next generation sequencing panel suitable for the detection of mutations, fusions and gene expression levels (including NR4A3) of 27 genes involved in salivary gland neoplasms. Here we present the validation of the "SalvGlandDx" panel on FFPE histological specimen including fine needle aspiration (FNA) cell block material, against the standard methods currently used at our institution. In a second part we describe selected unique cases in which the SalvGlandDx panel allowed proper diagnosis and new insights into special molecular characteristics of selected salivary gland tumors. We characterize a unique salivary gland adenocarcinoma harboring a ZCCHC7-NTRK2 fusion, a highly uncommon spindle cell and pseudoangiomatoid adenoid-cystic carcinoma with MYBL1-NFIB fusion, and a purely oncocytic mucoepidermoid carcinoma, whereas diagnosis could be made by detection of a CRTC3-MAML2 rearrangement on the cell block specimen of the FNA. Further, a rare case of a SS18-ZBTB7A rearranged low-grade adenocarcinoma previously described as potential spectrum of microsecretory adenocarcinoma, is reported. In addition, features of six cases within the spectrum of polymorphous adenocarcinoma / cribriform adenocarcinoma of salivary gland including PRKD1 p.E710D mutations and novel fusions involving PRKAR2A-PRKD1, SNX9-PRKD1 and ATL2-PRKD3, are described.

PMID:33878706 | PMC:PMC8081865 | DOI:10.1016/j.neo.2021.03.008

Similar therapeutic effects of (125)I seed radiotherapy and gamma-ray radiotherapy on lacrimal gland adenoid cystic carcinoma

Fetched: April 21st, 2021, 5:00am GMT by Rui Liu

Int J Ophthalmol. 2021 Apr 18;14(4):547-553. doi: 10.18240/ijo.2021.04.11. eCollection 2021.

ABSTRACT

AIM: To evaluate the survival outcomes of patients with lacrimal gland adenoid cystic carcinoma who underwent eye-sparing surgery combined with ¹²⁵I seed implantation radiotherapy or local external γ-ray radiotherapy.

METHODS: In this retrospective comparative case series, the clinical records of 27 primary and 8 recurrent patients were reviewed. Univariate and multivariate analyses were used to identify risk factors associated with distant metastasis (DM), and the overall survival (OS) after the initial surgery was analyzed.

RESULTS: The median follow-up after radiotherapy was 36mo (range 6-120mo). At the last follow-up after radiotherapy, 26 (74.3%) patients had no evidence of disease, 7 (20%) patients had DM, 2 (5.9%) patients died of DM, and 1 patient with DM was lost to follow-up. Univariate analyses showed that duration of symptoms, bone destruction, T stage classification, and wide excision surgery were risk factors influencing DM (P<0.05). The 5-year and 10-year OS rates after the initial surgery were 95.8% and 79.9%, respectively. The 5-year DM-free survival and disease-free survival rates after radiotherapy were 66.4% and 52.7%, respectively.

CONCLUSION: ¹²⁵I seed radiotherapy and local external γ-ray radiotherapy may have similar therapeutic effects in preventing DM. Patients with T1/T2 stage disease have a better prognosis than those with T3/T4 stage disease.

PMID:33875946 | PMC:PMC8025165 | DOI:10.18240/ijo.2021.04.11

Patterns of Care and Outcomes of Primary Adenoid Cystic Carcinoma of the Trachea

Fetched: April 20th, 2021, 5:00am GMT by Sara Behbahani

Ann Otol Rhinol Laryngol. 2021 Apr 19:34894211008101. doi: 10.1177/00034894211008101. Online ahead of print.

ABSTRACT

OBJECTIVE: Primary tracheal malignancies are relatively rare cancers, representing 0.1% to 0.4% of all malignancies. Adenoid cystic carcinoma (ACC) is the second most common histology of primary tracheal malignancy, after squamous cell carcinoma. This study aims to analyze demographic characteristics and potential influencing factors on survival of tracheal ACC (TACC).

METHODS: This was a retrospective cohort study utilizing the National Cancer Database (NCDB). The NCDB was queried for all cases of TACC diagnosed from 2004 to 2016 (n = 394). Kaplan-Meier (KM) and Cox proportional-hazards models were used to determine clinicopathological and treatment factors associated with survival outcomes.

RESULTS: Median age of diagnosis was 56 (IQR: 44.75-66.00). Females were affected slightly more than males (53.8% vs 46.2%). The most prevalent tumor diameter range was 20 to 39 mm (34.8%) followed by greater than 40 mm in diameter (17.8%). Median overall survival (OS) was 9.72 years with a 5- and 10-year OS of 70% and 47.5%, respectively. Localized disease was not associated with a survival benefit over invasive disease (P = .388). The most common intervention was surgery combined with radiation therapy (RT) at 46.2%, followed by surgery alone (16.8%), and standalone RT (8.9%). When adjusting for confounders, surgical resection was independently associated with improved OS (HR 0.461, 95% CI 0.225-0.946). Tumor size greater than 40 mm was independently associated with worse OS (HR 2.808; 95% CI 1.096-7.194).

CONCLUSION: Our data suggests that surgical resection, possibly in conjunction with radiation therapy, is associated with improved survival, and tumor larger than 40 mm are associated with worse survival.

PMID:33870720 | DOI:10.1177/00034894211008101

Permalink for 'Clinical Utility of Fine Needle Aspiration Cytology for Adenoid Cystic Carcinoma of the Trachea with Thyroid Invasion: A Case Report'

Clinical Utility of Fine Needle Aspiration Cytology for Adenoid Cystic Carcinoma of the Trachea with Thyroid Invasion: A Case Report

Fetched: April 20th, 2021, 5:00am GMT by Tomoo Jikuzono

J Nippon Med Sch. 2021 Apr 19. doi: 10.1272/jnms.JNMS.2022_89-207. Online ahead of print.

ABSTRACT

BACKGROUND: Adenoid cystic carcinoma of the trachea (ACCT) is a rare cancer; ACCT with thyroid invasion is particularly rare. We first suspected anaplastic thyroid carcinoma (ATC) but diagnosed ACC after performing fine needle aspiration cytology (FNAC). A tracheal origin was confirmed after operation.

CASE DESCRIPTION: We report the case of a 77-year-old female presenting to our hospital with acute inspiratory dyspnea requiring emergency tracheotomy. Physical examination revealed a right anterior neck swelling with a hard and unmovable mass. Computed tomography (CT) and ultrasonography (US) showed tumor extension to the right thyroid lobe, and between the first and third tracheal rings, which caused severe stenosis of the lumen. Next, we performed FNAC. Clinical findings were highly suspicious for ACCT with thyroid invasion. Thirty-five days after the first visit to our department, the patient underwent total laryngectomy, cervical esophagectomy, and thyroidectomy with bilateral selective neck dissections at another hospital. The tumor was located in the right posterior wall of the trachea, with extension into the right thyroid gland. Pathological examination showed an infiltrative carcinomatous proliferation with tubular and cribriform patterns. The tumor was classified as pT4N1. A definite diagnosis was made after histopathological analyis of the surgical specimen confirmed ACCT. The tumor was found to be positive for FABP7, a putative prognostic marker of ACC, and metastasized to the lungs 3 years after the surgery.

CONCLUSIONS: ACCT with thyroid invasion is an extremely rare malignant neoplasm. FNAC was useful for differentiating ACCT from other diagnoses and enabled appropriate surgical treatment.

PMID:33867428 | DOI:10.1272/jnms.JNMS.2022_89-207

Solitary Renal Metastasis Arising from a Pulmonary Adenoid Cystic Carcinoma: A Case Report and Review of the Literature

Fetched: April 17th, 2021, 5:00am GMT by Prodromos Philippou

Case Rep Urol. 2021 Mar 30;2021:8863475. doi: 10.1155/2021/8863475. eCollection 2021.

ABSTRACT

Adenoid cystic carcinoma (ACC) is a malignancy affecting the salivary glands and rarely involving the lung. Due to its rarity, primary lung ACC remains incompletely understood. We herein report the case of a 57-year-old female patient who was initially diagnosed with primary lung ACC and was treated by lobectomy. Seven years later, an abdominal computed tomography scan performed in the context of surveillance revealed the presence of a solid lesion arising from the lower pole of the left kidney. The patient underwent left partial nephrectomy, and histopathology confirmed a completely excised metastatic ACC.

PMID:33859861 | PMC:PMC8024106 | DOI:10.1155/2021/8863475

Permalink for 'Clinical and Demographic Factors, Treatment Patterns, and Overall Survival Associated With Rare Triple-Negative Breast Carcinomas in the US'

Clinical and Demographic Factors, Treatment Patterns, and Overall Survival Associated With Rare Triple-Negative Breast Carcinomas in the US

Fetched: April 13th, 2021, 5:00am GMT by Elizabeth B Elimimian

JAMA Netw Open. 2021 Apr 1;4(4):e214123. doi: 10.1001/jamanetworkopen.2021.4123.

ABSTRACT

IMPORTANCE: Triple-negative breast cancers are known collectively to demonstrate a more aggressive clinical course and earlier recurrence than cancers of other histological subtypes. However, the literature on rare triple-negative breast cancers and the association of histological type with survival and risk of metastasis is sparse.

OBJECTIVE: To present the clinical and demographic characteristics, treatment patterns, and overall survival (OS) for histologically rare (<10% of breast cancers) triple-negative breast cancer types: medullary carcinoma, adenoid cystic carcinoma, and metaplastic breast carcinoma.

DESIGN, SETTING, AND PARTICIPANTS: This cohort study was performed in the US using data reported by the National Cancer Database between 2010 and 2016. Confirmed cases of medullary carcinoma, adenoid cystic carcinoma, and metaplastic breast cancer were analyzed. Univariable analyses and multivariable Cox regression models were performed. Data analysis was performed from April to May 2020.

MAIN OUTCOMES AND MEASURES: The primary outcome was 5-year OS. Secondary outcomes included site of metastasis, effect of immunohistochemistry, management, and 2-year mortality.

RESULTS: A total of 8479 patients with breast cancer (mean [SD] age; 62.6 [14.3] years; 8435 women [99.48%]) were analyzed. Metaplastic carcinoma was the most commonly diagnosed histological type in this cohort, with 6867 patients (81%), followed by 1357 (16%) with adenoid cystic carcinoma and only 255 (3%) with medullary carcinoma. Medullary carcinoma presented earlier in life, at a median (interquartile range) age of 53 (45-62) years, compared with 62 (53-72) years for patients with adenoid cystic carcinoma and 63 (52-74) years for patients with metaplastic carcinoma. The proportion of tumors with triple-negative immunohistochemistry varied by histological type for medullary carcinoma (57 patients [22.4%]), adenoid cystic carcinoma (653 patients [48.1%]), and metaplastic carcinoma (3637 patients [53.0%]). Patients with adenoid cystic carcinoma were less likely to receive radiotherapy (711 patients [52.4%]) and chemotherapy (175 patients [12.9%]) compared with patients with medullary carcinoma (radiotherapy, 156 patients [61.2%]; chemotherapy, 190 patients [74.5%]) and metaplastic carcinoma (radiotherapy, 3416 patients [49.7%]; chemotherapy, 4709 patients [68.6%]). The 5-year OS rate was superior for patients with medullary (91.7%) and adenoid cystic carcinoma (88.4%) compared with patients with metaplastic carcinoma (63.1%). The 5-year mortality rate for adenoid cystic carcinoma was 8.33% vs 36.91% for metaplastic carcinoma.

CONCLUSIONS AND RELEVANCE: Nationally, over the course of 7 years, medullary carcinoma was most common and metaplastic carcinoma had the worst 5-year OS among the rare histological breast cancer subtypes analyzed. Factors associated with a poor prognosis for metaplastic carcinoma included advanced stage, lung metastasis, older age, and not receiving chemotherapy or radiation therapy. Future research focusing on rare subtypes of breast cancer is desirable and could inform the optimal management of these relatively understudied carcinomas.

PMID:33844001 | PMC:PMC8042532 | DOI:10.1001/jamanetworkopen.2021.4123

Permalink for 'Immunohistochemical Expression of Fatty Acid Synthase (FASN) is Correlated to Tumor Aggressiveness and Cellular Differentiation in Salivary Gland Carcinomas'

Immunohistochemical Expression of Fatty Acid Synthase (FASN) is Correlated to Tumor Aggressiveness and Cellular Differentiation in Salivary Gland Carcinomas

Fetched: April 13th, 2021, 5:00am GMT by Camila Matsunaga de Angelis

Head Neck Pathol. 2021 Apr 11. doi: 10.1007/s12105-021-01319-3. Online ahead of print.

ABSTRACT

Fatty acid synthase (FASN) expression is closely related to cancer progression, in particular, tumor aggressiveness and poor prognosis. This study aimed to analyse the expression of FASN in carcinomas of the salivary glands and correlate it with Ki-67 expression. We analysed by immunohistochemistry the expression of FASN and Ki-67 on tissue sections from 7 cases of adenocarcinoma, not otherwise specified (AdNOS), 6 cases of polymorphous adenocarcinoma (PAC), 16 cases of acinic cell carcinoma (AcCC), 19 cases of adenoid cystic carcinoma (AdCC), 15 cases of epithelial-myoepithelial carcinoma (EMC); 10 cases of secretory carcinoma (SC), 13 cases of mucoepidermoid carcinoma (MEC), 10 cases of salivary duct carcinoma (SDC) and 7 cases of myoepithelial carcinoma (MC). These carcinomas were classified into aggressive and indolent regarding their biological behaviour. Additionally, MEC and AdCC were also classified according to the histological grade. High expression of FASN was found in SDC (100%), SC (100%), AcCC (68.7%) and AdNOS (57.2%). No association was found between FASN and Ki-67 expression. Aggressive carcinomas showed a higher rate of Ki-67 proliferation (p < 0.001) and greater expression of FASN when compared to indolent carcinomas (p < 0.05). With regards to carcinomas categorized as indolent, FASN expression was much higher in the lesions that presented cell differentiation (SC and AcCC). Also, FASN expression was significantly higher in high-grade AdCC and MEC when compared to low-grade tumors (p < 0.05). We concluded that FASN expression was correlated to tumor aggressiveness and cellular differentiation in salivary gland carcinomas.

PMID:33843033 | DOI:10.1007/s12105-021-01319-3

Potential targets identified in adenoid cystic carcinoma point out new directions for further research

Fetched: April 13th, 2021, 5:00am GMT by Zhenan Liu

Am J Transl Res. 2021 Mar 15;13(3):1085-1108. eCollection 2021.

ABSTRACT

Adenoid cystic carcinoma (AdCC) of the head and neck originates from salivary glands, with high risks of recurrence and metastasis that account for the poor prognosis of patients. The purpose of this research was to identify key genes related to AdCC for further investigation of their diagnostic and prognostic significance. In our study, the AdCC sample datasets GSE36820, GSE59702 and GSE88804 from the Gene Expression Omnibus (GEO) database were used to explore the abnormal coexpression of genes in AdCC compared with their expression in normal tissue. A total of 115 DEGs were obtained by screening with GEO2R and FunRich software. According to functional annotation analysis using Enrichr, these DEGs were mainly enriched in the SOX2, AR, SMAD and MAPK signaling pathways. A protein-protein network of the DEGs was established by the Search Tool for the Retrieval of Interacting Genes (STRING) and annotated through the WEB-based Gene SeT AnaLysis Toolkit (WebGestalt) and was shown to be enriched with proteins involved in cardiac muscle cell proliferation and extracellular matrix organization. A Kyoto Encyclopedia of Genes and Genomes (KEGG) analysis revealed that ITGA9, LAMB1 and BAMBI were associated with the PI3K-Akt and TGF-β pathways. Furthermore, 36 potential target miRNAs were identified by the OncomiR and miRNA Pathway Dictionary Database (miRPathDB). In conclusion, SLC22A3, FOXP2, Cdc42EP3, COL27A1, DUSP1 and HSPB8 played critical roles according to the enrichment analysis; ITGA9, LAMB1 and BAMBI were involved in significant pathways according to the KEGG analysis; ST3Gal4 is a pivotal component of the PPI network of all the DEGs obtained; SPARC, COL4A2 and PRELP were highly related to multiple malignancies in pan-cancer research; hsa-miR-29-3p, hsa-miR-132-3p and hsa-miR-708-5p were potential regulators in AdCC. The involved pathways, biological processes and miRNAs have been shown to play significant roles in the genesis, growth, invasion and metastasis of AdCC. In this study, these identified DEGs were considered to have a potential influence on AdCC but have not been studied in this disease. The analysis results promote our understanding of the molecular mechanisms and biological processes of AdCC, which might be useful for targeted therapy or diagnosis.

PMID:33841642 | PMC:PMC8014416

Stereotactic body radiotherapy for adenoid cystic carcinoma metastatic to the lung: a case report

Fetched: April 13th, 2021, 5:00am GMT by Daijiro Kobayashi

J Med Case Rep. 2021 Apr 11;15(1):156. doi: 10.1186/s13256-021-02781-x.

ABSTRACT

BACKGROUND: Adenoid cystic carcinoma (ACC) is a rare malignant tumor involving mostly the head and neck region, and frequently the salivary glands. The development of lung metastasis after treatment of the primary tumor is a common occurrence in ACC. Although lung metastases show a slow rate of growth, approximately 10% of patients die from distant metastases. The radioresistance of ACC limits the efficacy of conventional radiotherapy for lung metastases, and the optimal dose remains to be determined. Stereotactic body radiotherapy (SBRT) using CyberKnife can deliver a high dose to the lung tumor, while sparing the surrounding normal lung tissues, leading to favorable local control in non-squamous cell lung cancer and metastatic lung tumors. We report a case of lung metastases from ACC treated successfully with SBRT using CyberKnife.

CASE PRESENTATION: A 76-year-old Japanese man with ACC who was treated with carbon ion radiotherapy for a primary oropharynx tumor presented with three metastatic lesions in the lung. The tumor masses were located in the right upper, right lower, and left lower lobes of the lungs. Surgical resection was not indicated because of the presence of multiple tumors. The patient underwent SBRT at 60 Gy in 10 sequential fractions for each tumor. The biologically effective dose based on an alpha/beta ratio of 2 Gy was 240 Gy per tumor. The percentage of the total lung volume irradiated with >20 Gy was 4.9%, 3.2%, and 2.6% for each tumor. The patient developed acute radiation pneumonitis during the initial therapy, which resolved at 6 months after the CyberKnife treatment. At 21 months after the first CyberKnife treatment, three tumors showed no signs of recurrence. No late toxicity was observed.

CONCLUSIONS: SBRT using CyberKnife is an effective and feasible approach to the management of multiple lung metastases of ACC.

PMID:33838695 | PMC:PMC8037832 | DOI:10.1186/s13256-021-02781-x

Permalink for 'Free anterolateral thigh flap with superficial temporal vessels as recipient vessels for reconstruction of defect after maxillectomy in malignant tumor'

Free anterolateral thigh flap with superficial temporal vessels as recipient vessels for reconstruction of defect after maxillectomy in malignant tumor

Fetched: April 10th, 2021, 5:00am GMT by T F Fan

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2021 Apr 7;56(4):369-373. doi: 10.3760/cma.j.cn115330-20200918-00755.

ABSTRACT

Objective: To study the effects of superficial temporal artery and vein as recipient vessels for the free anterolateral thigh flap on the appearance and functions after maxillectomy. Methods: Clinical data of 21 patients with malignant maxillary tumors in Department of Oral and Maxillofacial Surgery, the Second Xiangya Hospital of Central South University from January 2014 to November 2019, who were treated by free anterolateral thigh flap with temporal superficial vessels as the recipient vessels were analyzed retrospectively. There were 18 males and 3 females, with the age ranging from 29 to 73 years old, including 19 cases of squamous carcinoma, 1 case of adenoid cystic carcinoma and 1 case of osteosarcoma. Of those 7 patients underwent primary surgery, 14 patients received resurgery, and 6 patients had a history of postoperative radiotherapy and chemotherapy. Among 14 patients with resurgery, 13 had recurrent ipsilateral second site tumor and 1 had recurrent tumor, and all of them received the maxillectomy and reconstructive surgery with the free anterolateral thigh flap. Patients were evaluated with water swallow test and speech intelligibility score in 1, 3 and 6 months after operation. The data were statistically analyzed with SPSS 22.0 statistical software. Water swallow test results before and after operation were compared using the Wilcoxon rank sum test. The mean speech intelligibility scores before and after operation were compared by the paired t test. Results: Patients were followed up for 10-60 months. All free flaps survived after operation. No diplopia occurred. Breathing, swallowing and speaking functions were normal. No movement disorders caused by the donor of thigh flap. Water swallow test showed no phenomenon of water flowing into the nasal cavity or oral and nasal leakage with level Ⅰ for 4 cases, level Ⅱ for 13 cases, level Ⅲ for 3 cases and level Ⅳ for 1 case. The mean speech intelligibility scores before surgery and 1, 3 and 6 months after surgery were 4.31±0.13, 1.46±0.21, 2.15±0.45 and 2.87±0.76 respectively. There was statistically significant difference in the mean speech intelligibility scores between 1 and 6 months after surgery (F=78.456, P<0.05). Conclusion: It is safe and reliable to use the superficial temporal vessels as recipient vessels for free anterolateral thigh flap in the reconstruction of defect after maxillectomy in malignant tumors, with good outcomes of functions and a satisfactory restoration of outward appearance.

PMID:33832196 | DOI:10.3760/cma.j.cn115330-20200918-00755

Recurrence of ceruminous gland adenoid cystic carcinoma with intracranial dissemination: report of a case

Fetched: April 9th, 2021, 5:00am GMT by Q Y Zheng

Zhonghua Bing Li Xue Za Zhi. 2021 Apr 8;50(4):405-408. doi: 10.3760/cma.j.cn112151-20200707-00538.

NO ABSTRACT

PMID:33832007 | DOI:10.3760/cma.j.cn112151-20200707-00538

Adenomyoepithelioma of the breast: A proposal for classification

Fetched: April 9th, 2021, 5:00am GMT by Emad Rakha

Histopathology. 2021 Apr 7. doi: 10.1111/his.14380. Online ahead of print.

ABSTRACT

Breast lesions with a prominent myoepithelial cell component constitute a heterogeneous group of benign and malignant neoplastic proliferations. These lesions are often dual epithelial-myoepithelial but may be purely myoepithelial cell in nature. Benign epithelial-myoepithelial lesions typically maintain the morphology and immunophenotype of the normal bilayer epithelial myoepithelial structures. However, the distinction between the two cell components is not always clear-cut in malignant lesions in which the histogenesis of myoepithelial cells remains uncertain. Neoplastic biphasic epithelial-myoepithelial lesions of the breast include adenomyoepithelioma (AME), pleomorphic adenoma and adenoid cystic carcinoma. Four histological patterns of classical AME have been described: tubular, lobulated, spindle cell and adenosis variants. Overlapping patterns occur and some AMEs display an intraductal papillary pattern that may represent a fifth variant. AME can be benign or malignant. Classical AME may show atypical features, which are not sufficient for the diagnosis of malignancy (atypical AME). Atypical AME is recognised as a lesion of uncertain malignant potential with limited metastatic capability. Based on the histological features, we propose a classification of malignant AME (M-AME) into three variants: M-AME in situ, M-AME invasive and AME with invasive carcinoma. In this review, we provide an overview of myoepithelial lesions of the breast focusing on the classification of AME to improve not only the consistency of reporting but also help guide further management decision making.

PMID:33829532 | DOI:10.1111/his.14380

Symptoms and signs of parotid tumors and their value for diagnosis and prognosis: a 20-year review at a single institution

Fetched: April 8th, 2021, 5:00am GMT by Yuko Inaka

Int J Clin Oncol. 2021 Apr 7. doi: 10.1007/s10147-021-01901-3. Online ahead of print.

ABSTRACT

BACKGROUND: Detailed clinical features other than parotid mass have not been investigated in detail for parotid tumors. Symptoms and signs are useful for the differentiation of benign versus malignant, and may also be of value to determine the grade of malignancy and histological type as well as the assessment of prognosis.

METHODS: We reviewed symptoms and signs of 965 patients with benign tumors and 200 patients with malignant tumors. Symptoms and signs included pain/tenderness, adhesion to surrounding tissues, and facial nerve palsy. We reviewed the incidence in benign and malignant tumors, in histological type of benign tumors, and in grade and histology of malignant tumors. For each symptom or sign, covariates were analyzed, and their correlation with the prognosis was investigated.

RESULTS: The incidence of symptoms and signs was significantly higher in malignant than benign tumors, and more frequent in higher grade of malignancy. Facial nerve palsy was observed in 18.0% of malignant tumor cases, while none occurred in benign tumor cases. Pain/tenderness was more commonly observed in adenoid cystic carcinoma, while adhesion to surrounding tissues and facial nerve palsy were most frequently noted in salivary duct carcinoma. The prognosis of patients with these symptoms and signs was significantly poor.

CONCLUSION: A detailed investigation of symptoms and signs in parotid gland tumors is the first step that leads to the diagnosis of malignant tumors. Symptoms and signs are also useful for estimating the grade of malignancy and histological type, and they are important information for predicting prognosis.

PMID:33826026 | DOI:10.1007/s10147-021-01901-3

High-grade Transformation/Dedifferentiation in Salivary Gland Carcinomas: Occurrence Across Subtypes and Clinical Significance

Fetched: April 8th, 2021, 5:00am GMT by Alena Skalova

Adv Anat Pathol. 2021 May 1;28(3):107-118. doi: 10.1097/PAP.0000000000000298.

ABSTRACT

PMID:33825717 | DOI:10.1097/PAP.0000000000000298

Permalink for 'The immunophenotype of epithelial to mesenchymal transition inducing transcription factors in salivary gland adenoid cystic carcinomas'

The immunophenotype of epithelial to mesenchymal transition inducing transcription factors in salivary gland adenoid cystic carcinomas

Fetched: April 6th, 2021, 5:00am GMT by Iulia Cristiana Belulescu

Rom J Morphol Embryol. 2020 Jul-Sep;61(3):769-782. doi: 10.47162/RJME.61.3.15.

ABSTRACT

Adenoid cystic carcinoma (ACC) is the second most common malignant salivary glands neoplasms with a controversial biological behavior. Even though these tumors grow slowly, they have increased potential for recurrence and distant metastasis. In order to elucidate this behavior, our study aimed to investigate the immunoexpression in such tumors of the most important transcriptional factors [Twist, Snail, Slug, and zinc finger E-box binding homeobox 1 (ZEB1)] involved in the epithelial-mesenchymal transition process. The highest level of expression was recorded for Twist, present in all the investigated cases, followed by the Slug and Snail, while no tumor parenchyma reactivity was noticed for the ZEB1 factor. There were tumor reactivity differences regarding topography, histopathological variant, and nerve and lymph node invasion status. Thus, tumors developed from the intraoral minor salivary glands, with solid pattern, perineural invasion, locally aggressive and with lymph node metastasis were the most reactive. Therefore, these transcription factors could be useful as prognostic biomarkers and efficient therapeutic targets in such salivary malignancies.

PMID:33817718 | PMC:PMC8112789 | DOI:10.47162/RJME.61.3.15

Case Report: Efficacy of Pyrotinib in ERBB2 Amplification Pulmonary Adenoid Cystic Carcinoma

Fetched: April 6th, 2021, 5:00am GMT by Zhongben Tang

Front Oncol. 2021 Mar 18;11:605658. doi: 10.3389/fonc.2021.605658. eCollection 2021.

ABSTRACT

Primary pulmonary adenoid cystic carcinomas are salivary tumors that are low-grade malignant and prone to recurrence and metastasis. Surgery is currently the main treatment, but there is no standard with regard to postoperative adjuvant therapy. Adenoid cystic carcinoma is more sensitive to radiotherapy and patients benefit less from chemotherapy, but few studies have focused on targeted therapy, and their conclusions are inconsistent. With respect to primary pulmonary adenoid cystic carcinoma, large-scale studies cannot be conducted due to its low incidence, and studies on the targeted therapy of it are very scarce. A few case reports indicate that targeted therapy can be effective however, suggesting that it may be a good option. The current report is the first on the occurrence of human epidermal growth factor receptor 2 amplification in pulmonary adenoid cystic carcinoma. The patient was treated with pyrotinib for 6 months and achieved stable disease.

PMID:33816237 | PMC:PMC8012760 | DOI:10.3389/fonc.2021.605658

Mast Cells and Proteins Related to Myofibroblast Differentiation (PAR-2, IL-6, and TGFβ1) in Salivary Cancers: A Preliminary Study

Fetched: April 3rd, 2021, 5:00am GMT by Adna B Ismerim

Appl Immunohistochem Mol Morphol. 2021 Apr 1. doi: 10.1097/PAI.0000000000000924. Online ahead of print.

ABSTRACT

Salivary gland neoplasms represent an important group of cancers in the head and neck and myoepithelial cells play a key role on the development these tumors. This study evaluated the distribution of mast cells and related proteins (PAR-2, TGFβ1, IL-6) to the myofibroblastic differentiation in malignant tumors of salivary glands with and without myoepithelial differentiation. Immunohistochemical assessement for tryptase mast cells, SMA, PAR-2, TGFβ1, IL-6 was performed in 10 cases of polymorphous low-grade adenocarcinoma, 14 cases of mucoepidermoid carcinoma (MEC) and 10 cases of adenoid cystic carcinoma. When the density of mast cells were compared between tumors, their density was significantly higher in MEC (P=0.08). Tumors with high expression of PAR-2 (79.4%) exhibited a high density of mast cells. Myofibroblasts were more frequent in malignant tumors with low expression (<50%) of cell masts. Individual analysis of the tumors showed no significant difference between the expression of PAR-2, IL-6, TGFβ1, and myofibroblasts. When the density of mast cells, myofibroblasts and the expression of PAR-2 protein, IL-6, and TGFβ1 were compared, it was no statistically significant difference between tumors with and without myoepithelial differentiation. The results of present study suggest a possible participation of mast cells and especially of PAR-2 in the development and progression of malignant salivary cancers, regardless of myoepithelial content.

PMID:33797435 | DOI:10.1097/PAI.0000000000000924

A Case of Neglected, Recurrent Adenoid Cystic Carcinoma of Parotid Gland

Fetched: April 3rd, 2021, 5:00am GMT by Adeline Low

Ophthalmic Plast Reconstr Surg. 2021 Mar 31. doi: 10.1097/IOP.0000000000001947. Online ahead of print.

NO ABSTRACT

PMID:33795606 | DOI:10.1097/IOP.0000000000001947

Basaloid Squamous Cell Carcinoma of the Uterine Cervix: Report of a Case With Molecular Analysis

Fetched: April 2nd, 2021, 5:00am GMT by Jijgee Munkhdelger

Int J Surg Pathol. 2021 Apr 1:1066896921997132. doi: 10.1177/1066896921997132. Online ahead of print.

ABSTRACT

There is a lack of knowledge about molecular alterations in basaloid squamous cell carcinoma (BSCC) of the uterine cervix. A 72-year-old woman with a history of previous subtotal hysterectomy and current vaginal bleeding was referred to our hospital. Initially, adenoid cystic carcinoma (ACC) was diagnosed upon cervical cytology and biopsy. Chest imaging showed multiple metastatic lesions in both lungs. The surgical specimen showed BSCC with diffuse p16 immunoreactivity and negativity for S-100, c-kit, and neuroendocrine markers. There was a focal minor ACC component, which could have explained the previous cytology and biopsy diagnosis. Next-generation sequencing with two different panels showed coexisting PIK3CA mutation and NTRK2 fusion with 10 additional variants of unknown significance (ATR, DAXX, FAM123B, JAK1, KEL, MLL2, NOTCH2, PALB2, POLD1, POLE). The MYB gene fusions were not identified. The patient received chemotherapy with TRK inhibitor larotrectinib and carboplatin, which caused shrinkage of metastatic lung nodules. This is the first report of cervical BSCC with extensive molecular workup, which detected multiple genetic events, including targetable ones, which are potentially implicated in the development of a tumor. The accumulation of data and further studies on this tumor are necessary to define its diagnostic criteria and its clinical and biological behavior.

PMID:33792431 | DOI:10.1177/1066896921997132

Management of Adenoid Cystic Carcinoma of the Breast: A Single-Institution Study

Fetched: April 2nd, 2021, 5:00am GMT by Wenxiang Zhang

Front Oncol. 2021 Mar 15;11:621012. doi: 10.3389/fonc.2021.621012. eCollection 2021.

ABSTRACT

OBJECTIVE: The purpose of our study was to analyze the clinicopathologic features and surgical and oncological outcomes of adenoid cystic carcinoma (ACC) of the breast and to provide the basis for a clinical therapeutic schedule.

METHODS: A total of 14 patients with primary breast adenoid cystic carcinoma treated at Cancer Hospital of the Chinese Academy of Medical Sciences from January 2000 to December 2017 were included. Data on clinical presentation, treatment strategy, and outcome, as well as the pathological features of ACC, were reviewed and analyzed.

RESULTS: Fourteen patients were diagnosed with ACC of the breast, out of 23205 total patients treated for breast cancer (0.06%). All but three patients were postmenopausal, with a median age at diagnosis of 60.5 years (range, 39-73 years). The most common clinical presentation was a palpable mass (85.7%), and the imaging characteristics of all patients on color Doppler ultrasound and mammography were nonspecific. Six patients (42.9%) were suspected of having ACC by fine-needle aspiration cytology (FNAC) and were confirmed by postoperative histology and immunohistochemistry. All 14 patients underwent surgery, and no patient had a positive lymph node status. Median tumor size was 1.75 cm (range, 1-3 cm). Eight/14 (57.1%) patients were hormone receptor negative (HR-) and HER-2/neu (-) (HER2-). The remaining patients were hormone receptor positive (HR+). There was no significant difference in clinicopathological characteristics between the HR+ group and the HR- group (P>0.05). The mean follow-up period was 57 months. Local recurrence occurred in 14.3% of patients, 1.7% of patients had distant metastasis, all patients with local recurrence or distant metastasis were in the HR (-) group, and all patients were alive at the last follow-up.

CONCLUSION: ACC of the breast cannot be simply summarized as triple-negative breast cancer because it also includes a small number of hormone receptor-positive breast cancers. Establishing a preoperative diagnosis is difficult on the basis of clinical imaging examination, FNAC may be useful tool in the diagnosis. the final diagnosis can only be assessed based on the results of the histopathological and immunohistochemical examination. Breast-conserving surgery may be an alternative treatment strategy, and axillary lymph node dissection or sentinel node biopsy may not be necessary in some cases.

PMID:33791208 | PMC:PMC8005703 | DOI:10.3389/fonc.2021.621012

Permalink for 'Establishment of patient-derived xenograft models of adenoid cystic carcinoma to assess pre-clinical efficacy of combination therapy of a PI3K inhibitor and retinoic acid'

Establishment of patient-derived xenograft models of adenoid cystic carcinoma to assess pre-clinical efficacy of combination therapy of a PI3K inhibitor and retinoic acid

Fetched: April 2nd, 2021, 5:00am GMT by Bao Sun

Am J Cancer Res. 2021 Mar 1;11(3):773-792. eCollection 2021.

ABSTRACT

Due to the difficulties and long periods of establishment, preclinical animal models of adenoid cystic carcinoma (ACC) are scarce but imperative. The researches involving molecular features and therapeutic targets of ACC require an integrated group of preclinical animal models which can credibly retain the heterogeneity of this tumor. Currently chemotherapies and targeting therapies have modest efficacy in ACC and the overall response rate is rather low. Therefore, novel therapeutic regimen of ACC is urgently needed and remains a major clinical challenge. We transplanted a group of tumor samples from human salivary ACC into immunodeficient mice to establish patient-derived xenografts (PDXs). Patient tumors and their matched PDXs were conducted histological analyses, whole-exome sequencing (WES) and RNA-seq respectively. 13 PDXs were successfully established from 34 ACC, involved in 3 histological types, including cribriform, tubular, and solid. These ACC PDXs generally reflected the histopathological and molecular features of their corresponding original tumors. MYB/MYBL1-NFIB fusion (53.85%) and high-frequency mutation genes, such as KDM6A, KMT2C, KMT2D, NOTCH1, NOTCH2, SMARCA4 and PIK3CA were mainly conserved in PDXs. Guided by the genetic alterations, the efficiencies of retinoic acid (RA) and a PI3K inhibitor were evaluated in ACC PDX models harboring both MYB fusion and PIK3CA amplification/mutation. Combination treatment of the PI3K inhibitor and RA demonstrated remarkable inhibition of tumors in PDXs harboring both PIK3CA mutation/amplification and MYB-NFIB fusion gene in vivo and in vitro. In this study, we displayed the morphologically and genetic featured PDXs which recapitulated the heterogeneity of original ACC tumors, indicating that the models could be used as a platform for drug screening for therapy response. The feasibility of combination treatment approaches for dual targets were confirmed, providing new regimens for personalized therapies in ACC.

PMID:33791153 | PMC:PMC7994170

Radiotherapy as Part of Treatment Strategies in Nasal Cavity and Paranasal Sinus Malignancies

Fetched: April 1st, 2021, 5:00am GMT by Nora Owin

Anticancer Res. 2021 Mar;41(3):1587-1592. doi: 10.21873/anticanres.14919.

ABSTRACT

BACKGROUND/AIM: Modern intensity-modulated radiotherapy (IMRT) is frequently applied to treat patients with nasal cavity and paranasal sinus (NC/PNS) malignancies.

PATIENTS AND METHODS: One hundred and four patients who underwent radiotherapy (RT) between 1994 and 2020 were recognized. This analysis compared conventional-radiotherapy (CRT) and image-guided IMRT outcomes for NC/PNS malignancies.

RESULTS: The median follow-up was 69 months. Eighty-eight patients (85%) were managed with image-guided IMRT. The median initial radiation dose was 65 Gy, with 68 Gy applied for patients treated with primary RT versus 63 Gy applied for adjuvant therapy (p=0.1). The 5-year locoregional control (LRC) was 85%. The locoregional recurrence rate was 18% following IMRT versus 31% in the 2D/3D-conventional RT group (p=0.09). Moreover, IMRT was associated with a lower inner-ear toxicity rate (8% vs. 20%, respectively; p=0.045).

CONCLUSION: IMRT appears to be linked with higher LRC and lower inner-ear acute toxicities compared to conventional RT.

PMID:33788753 | DOI:10.21873/anticanres.14919

Masses of the Lacrimal Gland: Evaluation and Treatment

Fetched: March 30th, 2021, 5:00am GMT by Jane S Kim

J Neurol Surg B Skull Base. 2021 Feb;82(1):100-106. doi: 10.1055/s-0040-1722700. Epub 2021 Feb 18.

ABSTRACT

Lacrimal gland lesions account for approximately 9 to 10% of all biopsied orbital masses. Potential causes include nongranulomatous and granulomatous inflammation, autoimmune disease, lymphoproliferative disorders, benign epithelial proliferation, malignant neoplasia, and metastatic disease. Inflammatory lesions and lymphoproliferative disorders are the most common and may be unilateral or bilateral; they may also be localized to the orbit or associated with systemic disease. Both benign and malignant epithelial lacrimal gland masses tend to be unilateral and involve the orbital lobe, but a more rapid onset of symptoms and periorbital pain strongly suggest malignant disease. On orbital imaging, both inflammatory and lymphoproliferative lesions conform to the globe and surrounding structures, without changes in adjacent bone, whereas epithelial lacrimal gland masses often show scalloping of the lacrimal gland fossa. Malignant epithelial lacrimal gland tumors can also have radiographic evidence of bony invasion and destruction. Masses of the lacrimal gland may be due to a broad range of pathologies, and a good working knowledge of common clinical characteristics and radiographic imaging findings is essential for diagnosis and treatment. All patients with inflammatory, lymphoproliferative, and epithelial neoplastic lesions involving the lacrimal gland require long-term surveillance for disease recurrence and progression.

PMID:33777623 | PMC:PMC7987400 | DOI:10.1055/s-0040-1722700

Primary Malignant Orbital Tumors

Fetched: March 30th, 2021, 5:00am GMT by Jacquelyn Laplant

J Neurol Surg B Skull Base. 2021 Feb;82(1):81-90. doi: 10.1055/s-0040-1722635. Epub 2021 Feb 18.

ABSTRACT

Objective Primary orbital malignancy is rare. Awareness of the characteristic clinical and imaging features is imperative for timely identification and management. Surgery remains an important diagnostic and treatment modality for primary orbital malignancy, but determining the optimal surgical approach can be challenging. The purpose of this article is to explore recent advances in the diagnosis, management, and surgical approaches for primary orbital malignancies. Design In this review, the clinical presentation, imaging features, and medical and surgical management of primary orbital malignancies with representative cases will be discussed. Setting Outpatient and inpatient hospital settings. Participants Patients with diagnosed primary orbital malignancies. Main Outcome Measures Descriptive outcomes. Results Advancements in orbital imaging, microsurgical techniques, and multimodal therapy have improved the diagnosis and management of primary orbital malignancies. Special considerations for biopsy or resection are made based on the tumor's location, characteristics, nearby orbital structures, and goals of surgery. Minimally invasive techniques are supplanting traditional approaches to orbital surgery with less morbidity. Conclusions Advances in imaging technologies and surgical techniques have facilitated the diagnosis and management of primary orbital malignancies. Evolution toward less invasive orbital surgery with focus on preservation and restoration of function is underway.

PMID:33777620 | PMC:PMC7987380 | DOI:10.1055/s-0040-1722635

Successful Treatment of Adenoid Cystic Carcinoma with the Application of a High-Dose Stereotactic Body Radiotherapy Boost

Fetched: March 30th, 2021, 5:00am GMT by Paweł Polanowski

Case Rep Oncol. 2021 Mar 9;14(1):371-377. doi: 10.1159/000512069. eCollection 2021 Jan-Apr.

ABSTRACT

BACKGROUND: Adenoid cystic carcinoma (ACC) should be treated with a surgical procedure. Unfortunately, in some cases, such procedures are impossible to perform. In that event, radiotherapy can be used as a form of radical treatment, although ACC is established as a radio- and chemoresistant tumour. Therefore, unconventional fractionated radiotherapy needs to be considered.

CASE PRESENTATION: Here, we present a case study of a patient with an unresectable tumour of the choanae and nasopharynx treated with a stereotactic radiotherapy boost in combination with conventional radiotherapy. We achieved complete clinical regression after application of a 1 × 18 Gy boost followed by conventional radiotherapy at 50 Gy in 25 fractions. The early and late tolerance of this treatment were positive. During the 2-year follow-up, local and distant recurrence were not detected.

CONCLUSIONS: This case represents an individualized, modern and safe approach to unresectable ACC. This is one of the first cases to show the use of a combination of stereotactic and conventional radiotherapy in radical, conservative cancer treatment.

PMID:33776731 | PMC:PMC7983606 | DOI:10.1159/000512069

A Case of Solid Variant of Adenoid Cystic Carcinoma from Trachea: A Case Report and Review of Literature

Fetched: March 30th, 2021, 5:00am GMT by Feng Gao

Onco Targets Ther. 2021 Mar 18;14:1997-2002. doi: 10.2147/OTT.S296400. eCollection 2021.

ABSTRACT

INTRODUCTION: Primary tracheal adenoid cystic carcinoma (ACC) is a rare and heterogeneous group of neoplasms arising from the respiratory tract. The solid variant of ACC is a histologically distinct subtype with an unfavorable clinical course. We report on a case of tracheal ACC with immunohistochemical and molecular analysis together with a review of the literature.

CASE REPORT: We observed a case in which a 31-year-old male presented with a neoplasm bulging into the lumen and caused symptoms of tracheal obstruction and even hemoptysis. Cytological smears of an endobronchial fine needle aspiration revealed aggregates of basaloid cells with small to medium size, scant cytoplasm, and evenly hyperchromatic nuclei. Histologically, the tumor is characterized by a predominant compact sheet-like and nested pattern of rounded basaloid cells. Immunohistochemically, the tumor was diffusely positive for CK and CD117. CK7 and CK5/6 were focally positive in the genuine glandular structures. P63 was completely negative in the majority of neoplastic cells. Fluorescence in situ hybridization analysis revealed MYB gene rearrangement.

CONCLUSION: The solid variant of ACC from trachea is rare and hence poses diagnostic difficulty. Computed tomography (CT) scan and bronchoscopy help assess the extent of the disease. Histological features combined with immunophenotypic and molecular analysis aid in distinguishing this uncommon type from other round cell neoplasms. The accurate diagnosis can help expedite treatment of this highly aggressive tumor.

PMID:33776449 | PMC:PMC7987321 | DOI:10.2147/OTT.S296400

Permalink for 'Outcome of Adenoid Cystic Carcinoma of Head and Neck After Postoperative Intensity Modulation Radiotherapy: A Single Institution Study'

Outcome of Adenoid Cystic Carcinoma of Head and Neck After Postoperative Intensity Modulation Radiotherapy: A Single Institution Study

Fetched: March 25th, 2021, 5:00am GMT by Peng Xu

Cancer Manag Res. 2021 Mar 15;13:2411-2417. doi: 10.2147/CMAR.S283494. eCollection 2021.

ABSTRACT

OBJECTIVE: This study was retrospectively evaluated the outcome of postoperative intensity modulation radiotherapy (IMRT) for patients with adenoid cystic carcinoma of head and neck (ACCHN), and identified the unfavorable prognostic factors.

METHODS: Fifty-five ACCHN patients treated in Sichuan Cancer Hospital between January 2007 and December 2016 were retrospectively evaluated. Median age of patient was 47 years (range, 21-73 years). Thirty-five patients were male and 20 were female. In 30 patients, tumors were located in major salivary glands (54.5%), and 25 patients in minor salivary glands (45.5%). The numbers of R0, R1, and R2 surgical resection classification patients were 22 (40.0%), 20 (36.4%), and 13 (23.6%). The median total RT dose was 62 Gy (range, 46-72 Gy), and 54.5% of patients were treated with adjuvant chemotherapy. Statistical analyses were performed using the Log rank test for univariate analysis and the Cox proportional hazard model for multivariate analysis.

RESULTS: Median follow-up period was 68.5 months (12-132 months). The 5-year local-regional recurrence-free survival (LRRFS), distant metastasis-free survival (DMFS) and overall survival (OS) were 93.9%, 75.3% and 82.5%. In univariate analysis, T stages (p=0.025) and AJCC stages (p=0.036) were the prognostic factors for OS; Age (p=0.042), T stages (p=0.025), N stages (p=0.021), AJCC stages (p=0.021) and adjuvant chemotherapy (p=0.010) were the prognostic factors for DMFS; T stage (p=0.049) was the prognostic factor for LRRFS. In multivariate factors analyses, T stages (p=0.026), AJCC stages (p=0.028) and RT dose (p=0.025) were the significant prognosticators for OS. The most common acute toxicities over three degrees were myelosuppression (5.5%), mucositis (9.1%) and dermatitis (1.8%).

CONCLUSION: Postoperative IMRT seems to achieve reasonable local-regional control and OS in patients with adenoid cystic carcinoma of head and neck, with acceptable treatment relative toxicities.

PMID:33758540 | PMC:PMC7979337 | DOI:10.2147/CMAR.S283494

Permalink for 'Successful management of adenoid cystic carcinoma of the lacrimal sac with apatinib combined with concurrent chemoradiotherapy: a case report'

Successful management of adenoid cystic carcinoma of the lacrimal sac with apatinib combined with concurrent chemoradiotherapy: a case report

Fetched: March 24th, 2021, 5:00am GMT by Shuyuan Zhang

Ann Palliat Med. 2021 Mar 12:apm-20-1934. doi: 10.21037/apm-20-1934. Online ahead of print.

ABSTRACT

Adenoid cystic carcinoma (ACC) of the lacrimal sac is an extremely rare disease with a poor prognosis. There is currently no standard treatment for this malignancy. Radical surgical excision followed by radiotherapy and chemotherapy is the preferred treatment for localized ACC of the lacrimal sac. Apatinib has shown efficacy in recurrent/metastatic ACC. The role of apatinib in combination with concurrent chemoradiotherapy in patients with non-operated localized advanced ACC is not clear. Here, we report a 30-year-old man with a 1-year history of epiphora of the left eye and nasal congestion who was diagnosed as ACC of the lacrimal sac. The computed tomography (CT) scan showed the tumor invading the left orbit and left maxillary sinus. He refused surgical resection and instead received combined apatinib and nedaplatinbased concurrent chemoradiotherapy. He experienced moderate adverse effects such as nausea, hypertension, myelosuppression that were well controlled after symptomatic supportive care. Complete response was observed 3 weeks following the treatment. The patient remained free of disease until the last follow up by December 2020, 22 months after the treatment. This case suggested that combined apatinib and concurrent chemoradiotherapy might be an option for locally advanced ACC patients who are ineligible for or rejective to surgical resection.

PMID:33752415 | DOI:10.21037/apm-20-1934

Adenoid cystic carcinoma of the palpebral lobe of the lacrimal gland - case report and literature review

Fetched: March 23rd, 2021, 5:00am GMT by Ana Filipa Duarte

Orbit. 2021 Mar 22:1-6. doi: 10.1080/01676830.2021.1901293. Online ahead of print.

ABSTRACT

Epithelial tumors of the lacrimal gland are rare and usually develop in the orbital lobe. We report the exceedingly rare occurrence of a primary adenoid cystic carcinoma in the palpebral lobe of the lacrimal gland. A 26-year-old female was referred for evaluation of a gradually enlarging mass in the lateral upper eyelid, previously diagnosed as a chalazion. Computed tomography revealed a heterogeneous round lesion anterior to the orbital rim. Excisional biopsy was compatible with an adenoid cystic carcinoma. After excluding distant metastasis, and as the patient refused adjuvant radiotherapy, a second surgical procedure, with wide local excision, was indicated. Follow-up showed no recurrence. This case highlights the importance of performing a thorough clinical examination when diagnosing any lateral upper eyelid mass. A high index of suspicion for malignant tumors of the lacrimal gland should always be maintained, and a complete excision with histological analysis should be preferred whenever possible.

PMID:33749533 | DOI:10.1080/01676830.2021.1901293

Permalink for 'Bioinformatics identification of the candidate microRNAs and construction of a competing endogenous RNA regulatory network in lacrimal gland adenoid cystic carcinoma high-grade transformation'

Bioinformatics identification of the candidate microRNAs and construction of a competing endogenous RNA regulatory network in lacrimal gland adenoid cystic carcinoma high-grade transformation

Fetched: March 23rd, 2021, 5:00am GMT by Meixia Jiang

Oncol Lett. 2021 May;21(5):360. doi: 10.3892/ol.2021.12621. Epub 2021 Mar 8.

ABSTRACT

Adenoid cystic carcinoma of the lacrimal gland (LACC) is a major orbital malignancy. The recurrence rate and mortality rate are higher in LACC high-grade transformation (LACC-HGT) compared with in LACC. The present study aimed to identify the candidate microRNAs (miRNAs/miRs) and construct a competing endogenous RNA (ceRNA) regulatory network for LACC-HGT. A miRNA microarray on paraffin-embedded tissues was performed to identify the differentially expressed miRNAs (DEMs) of LACC-HGT. The overlap with the salivary adenoid cystic carcinoma miRNA/RNA sequencing dataset in the Gene Expression Omnibus was used to identify candidate miRNAs. In order to construct a ceRNA regulatory network of LACC-HGT, a microarray of mRNA and circRNA in primary cell lines was performed. The circRNAs and genes with high expression in LACC-HGT were predicted as targeting miRNAs, and the circRNA-miRNA-mRNA regulatory network was constructed. miR-140-3p was identified as part of the ceRNA network and as a candidate miRNA, therefore this was further analyzed using reverse transcription-quantitative (RT-q)PCR. Overall, the Agilent Human microarray analysis identified a total of 16 DEMs from the LACC-HGT paraffin-embedded tissues. A total of 653 DECs and 9,566 DEGs of LACC-HGT primary cell lines were screened via the microarray of mRNA and circRNA. The ceRNA regulatory network was constructed using the cross-binding of circRNA-miRNA, miRNA-mRNA and the downregulated miRNAs in LACC-HGT to clearly demonstrate the circRNA-miRNA-mRNA interaction relationship. RT-qPCR results confirmed that miR-140-3p was downregulated in LACC-HGT tissues and primary cell lines compared with LACC. Target genes CD200 and parathyroid hormone-related protein were significantly upregulated in LACC-HGT primary cell lines. miR-140-3p and its target genes may play an important role in LACC-HGT pathogenesis. In conclusion, the current bioinformatics study constructed a ceRNA network based on a microarray, which may help identify novel miRNA therapeutic targets for LACC-HGT.

PMID:33747217 | PMC:PMC7967933 | DOI:10.3892/ol.2021.12621

Recent updates in salivary gland tumors of the lung

Fetched: March 22nd, 2021, 5:00am GMT by Anja C Roden

Semin Diagn Pathol. 2021 Mar 11:S0740-2570(21)00011-3. doi: 10.1053/j.semdp.2021.03.001. Online ahead of print.

ABSTRACT

Salivary gland tumors are uncommon primary lesions in the lung. Their morphologic, immunophenotypic, and molecular characteristics resemble those of their counterparts in the head and neck or elsewhere. Most common primary pulmonary salivary gland tumors include mucoepidermoid carcinoma, adenoid cystic carcinoma, and epithelial-myoepithelial carcinoma. The study of these neoplasms is hampered by their paucity. Therefore, studies are in general small or restricted to individual cases. Despite this challenge recent advances have been made specifically at the molecular level. Molecular alterations such as MAML2 rearrangements in mucoepidermoid carcinoma, MYB rearrangements in adenoid cystic carcinomas, and EWSR1 rearrangements in hyalinizing clear cell carcinomas and myoepithelial tumors have been identified. These molecular alterations might be helpful in the distinction of these salivary gland tumors from other neoplasms in the lung. However, the distinction from metastatic disease remains challenging. Awareness of these tumors and knowledge of available ancillary studies to confirm the diagnosis is important to avoid misdiagnosis which might lead to differences in treatment, management, and prognosis. Further studies are needed to identify biomarkers to better predict patient's outcome and for individual management and treatment of patients.

PMID:33744018 | DOI:10.1053/j.semdp.2021.03.001

Assessment Of Tumour Infiltrating Lymphocytes And Pd-l1 Expression In Adenoid Cystic Carcinoma Of The Salivary Gland

Fetched: March 22nd, 2021, 5:00am GMT by Wenqian Chen

Clin Invest Med. 2021 Mar 21;44(1):E38-41. doi: 10.25011/cim.v44i1.35218.

ABSTRACT

PURPOSE: Early phase clinical studies are ongoing to evaluate the role of immune checkpoint inhibitors in adenoid cystic carcinoma (ACC) despite a paucity of information on the immune microenvironment. This study aims to better characterize the immune microenvironment of ACC tumours and evaluate survival outcomes based on tumour infiltrating lymphocyte (TIL) and programmed death-ligand 1 (PD-L1) expression.

METHODS: Patient characteristics, treatment and outcome data were collected for 24 ACC patients. The CD8+(cluster of differentiation 8) TIL and PD-L1 expression were quantified by immunohistochemistry. Marker expression and survival outcomes were evaluated by Kaplan-Meier analysis.

RESULTS: All cases were negative for PD-L1 expression; four cases had focal high, eight cases had focal moderate and 12 cases had low TIL expression. Based on TIL expression, there was no difference in disease-free or overall survival.

CONCLUSION: Adenoid cystic carcinoma tumours were found to be associated with a poor immunogenic microenvironment, with absent PD-L1 expression and low CD8+ TILs. There was no association between TIL expression and survival. These data suggest that PD-L1 and TIL expression are unlikely to be useful as predictive biomarkers for response to immunotherapy.

PMID:33743575 | DOI:10.25011/cim.v44i1.35218

A Drosophila platform identifies a novel, personalized therapy for a patient with adenoid cystic carcinoma

Fetched: March 19th, 2021, 5:00am GMT by Erdem Bangi

iScience. 2021 Feb 20;24(3):102212. doi: 10.1016/j.isci.2021.102212. eCollection 2021 Mar 19.

ABSTRACT

Adenoid cystic carcinoma (ACC) is a rare cancer type that originates in the salivary glands. Tumors commonly invade along nerve tracks in the head and neck, making surgery challenging. Follow-up treatments for recurrence or metastasis including chemotherapy and targeted therapies have shown limited efficacy, emphasizing the need for new therapies. Here, we report a Drosophila-based therapeutic approach for a patient with advanced ACC disease. A patient-specific Drosophila transgenic line was developed to model the five major variants associated with the patient's disease. Robotics-based screening identified a three-drug cocktail-vorinostat, pindolol, tofacitinib-that rescued transgene-mediated lethality in the Drosophila patient-specific line. Patient treatment led to a sustained stabilization and a partial metabolic response of 12 months. Subsequent resistance was associated with new genomic amplifications and deletions. Given the lack of options for patients with ACC, our data suggest that this approach may prove useful for identifying novel therapeutic candidates.

PMID:33733072 | PMC:PMC7940980 | DOI:10.1016/j.isci.2021.102212

Permalink for 'Case Report: Biologic graft placement with subsequent radiation therapy following radical vulvectomy for adenoid cystic carcinoma of the Bartholin's gland'

Case Report: Biologic graft placement with subsequent radiation therapy following radical vulvectomy for adenoid cystic carcinoma of the Bartholin's gland

Fetched: March 19th, 2021, 5:00am GMT by Keely Ulmer

Gynecol Oncol Rep. 2021 Feb 27;36:100736. doi: 10.1016/j.gore.2021.100736. eCollection 2021 May.

ABSTRACT

Adenoid cystic carcinoma (ACC) of the Bartholins gland, first described by Klob in 1864, is a rare form of vulvar cancer comprising approximately 2-7% of all invasive vulvar lesions (Cardosi, 2001). Treatment consists of excision followed by radiation therapy (Cardosi, 2001; Anaf, 1999; Barcellini, 2020). Progression is indolent with later recurrence and metastases in comparison to other forms of vulvar cancer (Yang, 2006). Resection remains the gold standard for treatment followed by radiation therapy if margins are positive (Cardosi, 2001; Yang, 2006; Chang et al., 2019). We present a case of ACC of the Bartholins gland that underwent radical vulvectomy and Surgisis graft placement due to the extent of disease resection. Radiation therapy was then pursued due to positive margins with no wound breakdown despite this being the most common complication of vulvectomy with or without radiation therapy (Leminen et al., 2000). To our knowledge this is only the second case of Cook Biodesign graft placement after vulvectomy and first case of subsequent local radiation therapy to the area.

PMID:33732851 | PMC:PMC7937557 | DOI:10.1016/j.gore.2021.100736

Carcinoma Expleomorphic Adenoma of the Lacrimal Gland

Fetched: March 13th, 2021, 5:00am GMT by Kaveh Vahdani

Ophthalmic Plast Reconstr Surg. 2021 Mar 8. doi: 10.1097/IOP.0000000000001957. Online ahead of print.

ABSTRACT

AIMS: To report the characteristics and long-term outcomes of patients presenting with carcinoma ex-pleomorphic adenoma (CXPA) or malignant mixed tumor of the lacrimal gland.

METHODS: A retrospective case-note review for patients with CXPA, seen at Moorfields Eye Hospital between 1985 and 2018, was performed for demographics, presentation, imaging, histopathology, management, and outcome.

RESULTS: Twenty-six patients (11 male; 42%) presented at a mean age of 46 years (median 46.4; range 24-81), with average symptom(s) duration of 67 months (median 24 months; range 6 weeks-53 years). The commonest symptoms were proptosis (13 patients; 50%), orbital ache or pain (11/26, 42%), having noted a lump in the upper eyelid (8/26, 31%), and diplopia (7/26, 27%). About 90% of patients had nonaxial globe displacement, over a half had a palpable mass and 3-quarters had reduced motility. Adenocarcinoma was the commonest malignant component of the tumor, occurring in 16 (62%) of the patients, followed by adenoid cystic carcinoma in 5 patients (19%); 10/26 (28%) tumors showed significant invasion on histological examination. A quarter of the patients had tumor-related death at an average of 2.8 years after diagnosis (median 2.5; range 1.5-4.4 years), and the overall probability of survival for the whole cohort was 63% at both 5 years and 10 years after treatment. The prognosis for CXPA was significantly worse with a history of prior incompletely excised pleomorphic adenoma (p < 0.001), a markedly invasive malignant component (p = 0.001), evidence of perineural invasion (p = 0.042), and an American Joint Committee on Cancer tumor size of T3 or greater (p = 0.024).

CONCLUSION: Intact excision of CXPAs that do not display histological evidence of extension beyond the tumor pseudocapsule have a good outcome, similar to that for intact excision of benign pleomorphic adenomas. In contrast, our patients with markedly invasive CXPA had a tendency to local recurrence or distant metastasis, and tumor-related death occurred in about two-thirds.

PMID:33710040 | DOI:10.1097/IOP.0000000000001957

Adenoid cystic carcinoma of the breast: a study of five cases

Fetched: March 13th, 2021, 5:00am GMT by Lixian Liu

J Radiol Case Rep. 2020 Nov 30;14(11):16-25. doi: 10.3941/jrcr.v14i11.3921. eCollection 2020 Nov.

ABSTRACT

Adenoid cystic carcinoma of the breast is a rare type of breast cancer. Most patients present with a bilateral palpable mass. Ultrasound and mammography are non-specific and can sometimes lead to misdiagnosis because of their variable imaging features. Pathological examination is the standard reference. Surgery is the mainstay of treatment for patients. Although adenoid cystic carcinoma has an excellent prognosis, metastatic cases have been reported. This report aims to discuss the clinical and imaging features of one case of adenoid cystic carcinoma with a poor prognosis and four cases with a good prognosis at our center.

PMID:33708341 | PMC:PMC7942960 | DOI:10.3941/jrcr.v14i11.3921

miR-5191 acts as a tumor suppressor in salivary adenoid cystic carcinoma by targeting Notch-2

Fetched: March 12th, 2021, 5:00am GMT by Zhi Li

Oral Dis. 2021 Mar 11. doi: 10.1111/odi.13841. Online ahead of print.

ABSTRACT

OBJECTIVE: This study sought to investigate the effect of miR-5191 on proliferation, invasion and metastasis in salivary adenoid cystic carcinoma (SACC).

MATERIALS AND METHODS: The differential expression level of miR-5191 between 5 primary tumor and adjacent non-neoplastic samples, and in two SACC cell lines was detected by quantitative real-time PCR. Cell proliferation, invasion, and migration were performed, followed by luciferase reporter assay and western analysis. The effect of miR-5191 on cell proliferation and apoptosis was evaluated by cell growth and apoptosis assay. The function of miR-5191 in SACC tumorigenesis and metastasis in vivo was investigated by nude mice experiment. The associations between miR-5191/Notch-2 expression and clinicopathological features were analyzed.

RESULTS: miR-5191 was downregulated in primary tumor tissues and SACC-LM cells. By targeting Notch-2, miR-5191 expression level affected the migration, invasion, and proliferation of SACC cells. Overexpression of miR-5191 inhibited the expression levels of Notch-2, followed by the decreased expression of c-Myc, Bcl-2, Hes-1, Hey-1, and Cyclin D1. In vivo, miR-5191 overexpression suppressed the SACC tumorigenesis and pulmonary metastasis in mice. In SACC patients, higher expression of miR-5191 was related to better prognoses and lower possibility of metastasis.

CONCLUSIONS: miR-5191 acts as a tumor suppressor in SACC by targeting Notch-2.

PMID:33694237 | DOI:10.1111/odi.13841

Intensity-modulated radiation therapy for nasal cavity and paranasal sinus tumors: Experience from a single institute

Fetched: March 10th, 2021, 5:00am GMT by Sarbani Ghosh Laskar

Head Neck. 2021 Mar 9. doi: 10.1002/hed.26669. Online ahead of print.

ABSTRACT

BACKGROUND: To assess the efficacy of intensity-modulated radiation therapy (IMRT) for tumors of the nasal cavity and paranasal sinus (PNS) region.

MATERIALS AND METHODS: Two hundred fourteen patients with tumors of the nasal cavity and PNS region treated with curative intent IMRT between 2007 and 2019 were included in this retrospective analysis.

RESULTS: Fifty-one (24.1%) received definitive RT/CTRT and 163 (75.9%) received adjuvant RT. Most common histology was squamous cell carcinoma (26.1%) followed by adenoid cystic carcinoma (21.5%). The median follow-up was 43.5 months. The 5-year local control (LC), event-free survival (EFS), and overall survival (OS) for the entire cohort was 66.9%, 59%, and 73.9%, respectively. On univariate analysis treatment with nonsurgical modality, T classification and undifferentiated/poorly differentiated histology were associated with inferior 5-year LC, EFS, and OS. Four patients had late Grade 3/Grade 4 ocular toxicity.

CONCLUSIONS: IMRT should be the standard of care for tumors of PNS region across all histologies and treatment setting.

PMID:33687114 | DOI:10.1002/hed.26669

Bilateral kidney metastases from adenoid cystic carcinoma of lung: a case report and literature review

Fetched: March 10th, 2021, 5:00am GMT by Pietro Piazza

CEN Case Rep. 2021 Mar 8. doi: 10.1007/s13730-021-00589-8. Online ahead of print.

ABSTRACT

Adenoid cystic carcinoma (ACC) is a rare malignant tumor, usually arising from salivary glands and rarely found in other locations. ACC is characterized by asymptomatic course, slow growth pattern, perineural invasion and high incidence of late metastasis. Renal localization of metastasis is rare. Only 12 cases of renal metastasis were published and, to our best knowledge, no case of bilateral metastasis to the kidney has ever been reported. We present a case of a 58 years old woman with bilateral renal metastasis from ACC of the right lung after fourteen years from lobectomy and radiotherapy for the primary presentation. The patient underwent bilateral robot-assisted partial nephrectomy in a two-stage approach. Despite its rare incidence and slow growth, ACC metastasis may simulate primary papillary renal carcinoma and occur many years after primary treatment. Therefore, lifelong follow-up, including abdominal imaging, is recommended.

PMID:33686592 | DOI:10.1007/s13730-021-00589-8

Taking a step down on the reconstruction ladder for head and neck reconstruction during the COVID-19 pandemic

Fetched: March 10th, 2021, 5:00am GMT by Haroon Ur Rashid

BMC Surg. 2021 Mar 8;21(1):120. doi: 10.1186/s12893-021-01134-1.

ABSTRACT

BACKGROUND: Most of the head and neck cancers are time-critical and need urgent surgical treatment. Our unit is one of the departments in the region, at the forefront in treating head and neck cancers in Pakistan. We have continued treating these patients in the COVID-19 pandemic with certain modified protocols. The objective of this study is to share our experience and approach towards head and neck reconstruction during the COVID-19 pandemic.

RESULTS: There were a total of 31 patients, 20 (64.5%) were males and 11 (35.4%) patients were females. The mean age of patients was 52 years. Patients presented with different pathologies, i.e. Squamous cell carcinoma n = 26 (83.8%), mucoepidermoid carcinoma n = 2 (6.4%), adenoid cystic carcinoma n = 2 (6.4%) and mucormycosis n = 1 (3%). The reconstruction was done with loco-regional flaps like temporalis muscle flap n = 12 (38.7%), Pectoralis major myocutaneous flap n = 8 (25.8%), supraclavicular artery flap n = 10 (32.2%) and combination of fore-head, temporalis major and cheek rotation flaps n = 1 (3%). Defects involved different regions like maxilla n = 11 (35.4%), buccal mucosa n = 6 (19.3%), tongue with floor of mouth n = 6 (19.3%), mandible n = 4 (12.9%), parotid gland, mastoid n = 3 (9.6%) and combination of defects n = 1 (3%). Metal reconstruction plate was used in 3 (9.6%) patients with mandibular defects. All flaps survived, with the maximum follow-up of 8 months and minimum follow-up of 6 months.

CONCLUSION: Pedicled flaps are proving as the workhorse for head and neck reconstruction in unique global health crisis. Vigilant use of proper PPE and adherence to the ethical principles proves to be the only shield that will benefit patients, HCW and health system.

PMID:33685447 | PMC:PMC7938274 | DOI:10.1186/s12893-021-01134-1

Adenoid Cystic Carcinoma Developed from the Parotid Gland to the Ear Lobe of a Young Woman

Fetched: March 9th, 2021, 5:00am GMT by Gaku Nojiri

Plast Reconstr Surg Glob Open. 2021 Feb 16;9(2):e3393. doi: 10.1097/GOX.0000000000003393. eCollection 2021 Feb.

ABSTRACT

Adenoid cystic carcinoma (ACC) is a relatively rare malignant tumor. It is more common in women than in men and typically develops in the lacrimal, salivary, and breast glands. ACC of the external auditory canal (EAC) is exceedingly rare, and its invasion into the ear lobe is even more unusual. In this report, we present a case of ACC that presented as a mass on the surface of the ear lobe in a 28-year-old woman and was initially diagnosed as infected atheroma. For wide resection of the tumor, half of the entire auricula was resected and superficial parotidectomy was performed. After confirming no tumor cells on the surface of the facial nerve, the defect was reconstructed by the combination of platysma muscle flap to prevent Frey syndrome and free forearm flap for the ear lobe form. There was no recurrence or metastasis of the tumor, and Frey syndrome did not occur at 2 years and 8 months after surgery. The patient was satisfied with the result, oncologically and cosmetically. Even in young patients, comprehensive treatments (including diagnosis, resection, and reconstruction) are important in painful ear lobe masses.

PMID:33680648 | PMC:PMC7929633 | DOI:10.1097/GOX.0000000000003393

Permalink for 'Prognostic impact of Notch1 receptor and clinicopathological High-Risk Predictors in lacrimal gland adenoid cystic carcinoma'

Prognostic impact of Notch1 receptor and clinicopathological High-Risk Predictors in lacrimal gland adenoid cystic carcinoma

Fetched: March 7th, 2021, 5:00am GMT by Shahzan Anjum

Acta Ophthalmol. 2021 Mar 6. doi: 10.1111/aos.14812. Online ahead of print.

ABSTRACT

PURPOSE: Adenoid Cystic Carcinoma (ACC) is an aggressive malignant lacrimal gland tumour associated with poor prognosis. Aberrant Notch signalling has been investigated in various tumours. However, very few studies on Notch signalling in lacrimal gland ACC are reported. The aim of the present study was to evaluate the status of Notch1 receptor and activated Notch1 (NICD1) in lacrimal gland ACC and to correlate it with high-risk clinicopathological features.

METHODS: A total of 23 cases of histopathologically proven lacrimal gland ACC, who underwent surgical treatment, were included in this study. Expression of Notch1 receptor and NICD1 was evaluated by immunohistochemistry on formalin fixed paraffin embedded tissues. The results obtained were correlated with clinicopathological high-risk features and survival of the patients. Kaplan-Meier survival and multivariate analysis was performed to determine the prognostic significance.

RESULTS: Overexpression of Notch1 receptor and NICD1 was observed in 65% and 39% of lacrimal gland ACC cases, respectively. On Kaplan-Meier survival analysis, patients with Notch1 receptor overexpression had reduced disease free survival. On univariate analysis, male gender, bone erosion, perineural invasion, solid histologic pattern, intracranial extension and advanced tumour stage were also indicators of poor prognosis. On multivariate analysis bone erosion was the most significant poor prognostic indicator.

CONCLUSION: Our study demonstrates that overexpression of Notch1 receptor plays a critical role in the biology and aggressive behaviour of lacrimal gland ACC. Bone erosion, solid histologic pattern, advanced T stage, perineural invasion and intracranial extension are other high-risk clinicopathological predictors of lacrimal gland ACC.

PMID:33675181 | DOI:10.1111/aos.14812

Nasopharyngeal adenoid cystic carcinoma presenting with exophthalmos: A case report

Fetched: March 6th, 2021, 5:00am GMT by S Sninate

Radiol Case Rep. 2021 Feb 15;16(4):956-960. doi: 10.1016/j.radcr.2021.02.011. eCollection 2021 Apr.

ABSTRACT

Adenoid cystic carcinoma is a malignant tumor involving the salivary glands, rarely developing in the nasopharynx. It is a slowly evolving entity with strong local aggressiveness and a high tendency to recurrence. We report the case of 23-year-old patient with adenoid cystic carcinoma of the nasopharynx presenting with exophthalmos in which radiation therapy is the sole therapeutic option.

PMID:33664920 | PMC:PMC7897933 | DOI:10.1016/j.radcr.2021.02.011

Multi-Omics Characterization and Origin Assessment of Bilateral Lung Adenoid Cystic Carcinoma: A Case Report

Fetched: March 6th, 2021, 5:00am GMT by Minhua Ye

Cancer Manag Res. 2021 Feb 25;13:1981-1987. doi: 10.2147/CMAR.S292789. eCollection 2021.

ABSTRACT

BACKGROUND: Primary adenoid cystic carcinoma (ACC) of the lung, which arises from the bronchial gland and is rare, accounting for only 0.04-0.2% of all primary lung tumors. The genetic profiling of bilateral ACC of unknown primary site and application in postoperative decision-making are less reported.

CASE PRESENTATION: A 57-year-old male with a smoking history of over 30 years and multiple nodules in both lungs was present to our department. After assessing the bilateral solid nodules in his Positron Emission Tomography-Computed Tomography (PET/CT) scan, malignant lesions at the left lower lung, right lower lung, and right middle lung are suspected. Sequential selective video-assisted thoracoscopic surgeries (VATS) were performed. A genetic alteration test of 425 cancer-related genes and global gene expression profile of the specimens revealed intrapulmonary metastasis existed. The patient was followed up for three years without recurrence and tissue mutations in liquid biopsy.

CONCLUSION: We present a way of omics-based multiple pulmonary lesions origin assessment, facilitating post-operative differential diagnosis and treatment decision for difficult cases.

PMID:33664590 | PMC:PMC7921630 | DOI:10.2147/CMAR.S292789

Adenocarcinoma NOS arising from accessory lacrimal glands: A case report and a review of the literature

Fetched: March 6th, 2021, 5:00am GMT by Maria Antonietta Blasi

Eur J Ophthalmol. 2021 Mar 4:1120672121999993. doi: 10.1177/1120672121999993. Online ahead of print.

ABSTRACT

Primary malignant epithelial tumours arising from accessory lacrimal glands (ALGs) are extremely rare, with only few cases reported in literature. They generally appear as gradually increasing masses of the upper or the lower eyelid. Only one case of primary adenocarcinoma or adenocarcinoma not otherwise specified (ACNOS) from ALGs has been reported in literature. Herein, we describe a case of ACNOS arising from ALGs with an atypical clinical presentation and review prior cases of ALGs epithelial malignancies reported in the literature. A 78-year-old man referred to our Ocular Oncology Unit for adjuvant therapy after the excision of a conjunctival tumour of the left eye with a histological diagnosis of squamous cell carcinoma. He underwent topical chemotherapy with MMC and during follow up he presented a multinodular iris mass in his left eye. The MRI of the orbit showed an ocular mass infiltrating orbital soft tissues of the inferior palpebral region with an involvement of the corresponding zygomatic cutis. We performed orbital exenteration and histological studies revealed an epithelial neoplasm with a solido-glandular growth pattern with lumens containing an eosinophilic material positive for PAS and PAS-D. The immunohistochemical findings confirmed the diagnosis of adenocarcinoma NOS from ALGs. Although ALGs epithelial malignancies are extremely uncommon, they should be considered in the differential diagnosis of ocular tumours. A vigilant approach towards these entities is required, since they can be clinically insidious and locally aggressive.

PMID:33663254 | DOI:10.1177/1120672121999993

Permalink for 'Application of superficial inferior epigastric artery flaps to repair oral and maxillofacial defects with the aid of digital three-dimensional reconstruction technique'

Application of superficial inferior epigastric artery flaps to repair oral and maxillofacial defects with the aid of digital three-dimensional reconstruction technique

Fetched: March 6th, 2021, 5:00am GMT by D Wang

Zhonghua Kou Qiang Yi Xue Za Zhi. 2021 Mar 9;56(3):263-267. doi: 10.3760/cma.j.cn112144-20200826-00478.

ABSTRACT

Objective: To explore the application value of digital three-dimensional(3D) reconstruction technology in the repair of oral and maxillofacial defects with superficial inferior epigastric artery (SIEA) flap. Methods: Twelve cases of oral cancer patients, including 8 males and 4 females; aged (57.4±12.6) years, were selected from the Department of Oral and Maxillofacial Surgery, The First Affiliated Hospital of Bengbu Medical College from January 2018 to October 2019 and were proposed to repair with SIEA flap. There were 10 cases of squamous cell carcinoma, one case of adenoid cystic carcinoma and 1 case of mucinous epidermal carcinoma. The data were imported into AW4.7 software for post-processing. The left or right dominant donor area was selected to clarify the origin, diameter, alignment, and location of penetration point of the flap blood supply, and digital 3D reconstruction technology was used to guide the flap preoperative design. Results: Eleven cases were repaired by SIEA flap in 12 patients, one case was repaired by superficial iliac artery flap because the source artery was undiscovered, one case had venous vascular crisis after surgery, and the rest of the flap survived. In 11 patients repaired with SIEA flap, there was no significant difference between the preoperative SIEA diameter measured by CTA [(1.0±0.3) mm] and the actual measured value [(1.1±0.3) mm] (P>0.05). The follow-up was 6 to 12 months, with an average of 10 months, and the donor-receiver areas were all healed in phase Ⅰ. No obvious complications occurred, and the abdominal scar was hidden. Conclusions: In the SIEA flap repair oral and maxillofacial defect reconstruction surgery, the use of digital 3D reconstruction technology can objectively reflect the diameter and the location of the superficial artery of the abdominal wall before surgery, effectively reduce the difficulty and risk of flap surgery.

PMID:33663156 | DOI:10.3760/cma.j.cn112144-20200826-00478

A Case of Carotid Body Tumor with Perineural Tumor Spread along the Superior Laryngeal Nerve

Fetched: March 4th, 2021, 5:00am GMT by Ashley Renay Way

J Clin Imaging Sci. 2021 Feb 9;11:6. doi: 10.25259/JCIS_190_2020. eCollection 2021.

ABSTRACT

We report a case of a patient with a carotid body tumor with perineural tumor spread along the right superior laryngeal nerve. Perineural spread is most commonly associated with squamous cell, adenoid cystic, and mucoepidermoid carcinoma. To the best of our knowledge, this has not been reported previously with carotid body tumor.

PMID:33654575 | PMC:PMC7911031 | DOI:10.25259/JCIS_190_2020

Laryngeal adenoid cystic carcinoma: Radical or conservative surgery?

Fetched: March 3rd, 2021, 5:00am GMT by Marco Lionello

Am J Otolaryngol. 2021 Feb 16;42(4):102974. doi: 10.1016/j.amjoto.2021.102974. Online ahead of print.

ABSTRACT

PURPOSE: The present paper describes our experience in surgical treatment of laryngeal ACC, and discuss the effectiveness of conservative surgery.

METHODS: We retrospectively reviewed the clinical charts of 17 patients with laryngeal ACC treated surgically at the Otolaryngology Unit of Vittorio Veneto Hospital (Italy) from November 1989 to April 2020.

RESULTS: Fourteen patients underwent partial laryngectomy, and three had a total laryngectomy. Five patients (29%) experienced a laryngeal ACC relapse after a disease-free survival of 66.6 ± 50.1 months. The distant metastasis rate was 17%. At latest follow-up, two patients had died of distant metastatic disease after 156 and 243 months.

CONCLUSIONS: Radical surgery for laryngeal ACC does not warrant free margins and even cases with positive deep margins rarely experience any relapsing disease. We recommend that surgical treatment for laryngeal ACC be as conservative as possible.

PMID:33652330 | DOI:10.1016/j.amjoto.2021.102974

Permalink for 'Intensity-modulated proton therapy (IMPT) versus intensity-modulated radiation therapy (IMRT) for the treatment of head and neck cancer: A dosimetric comparison'

Intensity-modulated proton therapy (IMPT) versus intensity-modulated radiation therapy (IMRT) for the treatment of head and neck cancer: A dosimetric comparison

Fetched: March 3rd, 2021, 5:00am GMT by My-Lien Nguyen

Med Dosim. 2021 Feb 26:S0958-3947(21)00013-3. doi: 10.1016/j.meddos.2021.02.001. Online ahead of print.

ABSTRACT

It is the goal of this study to compare the dosimetric advantages of IMPT when compared to IMRT. From January 2019 to August 2020, 25 patients were treated with intensity modulated proton therapy (IMPT) at our institution for either recurrent, metastatic, benign, or primary tumors in the head and neck region. Twenty-one patients met criteria for dosimetric analysis. Histology of disease included squamous cell carcinoma, acinic cell carcinoma, sarcomatoid sinonasal carcinoma, paraganglioma, adenoid cystic carcinoma, salivary high grade carcinoma, and papillary thyroid carcinoma. For IMRT planning, gross tumor volume (GTV) and clinical target volume (CTV) were contoured with the expansion of 3-5 mm to create the planning target volume (PTV) and dose was prescribed to the PTV. For the IMPT planning, dose was prescribed to CTV and robust optimization was utilized which accounted for a 5 mm setup and range uncertainty. The minimum, mean and maximum target doses for IMRT and IMPT plans as well as mean and maximum normal tissue doses are reported for the 21 patients meeting criteria. Mean doses for IMRT and IMPT were 6278.2 cGy and 6449.8 cGyRBE respectively with p-value of 0.0001. Maximum doses for IMRT and IMPT were 6579.5 cGy and 6772.1 cGyRBE respectively with p-value of 0.0014. Minimum doses for IMRT and IMPT were 5440.6 cGy and 5617.9 cGyRBE respectively with p-value of 0.3576. IMPT had an overall advantage in OAR doses in the brain stem, spinal cord, optic structures, cochlea, larynx, contralateral parotid, and oral cavity with only a few exceptions. Our study thus demonstrates a dosimetric advantage for IMPT in treating head and neck tumors in mean and max dose delivered as well as dose to OARs. Given that our patient cohort were mainly unilateral head and neck cases, our study supports the treatment of this specific subset of patients regardless of histology with IMPT. This may aid in appropriate patient selection for IMPT treatment. Further studies will need to determine if this dosimetric advantage translates to a therapeutic advantage for patients.

PMID:33648823 | DOI:10.1016/j.meddos.2021.02.001

Clinico-pathological Profile of Head and Neck Tumours with Intracranial Extension

Fetched: March 1st, 2021, 5:00am GMT by M O Udoh

West Afr J Med. 2021 Feb;38(2):131-136.

ABSTRACT

BACKGROUND: Intracranial extensions of Head and Neck region tumours are a result of direct spread, into the brain and meninges, of tumours arising in the pharynx, paranasal sinuses, orbit, middle ear, scalp, skull and neck tissues. Presenting features may resemble those of an intracranial mass, or the contiguous structure of origin, or both. Delayed diagnosis is not uncommon when patients are not able to afford diagnostic imaging or when the possibility of an intracranial extension is not readily considered in the evaluation of patients with head and neck lesions.

AIM: To highlight the important histologic entities in the head and neck region presenting as intracranial mass lesions.

METHODS: This was a retrospective database study of tumours in the head and neck region with extension into the cranial cavity.

RESULTS: We present 13 patients with intracranial extension of tumours from contiguous structures. There were 7 males and 6 females. Age ranged from 16 to 80 years. Mean age of the patients was 48.92 ± 19.13 years. Specific histologic entities included: nasopharyngeal carcinoma, olfactory neuroblastoma, squamous cell carcinoma, rhabdomyosarcoma and adenoid cystic carcinoma, amongst others CONCLUSION: Early and accurate diagnosis requires a raised awareness of tumours in the head and neck region; their likelihood to invade the cranial cavity; and a familiarity with the likely presentation. Multidisciplinary approach is needed when clinical and radiological features are suggestive of mass lesions breaching the skull and invading the brain from adjacent structures. Confirmation of clinical suspicion by histology is essential.

PMID:33641147

Permalink for 'Aberrantly expressed GFRalpha-1/RET in patients with lacrimal adenoid cystic carcinoma is associated with high recurrence risk: a retrospective study of 51 LACC cases'

Aberrantly expressed GFRalpha-1/RET in patients with lacrimal adenoid cystic carcinoma is associated with high recurrence risk: a retrospective study of 51 LACC cases

Fetched: February 26th, 2021, 5:00am GMT by Lin Liu

Cancer Biol Med. 2021 Feb 15;18(1):199-205. doi: 10.20892/j.issn.2095-3941.2020.0271.

ABSTRACT

OBJECTIVE: Because of the poor prognosis of lacrimal adenoid cystic carcinoma (LACC), we aimed to investigate the effects of perineural invasion (PNI) and consequent aberrations in GDNF/GFRα-1/RET protein expression on LACC recurrence.

METHODS: Clinicopathological data for 51 histologically confirmed patients with LACC enrolled between 2001 and 2017 were retrospectively analyzed. Hematoxylin and eosin staining was applied to assess PNI. Tissue-based immunohistochemistry (IHC) detection of GDNF, GFRα-1, and RET proteins was performed on LACC formalin-fixed, paraffin-embedded specimens. We generated semi-quantitative data of the IHC results and compared them with the clinicopathological data for the 51 patients.

RESULTS: Of the 51 patients, 19 (37.3%) were PNI positive. Recurrence was more common for LACC with than without PNI (73.7% vs. 37.5%, P = 0.01). GDNF, GFRα-1, and RET proteins were expressed in 62.7%, 62.7%, and 54.9% of the 51 patients with LACC, respectively. The expression of all 3 proteins was more common in patients with than without PNI. In agreement with previous findings, PNI-associated GFRα-1 and RET positivity, as detected by IHC, remained significantly associated with recurrence, whereas GDNF expression, as detected by IHC, was not correlated with LACC recurrence. Specifically, patients with concurrent GFRα-1 and RET expression may have a high risk of PNI (89.5% positivity rate) and recurrence (84.2% positivity rate).

CONCLUSIONS: PNI may contribute to LACC recurrence. The concurrent expression of GFRα-1 and RET proteins, as detected by IHC, may potentially be associated with LACC PNI and recurrence.

PMID:33628594 | PMC:PMC7877180 | DOI:10.20892/j.issn.2095-3941.2020.0271

Adenoid cystic carcinoma and chronic lymphocytic leukaemia: synchronous presentations in the lung

Fetched: February 26th, 2021, 5:00am GMT by Philip S Webb

BMJ Case Rep. 2021 Feb 24;14(2):e236074. doi: 10.1136/bcr-2020-236074.

ABSTRACT

A 59-year-old male active smoker presented with a 6-month history of cough and breathlessness and was found to have a right upper lobe mass. Histology revealed this to be an adenoid cystic carcinoma (ACC) of the lung, while local lymph node dissection revealed a synchronous diagnosis of chronic lymphocytic leukaemia (CLL). The connection between CLL and solid organ malignancy is well documented, but the reporting of ACC in this context is novel. Mechanisms linking the two processes are revealed with the possibility of causality, and heightened vigilance for the development of primary lung tumours in CLL, and their management, is recommended.

PMID:33627346 | PMC:PMC7919549 | DOI:10.1136/bcr-2020-236074

Permalink for 'Outcomes Following Percutaneous Microwave and Cryoablation of Lung Metastases from Adenoid Cystic Carcinoma of the Head and Neck: A Bi-Institutional Retrospective Cohort Study'

Outcomes Following Percutaneous Microwave and Cryoablation of Lung Metastases from Adenoid Cystic Carcinoma of the Head and Neck: A Bi-Institutional Retrospective Cohort Study

Fetched: February 24th, 2021, 5:00am GMT by Konstantin S Leppelmann

Ann Surg Oncol. 2021 Feb 23. doi: 10.1245/s10434-021-09714-4. Online ahead of print.

ABSTRACT

OBECTIVE: The aim of this study was to report outcomes following percutaneous microwave and cryoablation of lung metastases from adenoid cystic carcinoma (ACC) of the head and neck.

MATERIAL AND METHODS: This bi-institutional retrospective cohort study included 10 patients (6 females, median age 59 years [range 28-81]) who underwent 32 percutaneous ablation sessions (21 cryoablation, 11 microwave) of 60 lung metastases (median 3.5 tumors per patient [range 1-16]) from 2007 to 2019. Median tumor diameter was 16 mm [range 7-40], significantly larger for cryoablation (22 mm, p = 0.002). A median of two tumors were treated per session [range 1-7]. Technical success, local control, complications, and overall survival were assessed.

RESULTS: Primary technical success was achieved for 55/60 tumors (91.7%). Median follow-up was 40.6 months (clinical) and 32.5 months (imaging, per tumor). Local control at 1, 2, and 3 years was 94.7%, 80.8%, and 76.4%, respectively, and did not differ between ablation modalities. Five of fifteen recurrent tumors underwent repeat ablation, and secondary technical success was achieved in four (80%). Assisted local tumor control at 1, 2, and 3 years was 96.2%, 89.8%, and 84.9%, respectively. Complications occurred following 24/32 sessions (75.0%) and 57.2% Common Terminology Criteria for Adverse Events (CTCAE) lower than grade 3. Of 13 pneumothoraces, 7 required chest tube placements. Hemoptysis occurred after 7/21 cryoablation sessions, and bronchopleural fistula developed more frequently with microwave (p = 0.037). Median length of hospital stay was 1 day [range 0-10], and median overall survival was 81.5 months (IQR 40.4-93.1).

CONCLUSION: Percutaneous computed tomography-guided microwave and cryoablation can treat lung metastases from ACC of the head and neck. Complications are common but manageable, with full recovery expected.

PMID:33620616 | DOI:10.1245/s10434-021-09714-4

Pathological evaluation of tumor grade for salivary adenoid cystic carcinoma: A proposal of an objective grading system

Fetched: February 24th, 2021, 5:00am GMT by Naruhiko Morita

Cancer Sci. 2021 Mar;112(3):1184-1195. doi: 10.1111/cas.14790. Epub 2021 Feb 2.

ABSTRACT

Three pathological grading systems advocated by Perzin/Szanto, Spiro, and van Weert are currently used for adenoid cystic carcinoma (AdCC). In these systems, the amount or presence of the solid tumor component in AdCC specimens is an important index. However, the "solid tumor component" has not been well defined. Salivary AdCC cases (N = 195) were collected after a central pathology review. We introduced a novel criterion for solid tumor component, minAmax (minor axis maximum). The largest solid tumor nest in each AdCC case was histologically screened, the maximum oval fitting the solid nest was estimated, and the length of the minor axis of the oval (minAmax) was measured. The prognostic cutoff for the minAmax was determined using training and validation cohorts. All cases were evaluated for the four grading systems, and their prognostic impact and interobserver variability were examined. The cutoff value for the minAmax was set at 0.20 mm. Multivariate prognostic analyses showed the minAmax and van Weert systems to be independent prognostic tools for overall, disease-free, and distant metastasis-free survival while the Perzin/Szanto and Spiro systems were selected for overall survival but not for disease-free or distant metastasis-free survival. The highest hazard ratio for overall survival (11.9) was obtained with the minAmax system. The reproducibility of the minAmax system (kappa coefficient of 0.81) was scored as very good while those of the other three systems were scored as moderate. In conclusion, the minAmax is a simple, objective, and highly reproducible grading system useful for prognostic stratification for salivary AdCC.

PMID:33377247 | DOI:10.1111/cas.14790

Palatine tonsil adenoid cystic carcinoma

Fetched: February 24th, 2021, 5:00am GMT by Dmitry Tretiakow

Oral Oncol. 2020 Dec 26:105135. doi: 10.1016/j.oraloncology.2020.105135. Online ahead of print.

NO ABSTRACT

PMID:33376056 | DOI:10.1016/j.oraloncology.2020.105135

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