Rare Cancer News & Clinical Trials

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Hati waja - Harian Metro

Posted: August 10th, 2017, 7:14pm GMT

Hati waja
Harian Metro
Meskipun pernah diuji dugaan hebat, disahkan menghidap penyakit kanser payu dara pada 2001 dan kanser Acinic Cell Carcinoma pada tahun 2014 namun kekentalan semangat wanita berusia 56 tahun, Renee Aziz Ahmad wajar dicontohi. Pesara jurutera ...

Generation of a pancreatic cancer model using a Pdx1-Flp recombinase knock-in allele.

Fetched: September 23rd, 2017, 5:00am GMT

Generation of a pancreatic cancer model using a Pdx1-Flp recombinase knock-in allele.

PLoS One. 2017;12(9):e0184984

Authors: Wu J, Liu X, Nayak SG, Pitarresi JR, Cuitiño MC, Yu L, Hildreth BE, Thies KA, Schilling DJ, Fernandez SA, Leone G, Ostrowski MC

Abstract
The contribution of the tumor microenvironment to the development of pancreatic adenocarcinoma (PDAC) is unclear. The LSL-KrasG12D/+;LSL-p53R172H/+;Pdx-1-Cre (KPC) tumor model, which is widely utilized to faithfully recapitulate human pancreatic cancer, depends on Cre-mediated recombination in the epithelial lineage to drive tumorigenesis. Therefore, specific Cre-loxP recombination in stromal cells cannot be applied in this model, limiting the in vivo investigation of stromal genetics in tumor initiation and progression. To address this issue, we generated a new Pdx1FlpO knock-in mouse line, which represents the first mouse model to physiologically express FlpO recombinase in pancreatic epithelial cells. This mouse specifically recombines Frt loci in pancreatic epithelial cells, including acinar, ductal, and islet cells. When combined with the Frt-STOP-Frt KrasG12D and p53Frt mouse lines, simultaneous Pdx1FlpO activation of mutant Kras and deletion of p53 results in the spectrum of pathologic changes seen in PDAC, including PanIN lesions and ductal carcinoma. Combination of this KPF mouse model with any stroma-specific Cre can be used to conditionally modify target genes of interest. This will provide an excellent in vivo tool to study the roles of genes in different cell types and multiple cell compartments within the pancreatic tumor microenvironment.

PMID: 28934293 [PubMed - in process]

Acinar cell carcinoma of the pancreas presenting as diffuse pancreatic enlargement: Two case reports and literature review.

Fetched: September 22nd, 2017, 5:00am GMT

Acinar cell carcinoma of the pancreas presenting as diffuse pancreatic enlargement: Two case reports and literature review.

Medicine (Baltimore). 2017 Sep;96(38):e7904

Authors: Luo Y, Hu G, Ma Y, Guo N, Li F

Abstract
RATIONALE: Pancreatic acinar cell carcinoma (ACC) is a rare malignant tumor of exocrine pancreas. It is typically a well-marginated large solid mass arising in a certain aspect of the pancreas. Diffuse involvement of ACC in the pancreas is very rare, and may simulate pancreatitis in radiological findings. We report 2 cases of ACC presenting as diffuse enlargement of the pancreas due to tumor involvement without formation of a distinct mass.
PATIENT CONCERNS: The patients consisted of a 41-year-old man with weight loss and a 77-year-old man who was asymptomatic.
DIAGNOSES: Computed tomography (CT) and 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT showed diffuse enlargement of the pancreas forming a sausage-like shape with homogenously increased FDG activity.
INTERVENTIONS: Endoscopic ultrasound (EUS)-guided fine needle aspiration (FNA) biopsy of the pancreatic lesion was performed.
OUTCOMES: Histopathology results from the pancreas confirmed the diagnosis of pancreatic ACC.
LESSONS: Because diffuse enlargement of the pancreas is a common imaging feature of pancreatitis, recognition of this rare morphologic pattern of ACC is important for radiological diagnosis of this tumor.

PMID: 28930825 [PubMed - in process]

Permalink for 'Pancreatic acinar cell carcinoma with extensive tumor embolism at the trunk of portal vein and pancreatic intraductal infiltration.'

Pancreatic acinar cell carcinoma with extensive tumor embolism at the trunk of portal vein and pancreatic intraductal infiltration.

Fetched: September 20th, 2017, 5:00am GMT

Pancreatic acinar cell carcinoma with extensive tumor embolism at the trunk of portal vein and pancreatic intraductal infiltration.

Clin J Gastroenterol. 2017 Sep 18;:

Authors: Kida A, Matsuda K, Takegoshi K, Matsuda M, Sakai A, Noda Y

Abstract
A 59-year-old man was admitted to the hospital with acute pancreatitis. The cause was suggested to be a pancreatic tumor based on computed tomography (CT). The pancreatic tumor was 45 mm with an extensive tumor embolism at the trunk of the portal vein and intraductal infiltration of the main pancreatic duct (MPD). The pancreatic tumor was diagnosed as acinar cell carcinoma (ACC) by endoscopic ultrasound guided fine needle aspiration. Therefore, the cause of acute pancreatitis was diagnosed to be intraductal infiltration of ACC in the MPD. Chemotherapy was initially performed because it was difficult to perform surgery due to extensive tumor embolism at the trunk of the portal vein. Degeneration and reduction of ACC and tumor embolism of the portal vein was noted on CT after chemotherapy, and extended distal pancreatectomy with portal vein reconstruction was performed. There has been no relapse for 5 years postoperative follow-up. This is an interesting and rare case because ACC with intraductal infiltration of MPD is low in frequency; most ACCs are asymptomatic when they are found, and many cases tend to have poor prognosis in spite of surgical cases.

PMID: 28921102 [PubMed - as supplied by publisher]

Acinar Cell Carcinoma of the Pancreas: A Case Report.

Fetched: September 20th, 2017, 5:00am GMT

Acinar Cell Carcinoma of the Pancreas: A Case Report.

Mymensingh Med J. 2017 Jul;26(3):684-688

Authors: Mohammed S, Chowdhury MM, Ullah AA, Towhid SM, Sobhan SA, Zeba IJ, Haque MM, Islam MT, Hussain M, Roy TK, Russel SR, Chowdhury AM, Alam MK

Abstract
Acinar cell carcinoma (ACC) of the pancreas is a very rare neoplasm. We report a case of pancreatic acinar cell carcinoma involving the uncinate process of the pancreas. A 45 year old man presented with a painful upper abdominal mass without any jaundice or weight loss. Computed Tomography (CT) and Magnetic Resonance Cholangio-Pancreatography (MRCP) indicated a mass lesion in the uncinate process of the pancreas. He underwent Whipple's procedure (Pancreaticoduodenectomy). Histological slides revealed features of Acinar cell carcinoma (ACC) in the uncinate process of the pancreas and a lymph node.

PMID: 28919629 [PubMed - in process]

Permalink for 'HMGA1 expression levels are elevated in pancreatic intraepithelial neoplasia cells in the Ptf1a-Cre; LSL-KrasG12D transgenic mouse model of pancreatic cancer.'

HMGA1 expression levels are elevated in pancreatic intraepithelial neoplasia cells in the Ptf1a-Cre; LSL-KrasG12D transgenic mouse model of pancreatic cancer.

Fetched: September 20th, 2017, 5:00am GMT

HMGA1 expression levels are elevated in pancreatic intraepithelial neoplasia cells in the Ptf1a-Cre; LSL-KrasG12D transgenic mouse model of pancreatic cancer.

Br J Cancer. 2017 Aug 22;117(5):639-647

Authors: Veite-Schmahl MJ, Joesten WC, Kennedy MA

Abstract
BACKGROUND: Pancreatic cancer is currently the third leading cause of cancer deaths in the United States and it is predicted to become the second by the year 2030. High-mobility group A1 protein (HMGA1) is an oncogenic transcription factor, localised and active in cell nuclei, that is linked to tumour progression in many human cancers, including pancreatic cancer. Overexpression of HMGA1 renders cancer cells resistant to chemotherapy. Although the Ptf1a-Cre; LSL-KrasG12D transgenic mouse is perhaps the most widely utilised animal model for human pancreatic cancer, expression levels of HMGA1 in pancreata from this mouse model have not been characterised.
METHODS: Quantitative immunohistochemical analysis was used to determine nuclear HMGA1 levels in pancreatic tissue sections from Ptf1a-Cre; LSL-KrasG12D mice aged 5, 11, and 15 months. The H Score method was used for quantitative analysis.
RESULTS: The HMGA1 levels were significantly elevated in pancreatic intraepithelial neoplasia (PanIN) epithelia compared with untransformed acinar tissues or fibroinflammatory stroma.
CONCLUSIONS: The PanINs have long been regarded as precancerous precursors to pancreatic adenocarcinoma. Significantly elevated HMGA1 levels observed in the nuclei of PanINs in Ptf1a-Cre; LSL-KrasG12D mice validate this animal model for investigating the role that HMGA1 plays in cancer progression and testing therapeutic approaches targeting HMGA1 in human cancers.

PMID: 28697176 [PubMed - indexed for MEDLINE]

Nodular oncocytic hyperplasia: Can cytomorphology allow for the preoperative diagnosis of a nonneoplastic salivary disease?

Fetched: September 20th, 2017, 5:00am GMT

Nodular oncocytic hyperplasia: Can cytomorphology allow for the preoperative diagnosis of a nonneoplastic salivary disease?

Cancer. 2017 Aug;125(8):627-634

Authors: Rooper LM, Onenerk M, Siddiqui MT, Faquin WC, Bishop JA, Ali SZ

Abstract
BACKGROUND: Nodular oncocytic hyperplasia (oncocytosis) of the salivary glands is a benign process that does not inherently require surgical excision. However, cytologic findings in fine-needle aspiration (FNA) of oncocytosis cases have not been well characterized previously, limiting preoperative identification.
METHODS: All available cases of oncocytosis with corresponding FNA specimens were identified from the pathology archives of 3 academic institutions. Clinical, cytologic, and histologic findings were tabulated for all cases.
RESULTS: Twelve cases of oncocytosis were identified from 11 patients, including 11 parotid FNA specimens and 1 submandibular FNA specimen. On the original diagnoses, 6 specimens were classified as benign, 4 as atypical, and 2 as nondiagnostic. Oncocytosis was listed in the differential diagnosis in only 1 case. Among diagnostic aspirates, 8 demonstrated low cellularity and 2 demonstrated moderate cellularity. All 10 cases demonstrated oncocytic cells in small to medium groups, with single cells in just 1 case. Spindled and squamous morphology were each noted in 3 cases. Four cases demonstrated cystic change and 1 showed background mucin without goblet cells. No necrosis or mitoses were observed.
CONCLUSIONS: Although oncocytosis demonstrates some overlap with Warthin tumor and oncocytoma, it lacks the diagnostic findings specific to oncocytic salivary gland malignancies such as salivary duct carcinoma, acinic cell carcinoma, mammary analog secretory carcinoma, and mucoepidermoid carcinoma. Despite current limitations in the understanding of oncocytic salivary gland lesions, the presence of a paucicellular specimen comprised of small groups of oncocytic cells should raise the possibility of oncocytosis in the differential diagnosis and can favor it in elderly patients with multiple salivary nodules. Cancer Cytopathol 2017;125:627-34. © 2017 American Cancer Society.

PMID: 28411376 [PubMed - indexed for MEDLINE]

Fhit down-regulation is an early event in pancreatic carcinogenesis.

Fetched: September 16th, 2017, 5:00am GMT

Fhit down-regulation is an early event in pancreatic carcinogenesis.

Virchows Arch. 2017 Jun;470(6):647-653

Authors: Fassan M, Rusev B, Corbo V, Gasparini P, Luchini C, Vicentini C, Mafficini A, Paiella S, Salvia R, Cataldo I, Scarpa A, Huebner K

Abstract
Aberrant Fhit expression characterizes a large proportion of primary pancreatic ductal adenocarcinomas (PDACs), but fragmentary information is available on Fhit expression during the phenotypic changes of pancreatic ductal epithelium during multistep transformation. We assessed Fhit expression by immunohistochemistry in two different multistep pancreatic carcinogenic processes: pancreatic intraepithelial neoplasia (PanIN) and intraductal papillary mucinous neoplasia (IPMN). We considered 105 surgically treated PDACs/IPMNs and selected 30 samples of non-neoplastic pancreatic parenchyma, 50 PanIN lesions, 30 IPMNs, 15 IPMNs with associated invasive carcinoma, and 60 adenocarcinomas. Normal pancreatic ducts and surrounding acinar cells consistently showed moderate to strong Fhit immunoreactivity. Significant down-regulation of Fhit expression was observed in association with increasing severity of dysplastia/neoplastia in both carcinogenic processes. This was further confirmed by studying multiple lesions obtained from the same surgical specimen. Of 60 PDACs, only 14 showed Fhit expression comparable to normal pancreatic ductal epithelium, while the remainder (77%) showed clearly negative or reduced Fhit expression. This study demonstrates that Fhit down-regulation is an early event in both multistep carcinogenic processes leading to PDAC.

PMID: 28289900 [PubMed - indexed for MEDLINE]

Ductal adenocarcinoma of the prostate: Clinical and biological profiles.

Fetched: September 13th, 2017, 5:00am GMT

Ductal adenocarcinoma of the prostate: Clinical and biological profiles.

Prostate. 2017 Sep;77(12):1242-1250

Authors: Vinceneux A, Bruyère F, Haillot O, Charles T, de la Taille A, Salomon L, Allory Y, Ouzaid I, Choudat L, Rouprêt M, Comperat E, Houede N, Beauval JB, Vourc'h P, Fromont G

Abstract
BACKGROUND: Ductal adenocarcinoma (DAC) is a rare and aggressive subtype of prostate cancer (PCa). In the present study, we analyzed the clinical and biological characteristics of DAC, in comparison with high grade conventional acinar PCa.
METHODS: Samples and data were retrospectively collected from seven institutions and centrally reviewed. Immunohistochemistry was performed on tissue microarrays to assess the expression of candidate proteins, based on the molecular classification of PCa, including ERG, PTEN, and SPINK1. SPOP mutations were investigated from tumor DNA by Sanger sequencing. Relationships with outcome were analyzed using log-rank analysis and multivariable Cox regression.
RESULTS: Among 56 reviewed prostatectomy specimens, 45 cases of DAC were finally confirmed. The pathological stage was pT3 in more than 66% of cases. ERG was expressed in 42% of DAC, SPINK1 in 9% (all ERG-negative), and two cases (ERG-negative) harbored a SPOP mutation. Compared to high grade conventional PCa matched for the pathological stage, cell proliferation was higher (P = 0.04) in DAC, and complete PTEN loss more frequent (P = 0.023). In multivariate analysis, SPINK1 overexpression (P = 0.017) and loss of PSA immunostaining (P = 0.02) were significantly associated with biochemical recurrence.
CONCLUSION: these results suggest that, despite biological differences that highlighted DAC aggressiveness, the molecular classification recently proposed in conventional PCa could also be applied in DAC.

PMID: 28699202 [PubMed - indexed for MEDLINE]

Histologic features of stromogenic carcinoma of the prostate (carcinomas with reactive stroma grade 3).

Fetched: September 13th, 2017, 5:00am GMT

Histologic features of stromogenic carcinoma of the prostate (carcinomas with reactive stroma grade 3).

Hum Pathol. 2017 May;63:202-211

Authors: De Vivar AD, Sayeeduddin M, Rowley D, Cubilla A, Miles B, Kadmon D, Ayala G

Abstract
Prostatic carcinoma, like many other carcinomas, generates a stromal reaction. This phenomenon is well established in the scientific literature. The normal parenchymal smooth muscle phenotype switches to a myofibroblastic phenotype in response to the presence of cancer cells, with an expansion of the extracellular matrix compartment. The amount of reactive stroma is a predictor of biochemical recurrence in both radical prostatectomies and biopsies. It is a predictor of prostate cancer-specific death in prostatectomies. The aim of this study is to improve our histologic understanding of reactive stroma in prostate cancer and to determine the histologic features of the malignant epithelium found in stromogenic carcinomas or carcinomas with reactive stromal grade 3. Tissue microarrays of 800 patients and hematoxylin and eosin-stained sections of 120 radical prostatectomies, previously determined to contain a high proportion of areas with stromogenic carcinoma, were evaluated and findings systematically recorded. We identified 3 histologic patterns of reactive stroma: extracellular matrix-rich, cellular variant and edematous/myxoid variant. The most common pattern of carcinoma in stromogenic areas is of the acinar type with angulated glands and periglandular halos. The nuclei are enlarged, opened, with prominent nucleoli. Luminal borders are undulated, and amorphous pink secretion is often seen. Perineural invasion is frequently identified. Because of the clinical relevance, identification and quantification of areas with high reactive stromal grade by pathologists and reproducibility of our findings by others become essential. We believe that with the previously proposed grading system and the present morphologic description, both can be achieved.

PMID: 28315427 [PubMed - indexed for MEDLINE]

[A Case of Primary Acinic Cell Carcinoma(ACC)of the Breast].

Fetched: September 10th, 2017, 5:00am GMT

[A Case of Primary Acinic Cell Carcinoma(ACC)of the Breast].

Gan To Kagaku Ryoho. 2016 Nov;43(12):2019-2021

Authors: Kawai H, Sugimoto R, Iga N, Ikeda H, Yoshida R, Waki N, Ishizaki M, Nishi H, Yamashita K

Abstract
A49 -year-old woman presented with a 3.5 cm mass in her right breast. Mammography revealed a lobular mass with poorly defined margins and no microcalcification. Ultrasonography showed a hypoechoic mass with an irregular margin. The tumor was diagnosed as breast carcinoma using a core needle biopsy. The patient underwent a modified radical mastectomy with sentinel lymph node biopsy, and received adjuvant chemotherapy. The tumor consisted of 2 types of carcinoma. The center of the tumor was solid-tubular carcinoma, and the periphery was acinic cell carcinoma(ACC). Histopathologically, the neoplastic cells of the periphery were characterized by widespread acinic cell-like differentiation with a eosinophilic granular or clear cytoplasm, resembling acinic cells of the parotid gland(t3, f[+], ly0, v0, n0, stage II B). Immunohistochemically, the specimens tested positive for salivary gland amylase, and negative for collagen type IV , ER, PgR, and HER2. We administered UFT as adjuvant chemotherapy. Eight months after surgery, local recurrence was observed. ACC of the breast is rare, and has been reported to have a good prognosis. Further investigations are needed to elucidate its true histogenesis the appropriate treatment.

PMID: 28133208 [PubMed - indexed for MEDLINE]

Lipocalin-2 Promotes Pancreatic Ductal Adenocarcinoma by Regulating Inflammation in the Tumor Microenvironment.

Fetched: September 8th, 2017, 5:00am GMT

Lipocalin-2 Promotes Pancreatic Ductal Adenocarcinoma by Regulating Inflammation in the Tumor Microenvironment.

Cancer Res. 2017 May 15;77(10):2647-2660

Authors: Gomez-Chou SB, Swidnicka-Siergiejko AK, Badi N, Chavez-Tomar M, Lesinski GB, Bekaii-Saab T, Farren MR, Mace TA, Schmidt C, Liu Y, Deng D, Hwang RF, Zhou L, Moore T, Chatterjee D, Wang H, Leng X, Arlinghaus RB, Logsdon CD, Cruz-Monserrate Z

Abstract
Lipocalin-2 (LCN2) promotes malignant development in many cancer types. LCN2 is upregulated in patients with pancreatic ductal adenocarcinoma (PDAC) and in obese individuals, but whether it contributes to PDAC development is unclear. In this study, we investigated the effects of Lcn2 depletion on diet-induced obesity, inflammation, and PDAC development. Mice with acinar cell-specific expression of Kras(G12D) were crossed with Lcn2-depleted animals and fed isocaloric diets with varying amounts of fat content. Pancreas were collected and analyzed for inflammation, pancreatic intraepithelial neoplasia (PanIN), and PDAC. We also used a syngeneic orthotopic PDAC mouse model to study tumor growth in the presence or absence of Lcn2 expression. In addition, to understand the mechanistic role of how LCN2 could be mediating PDAC, we studied LCN2 and its specific receptor solute carrier family 22 member 17 (SLC22A17) in human pancreatic cancer stellate cells (PSC), key mediators of the PDAC stroma. Depletion of Lcn2 diminished extracellular matrix deposition, immune cell infiltration, PanIN formation, and tumor growth. Notably, it also increased survival in both obesity-driven and syngeneic orthotopic PDAC mouse models. LCN2 modulated the secretion of proinflammatory cytokines in PSC of the PDAC tumor microenvironment, whereas downregulation of LCN2-specific receptor SLC22A17 blocked these effects. Our results reveal how LCN2 acts in the tumor microenvironment links obesity, inflammation, and PDAC development. Cancer Res; 77(10); 2647-60. ©2017 AACR.

PMID: 28249896 [PubMed - indexed for MEDLINE]

Metaplasia: tissue injury adaptation and a precursor to the dysplasia-cancer sequence.

Fetched: September 3rd, 2017, 5:00am GMT

Metaplasia: tissue injury adaptation and a precursor to the dysplasia-cancer sequence.

Nat Rev Cancer. 2017 Sep 01;:

Authors: Giroux V, Rustgi AK

Abstract
Metaplasia is the replacement of one differentiated somatic cell type with another differentiated somatic cell type in the same tissue. Typically, metaplasia is triggered by environmental stimuli, which may act in concert with the deleterious effects of microorganisms and inflammation. The cell of origin for intestinal metaplasia in the oesophagus and stomach and for pancreatic acinar-ductal metaplasia has been posited through genetic mouse models and lineage tracing but has not been identified in other types of metaplasia, such as squamous metaplasia. A hallmark of metaplasia is a change in cellular identity, and this process can be regulated by transcription factors that initiate and/or maintain cellular identity, perhaps in concert with epigenetic reprogramming. Universally, metaplasia is a precursor to low-grade dysplasia, which can culminate in high-grade dysplasia and carcinoma. Improved clinical screening for and surveillance of metaplasia might lead to better prevention or early detection of dysplasia and cancer.

PMID: 28860646 [PubMed - as supplied by publisher]

Permalink for 'Association of PD-L1 expression and PD-L1 gene polymorphism with poor prognosis in lung adenocarcinoma and squamous cell carcinoma.'

Association of PD-L1 expression and PD-L1 gene polymorphism with poor prognosis in lung adenocarcinoma and squamous cell carcinoma.

Fetched: September 1st, 2017, 5:00am GMT

Association of PD-L1 expression and PD-L1 gene polymorphism with poor prognosis in lung adenocarcinoma and squamous cell carcinoma.

Hum Pathol. 2017 Aug 26;:

Authors: Yeo MK, Choi SY, Seong IO, Suh KS, Kim JM, Kim KH

Abstract
Programmed cell death 1 receptor (PD-1)/programmed death-1 ligand-1 (PD-L1) interaction has been linked to tumor immune evasion. PD-L1 expression has been indicated in identifying non-small cell lung carcinoma (NSCLC) patients for treatment with anti-PD-1 or anti-PD-L1 therapy. The goal of this study was to evaluate the clinicopathologic values of PD-L1 expression and single nucleotide polymorphisms (SNPs) in the PD-L1 gene in lung adenocarcinoma (ADC) and squamous cell carcinoma (SqCC). The 147 NSCLC tissues consisted of 84 samples of ADC and 63 samples of SqCC. All tissue microarray paraffin blocks were used for PD-L1 immunohistochemical assays with 22C3, SP263 and SP142 clones. Three SNPs in the PD-L1 gene, rs4143815, rs822336, and rs822337, were genotyped using SNP pyrosequencing. The PD-L1 expression was significantly higher in SqCC than in ADC. Among ADCs, PD-L1 expression was significantly higher in papillary and solid types than in lepidic and acinar types. Statistical associations of the PD-L1 expression with a shorter disease-free survival outcome and lymph node metastasis in the ADCs were found but no associations in SqCCs. Among the three SNPs, the rs4143815 genotype CC was statistically associated with positive a 22C3 PD-L1 labelling in NSCLCs. The rs4143815 genotype GG instead showed a trend of shorter survival outcomes but did not reach statistical significance in the ADCs. Our results showed a significantly higher prevalence of positive PD-L1 expression in lung SqCC than in ADC. However, the PD-L1 expression and rs4143815 genotype GG might be useful for the prediction of poor prognosis in lung ADC cases.

PMID: 28851662 [PubMed - as supplied by publisher]

Permalink for 'GNAS(R201H) and Kras(G12D) cooperate to promote murine pancreatic tumorigenesis recapitulating human intraductal papillary mucinous neoplasm.'

GNAS(R201H) and Kras(G12D) cooperate to promote murine pancreatic tumorigenesis recapitulating human intraductal papillary mucinous neoplasm.

Fetched: September 1st, 2017, 5:00am GMT

GNAS(R201H) and Kras(G12D) cooperate to promote murine pancreatic tumorigenesis recapitulating human intraductal papillary mucinous neoplasm.

Oncogene. 2016 May 05;35(18):2407-12

Authors: Taki K, Ohmuraya M, Tanji E, Komatsu H, Hashimoto D, Semba K, Araki K, Kawaguchi Y, Baba H, Furukawa T

Abstract
Intraductal papillary mucinous neoplasm (IPMN), the most common pancreatic cystic neoplasm, is known to progress to invasive ductal adenocarcinoma. IPMNs commonly harbor activating somatic mutations in GNAS and KRAS, primarily GNAS(R201H) and KRAS(G12D). GNAS encodes the stimulatory G-protein α subunit (Gsα) that mediates a stimulatory signal to adenylyl cyclase to produce cyclic adenosine monophosphate (cAMP), subsequently activating cAMP-dependent protein kinase A. The GNAS(R201H) mutation results in constitutive activation of Gsα. To study the potential role of GNAS in pancreatic tumorigenesis in vivo, we generated lines of transgenic mice in which the transgene consisted of Lox-STOP-Lox (LSL)-GNAS(R201H) under the control of the CAG promoter (Tg(CAG-LSL-GNAS)). These mice were crossed with pancreatic transcription factor 1a (Ptf1a)-Cre mice (Ptf1a(Cre/+)), generating Tg(CAG-LSL-GNAS);Ptf1a(Cre/+) mice. This mouse line showed elevated cAMP levels, small dilated tubular complex formation, loss of acinar cells and fibrosis in the pancreas; however, no macroscopic tumorigenesis was apparent by 2 months of age. We then crossed Tg(CAG-LSL-GNAS);Ptf1a(Cre/+) mice with LSL-Kras(G12D) mice, generating Tg(CAG-LSL-GNAS);LSL-Kras(G12D);Ptf1a(Cre/+) mice. We used these mice to investigate a possible cooperative effect of GNAS(R201H) and Kras(G12D) in pancreatic tumorigenesis. Within 5 weeks, Tg(CAG-LSL-GNAS);LSL-Kras(G12D);Ptf1a(Cre/+) mice developed a cystic tumor consisting of marked dilated ducts lined with papillary dysplastic epithelia in the pancreas, which closely mimicked the human IPMN. Our data strongly suggest that activating mutations in GNAS and Kras cooperatively promote murine pancreatic tumorigenesis, which recapitulates IPMN. Our mouse model may serve as a unique in vivo platform to find biomarkers and effective drugs for diseases associated with GNAS mutations.

PMID: 26257060 [PubMed - indexed for MEDLINE]

Insulinoma-associated protein 1 (INSM1) is a useful marker for pancreatic neuroendocrine tumor.

Fetched: August 31st, 2017, 5:00am GMT

Insulinoma-associated protein 1 (INSM1) is a useful marker for pancreatic neuroendocrine tumor.

Med Mol Morphol. 2017 Aug 28;:

Authors: Tanigawa M, Nakayama M, Taira T, Hattori S, Mihara Y, Kondo R, Kusano H, Nakamura K, Abe Y, Ishida Y, Okabe Y, Hisaka T, Okuda K, Fujino K, Ito T, Kawahara A, Naito Y, Yamaguchi R, Akiba J, Akagi Y, Yano H

Abstract
Insulinoma-associated protein 1 (INSM1) is an important biomarker of Achaete-scute homolog-like 1-driven pathways. For diagnosis of pancreatic neuroendocrine tumors (PanNET), chromogranin A (CGA), synaptophysin (SYP), and neural cell adhesion molecule (NCAM) were also considered as potential biomarkers. However, it is often difficult to diagnose it immunohistochemically. Hence, we examined the expression pattern of INSM1 in pancreatic solid tumors. We detected INSM1, CGA, SYP, and NCAM immunohistochemically, in 27 cases of NET [pure type: 25 cases, mixed adenoneuroendocrine carcinoma (MANEC): 2 cases]. We included 5 cases of solid-pseudopapillary neoplasm (SPN), 7 cases of acinar cell carcinoma (ACC), and 15 cases of pancreatic ductal adenocarcinoma (PDAC) as the control group. Nuclear expression of INSM1 was found in all PanNET pure type cases. However, expression of INSM1 was negative in PDAC, ACC, and SPN in all cases, whereas faint expression was seen in the cytoplasm from SPN. MANEC comprises of two components: neuroendocrine carcinoma and adenocarcinoma components. The NET component was positive for INSM1 expression, whereas the PDAC component does not express INSM1, which aids in distinguishing these components. Our results suggest that INSM1 is a useful immunohistochemical marker for diagnosing pancreatic neuroendocrine tumor.

PMID: 28849340 [PubMed - as supplied by publisher]

Lunatic Fringe is a potent tumor suppressor in Kras-initiated pancreatic cancer.

Fetched: August 30th, 2017, 5:00am GMT

Lunatic Fringe is a potent tumor suppressor in Kras-initiated pancreatic cancer.

Oncogene. 2016 May 12;35(19):2485-95

Authors: Zhang S, Chung WC, Xu K

Abstract
Notch controls pancreatic differentiation during development and is reactivated in pancreatic cancer. In recent years, the importance of Notch signaling in pancreatic tumorigenesis has become increasingly evident; however, it remains unclear how Notch activities are regulated in this context. Here we report differential regulation of Notch receptors by Lunatic Fringe (Lfng), which encodes an O-fucosylpeptide 3-β-N-acetylglucosaminyltransferase known to modify epidermal growth factor repeats in the Notch extracellular domain, during pathogenesis of Kras-induced pancreatic ductal adenocarcinoma (PDAC). We show that Lfng is uniquely expressed in a subset of acinar cells in the adult pancreas. Deletion of Lfng in the Kras(LSL-G12D/+);Pdx1-Cre mouse model caused increased activation of Notch3 throughout PDAC initiation and progression, and Notch1 after the onset of disease, associated with marked upregulation of Notch target gene Hes1. Deletion of Lfng also resulted in accumulation of Aldh1-positive cell population. We found that loss of Lfng significantly accelerated Kras-initiated PDAC development and shortened survival of the PDAC mice. Interestingly, Lfng-deficient tumors showed a propensity for a poorly differentiated state with features of epithelial-to-mesenchymal transition. Likewise, knockdown of LFNG in human PDAC cell lines caused elevated Notch activation, associated with either accelerated cell proliferation or expanded Aldh1-positive cell population. Deletion of Lfng resulted in downregulation of Tgfb1, Tgfb2 and Tgfbr2 expression in the wild-type pancreas at all ages examined, and in the Kras(LSL-G12D/+);Pdx1-Cre pancreas after PDAC onset, as well as reduced phospho-Smad2 levels in pancreatic tumors. We provide evidence that Lfng regulates transforming growth factor (TGF)-β signaling through Notch-mediated transcriptional repression of TGF-β pathway genes. Taken together, our results reveal a potent tumor-suppressive function for Lfng and crosstalk between Notch and TGF-β pathways in the pancreas, which provides new insight into initiation of PDAC and signals involved in disease progression.

PMID: 26279302 [PubMed - indexed for MEDLINE]

Pancreatic Panniculitis and Polyarthritis.

Fetched: August 29th, 2017, 5:00am GMT

Pancreatic Panniculitis and Polyarthritis.

Curr Rheumatol Rep. 2017 Aug 26;19(10):62

Authors: Zundler S, Strobel D, Manger B, Neurath MF, Wildner D

Abstract
PURPOSE OF REVIEW: Polyarthritis can have numerous reasons and may thus constitute a challenge for differential diagnosis. One rare potential reason for sterile polyarthritis is underlying pancreatic disease with systemic hyperlipasemia, most often accompanied by painful skin lesions caused by a subcutaneous inflammatory process known as panniculitis. Systematic evidence on pancreatic panniculitis and polyarthritis is limited, particularly regarding its feature as facultative paraneoplasia with underlying intra- or even extra-pancreatic malignancy. Therefore, we will summarize the current knowledge about this orphan disease including epidemiological, pathophysiological, diagnostic, and treatment aspects in the present review.
RECENT FINDINGS: Although direct evidence is lacking, it is highly probable that pancreatic polyarthritis and panniculitis are caused by peripheral lipolytic activity of lipase systemically circulating due to benign (e.g., acute or chronic pancreatitis) or malign (e.g., acinar cell carcinoma (ACC) or adenocarcinoma) pancreatic disease. In the latter case, pancreatic polyarthritis and panniculitis are associated with poor outcome. Pancreatic polyarthritis and panniculitis should always be included into diagnostic considerations, and once suspected, a thorough work-up to identify the underlying disease has to be performed.

PMID: 28844095 [PubMed - in process]

Permalink for 'Recurrent rearrangements of the Myb/SANT-like DNA-binding domain containing 3 gene (MSANTD3) in salivary gland acinic cell carcinoma.'

Recurrent rearrangements of the Myb/SANT-like DNA-binding domain containing 3 gene (MSANTD3) in salivary gland acinic cell carcinoma.

Fetched: August 20th, 2017, 5:00am GMT

Recurrent rearrangements of the Myb/SANT-like DNA-binding domain containing 3 gene (MSANTD3) in salivary gland acinic cell carcinoma.

PLoS One. 2017;12(2):e0171265

Authors: Barasch N, Gong X, Kwei KA, Varma S, Biscocho J, Qu K, Xiao N, Lipsick JS, Pelham RJ, West RB, Pollack JR

Abstract
Pathogenic gene fusions have been identified in several histologic types of salivary gland neoplasia, but not previously in acinic cell carcinoma (AcCC). To discover novel gene fusions, we performed whole-transcriptome sequencing surveys of three AcCC archival cases. In one specimen we identified a novel HTN3-MSANTD3 gene fusion, and in another a novel PRB3-ZNF217 gene fusion. The structure of both fusions was consistent with the promoter of the 5' partner (HTN3 or PRB3), both highly expressed salivary gland genes, driving overexpression of full-length MSANTD3 or ZNF217. By fluorescence in situ hybridization of an expanded AcCC case series, we observed MSANTD3 rearrangements altogether in 3 of 20 evaluable cases (15%), but found no additional ZNF217 rearrangements. MSANTD3 encodes a previously uncharacterized Myb/SANT domain-containing protein. Immunohistochemical staining demonstrated diffuse nuclear MSANTD3 expression in 8 of 27 AcCC cases (30%), including the three cases with MSANTD3 rearrangement. MSANTD3 displayed heterogeneous expression in normal salivary ductal epithelium, as well as among other histologic types of salivary gland cancer though without evidence of translocation. In a broader survey, MSANTD3 showed variable expression across a wide range of normal and neoplastic human tissue specimens. In preliminary functional studies, engineered MSANTD3 overexpression in rodent salivary gland epithelial cells did not enhance cell proliferation, but led to significant upregulation of gene sets involved in protein synthesis. Our findings newly identify MSANTD3 rearrangement as a recurrent event in salivary gland AcCC, providing new insight into disease pathogenesis, and identifying a putative novel human oncogene.

PMID: 28212443 [PubMed - indexed for MEDLINE]

Hes1 Controls Exocrine Cell Plasticity and Restricts Development of Pancreatic Ductal Adenocarcinoma in a Mouse Model.

Fetched: August 18th, 2017, 5:00am GMT

Hes1 Controls Exocrine Cell Plasticity and Restricts Development of Pancreatic Ductal Adenocarcinoma in a Mouse Model.

Am J Pathol. 2016 Nov;186(11):2934-2944

Authors: Hidalgo-Sastre A, Brodylo RL, Lubeseder-Martellato C, Sipos B, Steiger K, Lee M, von Figura G, Grünwald B, Zhong S, Trajkovic-Arsic M, Neff F, Schmid RM, Siveke JT

Abstract
Perturbation of pancreatic acinar cell state can lead to acinar-to-ductal metaplasia (ADM), a precursor lesion to the development of pancreatic ductal adenocarcinoma (PDAC). In the pancreas, Notch signaling is active both during development and in adult cellular differentiation processes. Hes1, a key downstream target of the Notch signaling pathway, is expressed in the centroacinar compartment of the adult pancreas as well as in both preneoplastic and malignant lesions. In this study, we used a murine genetic in vivo approach to ablate Hes1 in pancreatic progenitor cells (Ptf1a(+/Cre); Hes1(fl/fl)). Using this model, we studied the role of Hes1 in both acinar cell plasticity and pancreatic regeneration after caerulein-induced pancreatitis and in Kras(G12D)-driven PDAC development. We show that, although pancreatic development is not perturbed on the deletion of Hes1, terminal acinar differentiation in the adult pancreas is compromised. Moreover, the loss of Hes1 leads to the impaired regeneration of the exocrine compartment, accelerated fatty metaplasia, and persistent ADM after acute caerulein-induced pancreatitis. In Kras(G12D)-driven carcinogenesis, Hes1 ablation resulted in increased ADM, decreased formation of high-grade pancreatic intraepithelial neoplasias, and accelerated development of PDAC with shortened survival time. In conclusion, Hes1 plays a key role in acinar cell integrity and plasticity on cellular insults. Furthermore, Hes1 is an essential component of the pancreatic intraepithelial neoplasias-to-PDAC route in Kras(G12D)-driven mouse pancreatic carcinogenesis.

PMID: 27639167 [PubMed - indexed for MEDLINE]

Cytologic characteristics of circulating epithelioid cells in pancreatic disease.

Fetched: August 16th, 2017, 5:00am GMT

Cytologic characteristics of circulating epithelioid cells in pancreatic disease.

Cancer. 2017 May;125(5):332-340

Authors: Rosenbaum MW, Cauley CE, Kulemann B, Liss AS, Castillo CF, Warshaw AL, Lillemoe KD, Thayer SP, Pitman MB

Abstract
BACKGROUND: Circulating epithelioid cells (CECs), also known as circulating tumor, circulating cancer, circulating epithelial, or circulating nonhematologic cells, are a prognostic factor in various malignancies that can be isolated via various protocols. In the current study, the authors analyzed the cytomorphologic characteristics of CECs isolated by size in a cohort of patients with benign and malignant pancreatic diseases to determine whether cytomorphological features could predict CEC origin.
METHODS: Blood samples were collected from 9 healthy controls and 171 patients with pancreatic disease who were presenting for surgical evaluation before treatment. Blood was processed with the ScreenCell size-based filtration device. Evaluable CECs were analyzed in a blinded fashion for cytomorphologic characteristics, including cellularity; nucleoli; nuclear size, irregularity, variability, and hyperchromasia; and nuclear-to-cytoplasmic ratio. Statistical differences between variables were analyzed via the Fisher exact test.
RESULTS: No CECs were identified among the 9 normal healthy controls. Of the 115 patients with CECs (positive or suspicious for), 25 had nonmalignant disease and 90 had malignancy. There were no significant differences in any of the cytologic criteria noted between groups divided by benign versus malignant, neoplastic versus nonneoplastic, or pancreatic ductal adenocarcinoma versus neuroendocrine tumor.
CONCLUSIONS: CECs were observed in patients with malignant and nonmalignant pancreatic disease, but not in healthy controls. There were no morphologic differences observed between cells from different pancreatic diseases, suggesting that numerous conditions may be associated with CECs in the circulation and that care must be taken not to overinterpret cells identified by cytomorphology as indicative of circulating tumor cells of pancreatic cancer. Additional studies are required to determine the origin and clinical significance of these cells. Cancer Cytopathol 2017;125:332-340. © 2017 American Cancer Society.

PMID: 28257167 [PubMed - indexed for MEDLINE]

A Prediction Model for ROS1-Rearranged Lung Adenocarcinomas based on Histologic Features.

Fetched: August 13th, 2017, 5:00am GMT

A Prediction Model for ROS1-Rearranged Lung Adenocarcinomas based on Histologic Features.

PLoS One. 2016;11(9):e0161861

Authors: Zhou J, Zhao J, Zheng J, Kong M, Sun K, Wang B, Chen X, Ding W, Zhou J

Abstract
AIMS: To identify the clinical and histological characteristics of ROS1-rearranged non-small-cell lung carcinomas (NSCLCs) and build a prediction model to prescreen suitable patients for molecular testing.
METHODS AND RESULTS: We identified 27 cases of ROS1-rearranged lung adenocarcinomas in 1165 patients with NSCLCs confirmed by real-time PCR and FISH and performed univariate and multivariate analyses to identify predictive factors associated with ROS1 rearrangement and finally developed prediction model. Detected with ROS1 immunochemistry, 59 cases of 1165 patients had a certain degree of ROS1 expression. Among these cases, 19 cases (68%, 19/28) with 3+ and 8 cases (47%, 8/17) with 2+ staining were ROS1 rearrangement verified by real-time PCR and FISH. In the resected group, the acinar-predominant growth pattern was the most commonly observed (57%, 8/14), while in the biopsy group, solid patterns were the most frequently observed (78%, 7/13). Based on multiple logistic regression analysis, we determined that female sex, cribriform structure and the presence of psammoma body were the three most powerful indicators of ROS1 rearrangement, and we have developed a predictive model for the presence of ROS1 rearrangements in lung adenocarcinomas.
CONCLUSIONS: Female, cribriform structure and presence of psammoma body were the three most powerful indicator of ROS1 rearrangement status, and predictive formula was helpful in screening ROS1-rearranged NSCLC, especially for ROS1 immunochemistry equivocal cases.

PMID: 27648828 [PubMed - indexed for MEDLINE]

Context-Dependent Epigenetic Regulation of Nuclear Factor of Activated T Cells 1 in Pancreatic Plasticity.

Fetched: August 10th, 2017, 5:00am GMT

Context-Dependent Epigenetic Regulation of Nuclear Factor of Activated T Cells 1 in Pancreatic Plasticity.

Gastroenterology. 2017 May;152(6):1507-1520.e15

Authors: Chen NM, Neesse A, Dyck ML, Steuber B, Koenig AO, Lubeseder-Martellato C, Winter T, Forster T, Bohnenberger H, Kitz J, Reuter-Jessen K, Griesmann H, Gaedcke J, Grade M, Zhang JS, Tsai WC, Siveke J, Schildhaus HU, Ströbel P, Johnsen SA, Ellenrieder V, Hessmann E

Abstract
BACKGROUND & AIMS: The ability of exocrine pancreatic cells to change the cellular phenotype is required for tissue regeneration upon injury, but also contributes to their malignant transformation and tumor progression. We investigated context-dependent signaling and transcription mechanisms that determine pancreatic cell fate decisions toward regeneration and malignancy. In particular, we studied the function and regulation of the inflammatory transcription factor nuclear factor of activated T cells 1 (NFATC1) in pancreatic cell plasticity and tissue adaptation.
METHODS: We analyzed cell plasticity during pancreatic regeneration and transformation in mice with pancreas-specific expression of a constitutively active form of NFATC1, or depletion of enhancer of zeste 2 homologue 2 (EZH2), in the context of wild-type or constitutively activate Kras, respectively. Acute and chronic pancreatitis were induced by intraperitoneal injection of caerulein. EZH2-dependent regulation of NFATC1 expression was studied in mouse in human pancreatic tissue and cells by immunohistochemistry, immunoblotting, and quantitative reverse transcription polymerase chain reaction. We used genetic and pharmacologic approaches of EZH2 and NFATC1 inhibition to study the consequences of pathway disruption on pancreatic morphology and function. Epigenetic modifications on the NFATC1 gene were investigated by chromatin immunoprecipitation assays.
RESULTS: NFATC1 was rapidly and transiently induced in early adaptation to acinar cell injury in human samples and in mice, where it promoted acinar cell transdifferentiation and blocked proliferation of metaplastic pancreatic cells. However, in late stages of regeneration, Nfatc1 was epigenetically silenced by EZH2-dependent histone methylation, to enable acinar cell redifferentiation and prevent organ atrophy and exocrine insufficiency. In contrast, oncogenic activation of KRAS signaling in pancreatic ductal adenocarcinoma cells reversed the EZH2-dependent effects on the NFATC1 gene and was required for EZH2-mediated transcriptional activation of NFATC1.
CONCLUSIONS: In studies of human and mouse pancreatic cells and tissue, we identified context-specific epigenetic regulation of NFATc1 activity as an important mechanism of pancreatic cell plasticity. Inhibitors of EZH2 might therefore interfere with oncogenic activity of NFATC1 and be used in treatment of pancreatic ductal adenocarcinoma.

PMID: 28188746 [PubMed - indexed for MEDLINE]

Hati waja - Harian Metro

Posted: August 10th, 2017, 7:14pm GMT

Harian Metro

Generation of a pancreatic cancer model using a Pdx1-Flp recombinase knock-in allele.

Fetched: September 23rd, 2017, 5:00am GMT

Generation of a pancreatic cancer model using a Pdx1-Flp recombinase knock-in allele.

PLoS One. 2017;12(9):e0184984

Authors: Wu J, Liu X, Nayak SG, Pitarresi JR, Cuitiño MC, Yu L, Hildreth BE, Thies KA, Schilling DJ, Fernandez SA, Leone G, Ostrowski MC

PMID: 28934293 [PubMed - in process]

Acinar cell carcinoma of the pancreas presenting as diffuse pancreatic enlargement: Two case reports and literature review.

Fetched: September 22nd, 2017, 5:00am GMT

Acinar cell carcinoma of the pancreas presenting as diffuse pancreatic enlargement: Two case reports and literature review.

Medicine (Baltimore). 2017 Sep;96(38):e7904

Authors: Luo Y, Hu G, Ma Y, Guo N, Li F

PMID: 28930825 [PubMed - in process]

Pancreatic acinar cell carcinoma with extensive tumor embolism at the trunk of portal vein and pancreatic intraductal infiltration.

Fetched: September 20th, 2017, 5:00am GMT

Pancreatic acinar cell carcinoma with extensive tumor embolism at the trunk of portal vein and pancreatic intraductal infiltration.

Clin J Gastroenterol. 2017 Sep 18;:

Authors: Kida A, Matsuda K, Takegoshi K, Matsuda M, Sakai A, Noda Y

PMID: 28921102 [PubMed - as supplied by publisher]

Acinar Cell Carcinoma of the Pancreas: A Case Report.

Fetched: September 20th, 2017, 5:00am GMT

Acinar Cell Carcinoma of the Pancreas: A Case Report.

Mymensingh Med J. 2017 Jul;26(3):684-688

Authors: Mohammed S, Chowdhury MM, Ullah AA, Towhid SM, Sobhan SA, Zeba IJ, Haque MM, Islam MT, Hussain M, Roy TK, Russel SR, Chowdhury AM, Alam MK

PMID: 28919629 [PubMed - in process]

HMGA1 expression levels are elevated in pancreatic intraepithelial neoplasia cells in the Ptf1a-Cre; LSL-KrasG12D transgenic mouse model of pancreatic cancer.

Fetched: September 20th, 2017, 5:00am GMT

HMGA1 expression levels are elevated in pancreatic intraepithelial neoplasia cells in the Ptf1a-Cre; LSL-KrasG12D transgenic mouse model of pancreatic cancer.

Br J Cancer. 2017 Aug 22;117(5):639-647

Authors: Veite-Schmahl MJ, Joesten WC, Kennedy MA

PMID: 28697176 [PubMed - indexed for MEDLINE]

Fhit down-regulation is an early event in pancreatic carcinogenesis.

Fetched: September 16th, 2017, 5:00am GMT

Fhit down-regulation is an early event in pancreatic carcinogenesis.

Virchows Arch. 2017 Jun;470(6):647-653

Authors: Fassan M, Rusev B, Corbo V, Gasparini P, Luchini C, Vicentini C, Mafficini A, Paiella S, Salvia R, Cataldo I, Scarpa A, Huebner K

PMID: 28289900 [PubMed - indexed for MEDLINE]

Permalink for 'Two Cases of Acinic Cell Carcinoma of Parotid Gland - Rare Papillary Cystic Variant: One Unusual Presentation in Older Male Patient.'

Two Cases of Acinic Cell Carcinoma of Parotid Gland - Rare Papillary Cystic Variant: One Unusual Presentation in Older Male Patient.

Fetched: September 15th, 2017, 5:00am GMT

Two Cases of Acinic Cell Carcinoma of Parotid Gland - Rare Papillary Cystic Variant: One Unusual Presentation in Older Male Patient.

Indian J Med Paediatr Oncol. 2017 Apr-Jun;38(2):242-244

Authors: Modi M, Patel T, Trivedi P

PMID: 28900343 [PubMed]

Ductal adenocarcinoma of the prostate: Clinical and biological profiles.

Fetched: September 13th, 2017, 5:00am GMT

Ductal adenocarcinoma of the prostate: Clinical and biological profiles.

Prostate. 2017 Sep;77(12):1242-1250

Authors: Vinceneux A, Bruyère F, Haillot O, Charles T, de la Taille A, Salomon L, Allory Y, Ouzaid I, Choudat L, Rouprêt M, Comperat E, Houede N, Beauval JB, Vourc'h P, Fromont G

PMID: 28699202 [PubMed - indexed for MEDLINE]

Histologic features of stromogenic carcinoma of the prostate (carcinomas with reactive stroma grade 3).

Fetched: September 13th, 2017, 5:00am GMT

Histologic features of stromogenic carcinoma of the prostate (carcinomas with reactive stroma grade 3).

Hum Pathol. 2017 May;63:202-211

Authors: De Vivar AD, Sayeeduddin M, Rowley D, Cubilla A, Miles B, Kadmon D, Ayala G

PMID: 28315427 [PubMed - indexed for MEDLINE]

Lipocalin-2 Promotes Pancreatic Ductal Adenocarcinoma by Regulating Inflammation in the Tumor Microenvironment.

Fetched: September 8th, 2017, 5:00am GMT

Lipocalin-2 Promotes Pancreatic Ductal Adenocarcinoma by Regulating Inflammation in the Tumor Microenvironment.

Cancer Res. 2017 May 15;77(10):2647-2660

PMID: 28249896 [PubMed - indexed for MEDLINE]

Metaplasia: tissue injury adaptation and a precursor to the dysplasia-cancer sequence.

Fetched: September 3rd, 2017, 5:00am GMT

Metaplasia: tissue injury adaptation and a precursor to the dysplasia-cancer sequence.

Nat Rev Cancer. 2017 Sep 01;:

Authors: Giroux V, Rustgi AK

PMID: 28860646 [PubMed - as supplied by publisher]

Association of PD-L1 expression and PD-L1 gene polymorphism with poor prognosis in lung adenocarcinoma and squamous cell carcinoma.

Fetched: September 1st, 2017, 5:00am GMT

Association of PD-L1 expression and PD-L1 gene polymorphism with poor prognosis in lung adenocarcinoma and squamous cell carcinoma.

Hum Pathol. 2017 Aug 26;:

Authors: Yeo MK, Choi SY, Seong IO, Suh KS, Kim JM, Kim KH

PMID: 28851662 [PubMed - as supplied by publisher]

GNAS(R201H) and Kras(G12D) cooperate to promote murine pancreatic tumorigenesis recapitulating human intraductal papillary mucinous neoplasm.

Fetched: September 1st, 2017, 5:00am GMT

GNAS(R201H) and Kras(G12D) cooperate to promote murine pancreatic tumorigenesis recapitulating human intraductal papillary mucinous neoplasm.

Oncogene. 2016 May 05;35(18):2407-12

Authors: Taki K, Ohmuraya M, Tanji E, Komatsu H, Hashimoto D, Semba K, Araki K, Kawaguchi Y, Baba H, Furukawa T

PMID: 26257060 [PubMed - indexed for MEDLINE]

Insulinoma-associated protein 1 (INSM1) is a useful marker for pancreatic neuroendocrine tumor.

Fetched: August 31st, 2017, 5:00am GMT

Insulinoma-associated protein 1 (INSM1) is a useful marker for pancreatic neuroendocrine tumor.

Med Mol Morphol. 2017 Aug 28;:

PMID: 28849340 [PubMed - as supplied by publisher]

Lunatic Fringe is a potent tumor suppressor in Kras-initiated pancreatic cancer.

Fetched: August 30th, 2017, 5:00am GMT

Lunatic Fringe is a potent tumor suppressor in Kras-initiated pancreatic cancer.

Oncogene. 2016 May 12;35(19):2485-95

Authors: Zhang S, Chung WC, Xu K

PMID: 26279302 [PubMed - indexed for MEDLINE]

Pancreatic Panniculitis and Polyarthritis.

Fetched: August 29th, 2017, 5:00am GMT

Pancreatic Panniculitis and Polyarthritis.

Curr Rheumatol Rep. 2017 Aug 26;19(10):62

Authors: Zundler S, Strobel D, Manger B, Neurath MF, Wildner D

PMID: 28844095 [PubMed - in process]

Hes1 Controls Exocrine Cell Plasticity and Restricts Development of Pancreatic Ductal Adenocarcinoma in a Mouse Model.

Fetched: August 18th, 2017, 5:00am GMT

Hes1 Controls Exocrine Cell Plasticity and Restricts Development of Pancreatic Ductal Adenocarcinoma in a Mouse Model.

Am J Pathol. 2016 Nov;186(11):2934-2944

Authors: Hidalgo-Sastre A, Brodylo RL, Lubeseder-Martellato C, Sipos B, Steiger K, Lee M, von Figura G, Grünwald B, Zhong S, Trajkovic-Arsic M, Neff F, Schmid RM, Siveke JT

PMID: 27639167 [PubMed - indexed for MEDLINE]

A systematic review including an additional pediatric case report: Pediatric cases of mammary analogue secretory carcinoma.

Fetched: August 16th, 2017, 5:00am GMT

A systematic review including an additional pediatric case report: Pediatric cases of mammary analogue secretory carcinoma.

Int J Pediatr Otorhinolaryngol. 2017 Sep;100:187-193

Authors: Ngouajio AL, Drejet SM, Phillips DR, Summerlin DJ, Dahl JP

Abstract
IMPORTANCE: Mammary Analogue Secretory Carcinoma (MASC) is a newly characterized salivary gland carcinoma resembling secretory carcinoma of the breast. Prior to being described, MASC was most commonly misdiagnosed as Acinic Cell Carcinoma. Though MASC is predominantly an adult neoplasm, cases have been reported in the pediatric population. Reporting and summarizing of known cases is imperative to understand the prognosis and clinical behavior of MASC.
OBJECTIVE: EVIDENCE REVIEW: Web of Science, Medline, EMBASE, and The Cochrane Library were searched for studies that included pediatric cases of MASC. Data on clinical presentation, diagnosis and management, and pathology were collected from all pediatric cases.
FINDINGS: CONCLUSIONS AND RELEVANCE: Since the first case of MASC in the pediatric population was described in 2011, only 12 cases, including this one, have been described in the literature. With this paucity of information, much remains unknown regarding this new pathologic diagnosis. The collection of clinical outcomes data of children with MASC is needed to better understand the behavior of this malignancy as well as determine optimal treatment regimens.

PMID: 28802370 [PubMed - in process]

Cytologic characteristics of circulating epithelioid cells in pancreatic disease.

Fetched: August 16th, 2017, 5:00am GMT

Cytologic characteristics of circulating epithelioid cells in pancreatic disease.

Cancer. 2017 May;125(5):332-340

Authors: Rosenbaum MW, Cauley CE, Kulemann B, Liss AS, Castillo CF, Warshaw AL, Lillemoe KD, Thayer SP, Pitman MB

PMID: 28257167 [PubMed - indexed for MEDLINE]

Metastatic Acinic Cell Carcinoma to the Vagina: A First Reported Case.

Fetched: August 13th, 2017, 5:00am GMT

Metastatic Acinic Cell Carcinoma to the Vagina: A First Reported Case.

Appl Immunohistochem Mol Morphol. 2017 Aug 09;:

Authors: Hom M, Fong A, Sanford M, Mhawech-Fauceglia P

Abstract
This is a case of a 62-year-old woman with a remote history of acinic cell carcinoma of the parotid gland, who presented with a palpable vaginal mass, anterior vaginal wall prolapse, and stress urinary incontinence. A 2 cm firm mobile mass on the anterior vaginal wall was found on clinical examination. A computed tomographic scan revealed a mass between the vaginal vault and bladder that was eventually surgically excised. The histology, supported by the immunohistochemistry, revealed metastatic acinic cell carcinoma to the vagina after 37 years of her initial diagnosis. This is the first reported case in the literature to occur in the vagina.

PMID: 28800013 [PubMed - as supplied by publisher]

A Prediction Model for ROS1-Rearranged Lung Adenocarcinomas based on Histologic Features.

Fetched: August 13th, 2017, 5:00am GMT

A Prediction Model for ROS1-Rearranged Lung Adenocarcinomas based on Histologic Features.

PLoS One. 2016;11(9):e0161861

Authors: Zhou J, Zhao J, Zheng J, Kong M, Sun K, Wang B, Chen X, Ding W, Zhou J

PMID: 27648828 [PubMed - indexed for MEDLINE]

Context-Dependent Epigenetic Regulation of Nuclear Factor of Activated T Cells 1 in Pancreatic Plasticity.

Fetched: August 10th, 2017, 5:00am GMT

Context-Dependent Epigenetic Regulation of Nuclear Factor of Activated T Cells 1 in Pancreatic Plasticity.

Gastroenterology. 2017 May;152(6):1507-1520.e15

PMID: 28188746 [PubMed - indexed for MEDLINE]

KELLIE'S KREW - The Daily Record (registration)

Posted: August 29th, 2017, 7:49am GMT

The Daily Record (registration)

KELLIE'S KREW
The Daily Record (registration)
Almost $34,000 was raised which will go to benefit those suffering from the rare cancer Adenoid Cystic Carcinoma (ACC). It is held in honor of the late Kellie Hinshaw. Cancer survivor Beth Wehlage is near the finish line as she takes part in the ninth ...

Cancer of the Middle and Inner Ear - News-Medical.net

Posted: August 25th, 2017, 2:09am GMT

Cancer of the Middle and Inner Ear
News-Medical.net
Adenoid Cystic Carcinoma (ACC): A unique and rare cancer form, ACC is unpredictable. The growth of the cancer is generally slow; however, it is gradual and progressive over time. Most often, it occurs in the oral cavity (primary tumors); however, it ...

Emotional Film Follows Gabe Grunewald's Track Season While Battling Cancer - Competitor Running (blog)

Posted: August 22nd, 2017, 3:31pm GMT

Competitor Running (blog)

Emotional Film Follows Gabe Grunewald's Track Season While Battling Cancer
Competitor Running (blog)
This was her third time fighting Adenoid Cystic Carcinoma, a rare form of cancer. Although the surgery went well, Grunewald was unfortunately not cancer-free for long. A post-op scan this past March revealed many small tumors had once again formed on ...

Clinical prognostic factors of salivary adenoid cystic carcinoma: A single-center analysis of 61 patients.

Fetched: September 24th, 2017, 5:00am GMT

Clinical prognostic factors of salivary adenoid cystic carcinoma: A single-center analysis of 61 patients.

J Craniomaxillofac Surg. 2017 Aug 10;:

Authors: Cordesmeyer R, Schliephake H, Kauffmann P, Tröltzsch M, Laskawi R, Ströbel P, Bremmer F

Abstract
PURPOSE: Adenoid cystic carcinomas are rare malignant tumors of the salivary glands. They are characterized by a high rate of local recurrence, late distant metastasis and a poor disease-free survival. In this study, we analyzed a series of 61 patients who were all treated at the University of Göttingen over a period of 21.0 years.
MATERIALS AND METHODS: In all 61 patients with salivary adenoid cystic carcinoma, clinical data, demographic data, risk factors, tumor location, tumor stage, status of surgical margin, surgical treatment, postoperative radiotherapy and follow-up interval were assessed.
RESULTS: The overall survival (OS) and the disease-free survival (DFS) of patients who underwent surgery and those who underwent combined surgery with radiotherapy showed no significant differences. Neither did the T-stage (T1/T2 vs. T3/T4) show significant differences in OS and DFS. Only the status of the surgical margin was significantly associated with a longer OS and a longer DFS.
CONCLUSION: The present results confirm that the radical surgical resection with clear tumor-free margins is the most important predictor for a longer survival. Adjuvant radiotherapy should be discussed from case to case, but should not be seen as an absolute prognostic factor for OS.

PMID: 28935487 [PubMed - as supplied by publisher]

Resection of the Tracheobronchial Bifurcation With Complete Preservation of Lung Parenchyma.

Fetched: September 24th, 2017, 5:00am GMT

Resection of the Tracheobronchial Bifurcation With Complete Preservation of Lung Parenchyma.

Ann Thorac Surg. 2017 Sep 18;:

Authors: Stamatis G, Fechner S, Rocha M, Weinreich G

Abstract
BACKGROUND: The resection of the tracheobronchial bifurcation with complete preservation of lung parenchyma remains a challenge owing to the limited indications for surgery, anesthesiologic management, operative technique, and postoperative course. The aim of this retrospective study was to evaluate factors influencing the perioperative course and long-term survival.
METHODS: Between 1989 and 2014, 19 patients underwent a resection of the distal trachea and carina with complete preservation of lung tissue, 16 for malignant tumors (7 adenoid cystic carcinomas, 3 carcinoid tumors, 3 mucoepidermoid tumors, 2 squamous cell carcinomas, and 1 small cell carcinomas), 2 for inflammatory stenosis, and 1 after a complex traumatic rupture.
RESULTS: Surgical approach was posterolateral thoracotomy in 17 patients and median sternotomy in 2. In 16 patients, end-to-end anastomosis was performed, and in 3 patients, combined end-to-end and side-to-end anastomosis were performed. The operative mortality was 0%, the perioperative complication rate was 26.3%. Six patients with adenoid cystic carcinoma and all patients with lung carcinoma received adjuvant radiotherapy; only 1 patient with small cell lung cancer had chemotherapy before surgery. Long-term results are excellent in patients with benign disease, typical and atypical carcinoid tumor, mucoepidermoid carcinoma, and in most patients with adenoid cystic carcinoma. Two patients with lung cancer died 28 and 45 months after surgery, and 1 patient with adenoid cystic carcinoma died 75 months after surgery.
CONCLUSIONS: Resection of the tracheobronchial bifurcation with complete preservation of lung indicated for selected patients with local tumor growth at the distal trachea and carina provides low perioperative mortality and complications and results in long-term survival rates.

PMID: 28935346 [PubMed - as supplied by publisher]

Cytological spectrum of salivary gland lesions and their correlation with epidemiological parameters.

Fetched: September 23rd, 2017, 5:00am GMT

Cytological spectrum of salivary gland lesions and their correlation with epidemiological parameters.

J Oral Maxillofac Pathol. 2017 May-Aug;21(2):203-210

Authors: Sandhu VK, Sharma U, Singh N, Puri A

Abstract
BACKGROUND: The role fine-needle aspiration (FNA) in the diagnosis of salivary gland lesions has evolved over the years. Although clinical and radiological parameters help to narrow the differential diagnosis the tissue diagnosis still remains the gold standard.
MATERIALS AND METHODS: This study is from January 2013 to December 2015 in our Department of Pathology where 170 salivary gland lesions were aspirated. The aim of the present study was to analyze adequacy rate in relation to the size of lesion and to evaluate varied cytological spectrum of salivary gland lesions with emphasis on differential diagnosis and to correlate cytological diagnosis with age, gender and anatomical site.
RESULTS: The 170 cytological smears were categorized into two groups: Group 1 adequate aspirations (88.2%), Group 2 inadequate aspirations (11.7%). The adequate aspirations were subdivided as neoplastic (53.33%) and nonneoplastic (46.66%). The distribution of the various neoplastic lesions (80; 53.33%) were 66 (82.5%) benign, 12 (15%) were malignant and 2 (2.5%) were suspicious of malignancy. Among benign neoplasms, the pleomorphic adenoma (62; 93.3%) was the most frequent followed by Warthins tumor (4; 6%). The most common malignant neoplasms were adenoid cystic carcinoma (6; 50%), followed by mucoepidermoid carcinoma (4; 33.3%), malignant lymphoma (1; 8.3%) and metastatic carcinomatous deposits (1; 8.3%). In two cases, cytological picture indicated suspicion for malignancy however specific tumor typing could not be done. The neoplasms occurred more frequently in the parotid gland (65%), followed by submandibular gland (21.3%) and minor salivary glands (13.8%). The nonneoplastic lesions (70) included 68.6% cases of chronic sialadenitis, 17.1% cases were reported as mucocele, 11.4% cases of acute sialadenitis 2.9% cases as tubercular granulomas.
CONCLUSION: FNA cytology provides useful information on the management of salivary gland lesions and prevents unnecessary surgery in cases of nonneoplastic lesions and identification of malignancy helps the surgeon in deciding type and extent of surgery.

PMID: 28932028 [PubMed]

Conical Modification of Forearm Free Flaps for Single-Stage Reconstruction After Total Orbital Exenteration.

Fetched: September 22nd, 2017, 5:00am GMT

Conical Modification of Forearm Free Flaps for Single-Stage Reconstruction After Total Orbital Exenteration.

J Craniofac Surg. 2017 Sep 19;:

Authors: Purnell CA, Vaca EE, Ellis MF

Abstract
BACKGROUND: Orbital exenteration is a significant reconstructive challenge for plastic surgeons. Options described for these defects range from healing by secondary intention to free tissue transfer. The authors present our preferred reconstruction of orbital defects with free forearm-based flaps, which provides quality soft tissue and orbital contouring in 1 stage to consistently allow placement of bone-anchored implants for eventual orbital prosthesis.
METHODS: This conical shape of the orbit is deconstructed into a "Pac-Man" type shaped flap that leaves adequate depth for a prosthesis. A retrospective chart review was performed of 2 separate patients receiving orbital exenteration reconstruction by the senior author (MFE).
RESULTS: Two patients presented with adenoid cystic carcinoma of the orbit. Both patients underwent nonlid sparing orbital exenteration with adjuvant chemotherapy and radiation. Each patient subsequently developed delayed wound healing after prior local flap reconstruction. Vascularized radial forearm flaps in a Pac-Man shape were used in both patients. Both patients were discharged from the hospital on postoperative day 3. No partial or total flap loss was encountered. To date, each patient has undergone planning for osseointegrated implants.
CONCLUSION: The authors present a simplified method of orbital reconstruction that provides high-quality vascularized tissue for resurfacing in 1 stage. This type of open-cavity flap reconstruction avoids the need for flap debulking procedures and facilitates 2-stage placement of osseointegrated implants.

PMID: 28930925 [PubMed - as supplied by publisher]

Salivary Gland Neoplasms: Does Morphological Diversity Reflect Tumor Heterogeneity?

Fetched: September 22nd, 2017, 5:00am GMT

Salivary Gland Neoplasms: Does Morphological Diversity Reflect Tumor Heterogeneity?

Pathobiology. 2017 Sep 21;:

Authors: Rito M, Fonseca I

Abstract
Salivary gland tumor classification encompasses a vast list of benign and malignant neoplasms. Their morphological diversity is recognized not only between different entities but also within individual tumors. Tumor categories as described by the World Health Organization reflect, in part, a true genetic heterogeneity (e.g., translocations involving CRTC1 and CRTC3-MAML2 genes in mucoepidermoid carcinoma and MYB-NFIB fusion in adenoid cystic carcinoma). Carcinoma ex pleomorphic adenoma shows diversity in its histological appearance, but recurrent rearrangements on PLAG1 and HMGA2 are common to its benign precursor. More recently, new categories have been defined, like secretory carcinoma with the t(12;15) (p13;q25) ETV6-NTRK3 translocation and clear-cell carcinoma with EWSR1-ATF1 fusion. Recent studies on cribriform adenocarcinoma of minor salivary gland origin and epithelial-myoepithelial carcinoma point to a correlation with their morphological features. All of these advances show that the search of a histogenetic and genetic basis for salivary gland tumors is helping to clarify morphological categories and unraveling new ones. Nevertheless, currently morphology is still the hallmark of tumor classification and the gold standard. The therapeutic options for advanced tumors remain very limited but the discovery of translocation-generated gene fusions and increased knowledge of the genomic information of salivary gland tumors is creating opportunities for the development of specific targeted therapies.

PMID: 28930752 [PubMed - as supplied by publisher]

Basal Cell Carcinoma With a Cylindromatous Phenotype.

Fetched: September 21st, 2017, 5:00am GMT

Basal Cell Carcinoma With a Cylindromatous Phenotype.

Actas Dermosifiliogr. 2016 Mar;107(2):165-7

Authors: Torres-Gómez FJ, Fernández-Machín P, Neila-Iglesias J

PMID: 26522236 [PubMed - indexed for MEDLINE]

Permalink for 'Diffuse Staining for Activated NOTCH1 Correlates With NOTCH1 Mutation Status and is Associated With Worse Outcome in Adenoid Cystic Carcinoma.'

Diffuse Staining for Activated NOTCH1 Correlates With NOTCH1 Mutation Status and is Associated With Worse Outcome in Adenoid Cystic Carcinoma.

Fetched: September 17th, 2017, 5:00am GMT

Diffuse Staining for Activated NOTCH1 Correlates With NOTCH1 Mutation Status and is Associated With Worse Outcome in Adenoid Cystic Carcinoma.

Am J Surg Pathol. 2017 Sep 13;:

Authors: Sajed DP, Faquin WC, Carey C, Severson EA, H Afrogheh A, A Johnson C, Blacklow SC, Chau NG, Lin DT, Krane JF, Jo VY, Garcia JJ, Sholl LM, Aster JC

Abstract
NOTCH1 is frequently mutated in adenoid cystic carcinoma (ACC). To test the idea that immunohistochemical (IHC) staining can identify ACCs with NOTCH1 mutations, we performed IHC for activated NOTCH1 (NICD1) in 197 cases diagnosed as ACC from 173 patients. NICD1 staining was positive in 194 cases (98%) in 2 major patterns: subset positivity, which correlated with tubular/cribriform histology; and diffuse positivity, which correlated with a solid histology. To determine the relationship between NICD1 staining and NOTCH1 mutational status, targeted exome sequencing data were obtained on 14 diffusely NICD1-positive ACC specimens from 11 patients and 15 subset NICD1-positive ACC specimens from 15 patients. This revealed NOTCH1 gain-of-function mutations in 11 of 14 diffusely NICD1-positive ACC specimens, whereas all subset-positive tumors had wild-type NOTCH1 alleles. Notably, tumors with diffuse NICD1 positivity were associated with significantly worse outcomes (P=0.003). To determine whether NOTCH1 activation is unique among tumors included in the differential diagnosis with ACC, we performed NICD1 IHC on a cohort of diverse salivary gland and head and neck tumors. High fractions of each of these tumor types were positive for NICD1 in a subset of cells, particularly in basaloid squamous cell carcinomas; however, sequencing of basaloid squamous cell carcinomas failed to identify NOTCH1 mutations. These findings indicate that diffuse NICD1 positivity in ACC correlates with solid growth pattern, the presence of NOTCH1 gain-of-function mutations, and unfavorable outcome, and suggest that staining for NICD1 can be helpful in distinguishing ACC with solid growth patterns from other salivary gland and head and neck tumors.

PMID: 28914715 [PubMed - as supplied by publisher]

Permalink for 'A multiplex preclinical model for adenoid cystic carcinoma of the salivary gland identifies regorafenib as a potential therapeutic drug.'

A multiplex preclinical model for adenoid cystic carcinoma of the salivary gland identifies regorafenib as a potential therapeutic drug.

Fetched: September 15th, 2017, 5:00am GMT

A multiplex preclinical model for adenoid cystic carcinoma of the salivary gland identifies regorafenib as a potential therapeutic drug.

Sci Rep. 2017 Sep 12;7(1):11410

Authors: Chen C, Choudhury S, Wangsa D, Lescott CJ, Wilkins DJ, Sripadhan P, Liu X, Wangsa D, Ried T, Moskaluk C, Wick MJ, Glasgow E, Schlegel R, Agarwal S

Abstract
Adenoid cystic carcinomas (ACC) are rare salivary gland cancers with a high incidence of metastases. In order to study this tumor type, a reliable model system exhibiting the molecular features of this tumor is critical, but none exists, thereby inhibiting in-vitro studies and the analysis of metastatic behavior. To address this deficiency, we have coupled an efficient method to establish tumor cell cultures, conditional reprogramming (CR), with a rapid, reproducible and robust in-vivo zebrafish model. We have established cell cultures from two individual ACC PDX tumors that maintain the characteristic MYB translocation. Additional mutations found in one ACC culture also seen in the PDX tumor. Finally, the CR/zebrafish model mirrors the PDX mouse model and identifies regorafenib as a potential therapeutic drug to treat this cancer type that mimic the drug sensitivity profile in PDX model, further confirming the unique advantages of multiplex system.

PMID: 28900283 [PubMed - in process]

Adenoid cystic carcinoma of the head and neck: a retrospective multicenter study.

Fetched: September 15th, 2017, 5:00am GMT

Adenoid cystic carcinoma of the head and neck: a retrospective multicenter study.

Acta Otolaryngol. 2017 Sep 12;:1-7

Authors: Takebayashi S, Shinohara S, Tamaki H, Tateya I, Kitamura M, Mizuta M, Tanaka S, Kojima T, Asato R, Maetani T, Ushiro K, Kitani Y, Ichimaru K, Honda K, Yamada K, Omori K

Abstract
BACKGROUND: Adenoid cystic carcinoma of the head and neck (ACCHN) is rare and difficult to study effective treatment at one institute. Our aim is to identify prognostic factors for this disease by conducting a multicenter study at 11 institutions in Japan.
METHODS: A retrospective multicenter study of ACCHN was performed. One hundred and three patients were identified between 2006 and 2015. The overall survival (OS) rate for all patients was calculated, and OS, locoregional control (LRC) rate, or no distant metastasis (NDM) rate was calculated for patients in that the surgery was performed without distant metastasis (DM). Statistical analyses were performed.
RESULTS: A significant difference with multivariate analysis was observed in patients in sublingual glands, stage IV and the use of radiation therapy ≥60Gy (sufficient RT) in OS for all patients. A significant difference was observed in the use of sufficient postoperative RT in the OS and the LRC rate, and in pathological surgical margins in the NDM rate.
CONCLUSION: Sublingual glands or stage IV was a poorer, and sufficient RT was a better prognostic factor for ACCHN. Sufficient RT was effective to prevent local recurrence after surgical resection. Positive surgical margins caused an increase in DM.

PMID: 28899226 [PubMed - as supplied by publisher]

Clinical and molecular insights into adenoid cystic carcinoma: Neural crest-like stemness as a target.

Fetched: September 14th, 2017, 5:00am GMT

Clinical and molecular insights into adenoid cystic carcinoma: Neural crest-like stemness as a target.

Laryngoscope Investig Otolaryngol. 2016 Aug;1(4):60-77

Authors: Yarbrough WG, Panaccione A, Chang MT, Ivanov SV

Abstract
OBJECTIVES: This review surveys trialed therapies and molecular defects in adenoid cystic carcinoma (ACC), with an emphasis on neural crest-like stemness characteristics of newly discovered cancer stem cells (CSCs) and therapies that may target these CSCs.
DATA SOURCES: Articles available on Pubmed or OVID MEDLINE databases and unpublished data.
REVIEW METHODS: Systematic review of articles pertaining to ACC and neural crest-like stem cells.
RESULTS: Adenoid cystic carcinoma of the salivary gland is a slowly growing but relentless cancer that is prone to nerve invasion and metastases. A lack of understanding of molecular etiology and absence of targetable drivers has limited therapy for patients with ACC to surgery and radiation. Currently, no curative treatments are available for patients with metastatic disease, which highlights the need for effective new therapies. Research in this area has been inhibited by the lack of validated cell lines and a paucity of clinically useful markers. The ACC research environment has recently improved, thanks to the introduction of novel tools, technologies, approaches, and models. Improved understanding of ACC suggests that neural crest-like stemness is a major target in this rare tumor. New cell culture techniques and patient-derived xenografts provide tools for preclinical testing.
CONCLUSION: Preclinical research has not identified effective targets in ACC, as confirmed by the large number of failed clinical trials. New molecular data suggest that drivers of neural crest-like stemness may be required for maintenance of ACC; as such, CSCs are a target for therapy of ACC.

PMID: 28894804 [PubMed]

Permalink for 'Genomic imbalances and MYB fusion in synchronous bilateral adenoid cystic carcinoma and invasive lobular carcinoma of the breast.'

Genomic imbalances and MYB fusion in synchronous bilateral adenoid cystic carcinoma and invasive lobular carcinoma of the breast.

Fetched: September 14th, 2017, 5:00am GMT

Genomic imbalances and MYB fusion in synchronous bilateral adenoid cystic carcinoma and invasive lobular carcinoma of the breast.

Mol Clin Oncol. 2017 Sep;7(3):322-326

Authors: Kovács A, Persson F, Persson M, Andersson MK, Stenman G

Abstract
The incidence of synchronous bilateral breast carcinomas (BBCs) has increased with a more frequent use of magnetic resonance imaging screening of the contralateral breast in women with newly diagnosed breast cancer. A total of 30% of all BBCs occur synchronously. In the present study, we describe a unique case of synchronous BBC in a 59-year-old previously healthy woman with no known family history of breast or ovarian cancer. At the time of diagnosis the patient had an invasive lobular carcinoma (ILC) in the right breast and an adenoid cystic carcinoma (ACC) in the left breast. To the best of our knowledge, this is the first published case of bilateral, simultaneously occurring ACC and ILC of the breast. Genome-wide genomic profiling of the tumors revealed that they had distinctly different genomic imbalances. The ACC had a 5.7 Mb interstitial 6q deletion with a breakpoint located in the 3'-part of MYB, resulting in loss of the last coding exon of MYB and its 3'-UTR. RT-PCR analysis confirmed that the tumor expressed an ACC-specific MYB-NFIB fusion transcript. In contrast, the ILC had no rearrangements of 6q or MYB-NFIB gene fusion but showed instead gain of 1q21.1-qter, loss of 16q11.2-qter, and 22q12.2-q12.3 as the sole genomic imbalances. Notably, concurrent gains of 1q and losses of 16q are characteristic features of ILC. Collectively, our findings indicate that the ACC and ILC had originated independently of each other and that the MYB-NFIB fusion is a specific biomarker for breast ACC.

PMID: 28894575 [PubMed]

Case report of apatinib mesylate treatment in rare advanced tracheal adenoid cystic carcinoma.

Fetched: September 13th, 2017, 5:00am GMT

Case report of apatinib mesylate treatment in rare advanced tracheal adenoid cystic carcinoma.

Thorac Cancer. 2017 Sep 11;:

Authors: Wang H

Abstract
A 57-year-old man was admitted to our department 10 years ago, diagnosed with tracheal adenoid cystic carcinoma. After discontinuing chemotherapy and radiotherapy, the disease recurred in December 2016. Apatinib mesylate (500 mg/day) was administered and computed tomography revealed that his symptoms were significantly relieved. Treatment with apatinib mesylate represents a novel method of treatment for tracheal adenoid cystic carcinoma.

PMID: 28892250 [PubMed - as supplied by publisher]

A comprehensive comparative analysis of treatment modalities for sinonasal malignancies.

Fetched: September 13th, 2017, 5:00am GMT

A comprehensive comparative analysis of treatment modalities for sinonasal malignancies.

Cancer. 2017 Aug 15;123(16):3040-3049

Authors: Robin TP, Jones BL, Gordon OM, Phan A, Abbott D, McDermott JD, Goddard JA, Raben D, Lanning RM, Karam SD

Abstract
BACKGROUND: Sinonasal malignancies are a rare and heterogeneous group of tumors for which there is a paucity of robust data with which to guide management decisions. The authors used the National Cancer Data Base to better understand the presenting characteristics of these tumors and to compare outcomes by treatment modality.
METHODS: The National Cancer Data Base was queried for sinonasal malignancies diagnosed between 2004 and 2012. Overall survival was assessed using multivariate analyses and propensity score matching.
RESULTS: A total of 11,160 patients were identified for the initial analysis. The majority were male, aged 40 to 69 years, with tumors of the nasal cavity or maxillary sinus. Squamous cell histology was most common. The majority of patients presented with advanced tumor stage but without locoregional lymph node or distant metastases. Treatment modalities were compared for squamous cell carcinomas. In multivariate analysis, compared with surgery alone, patients who received adjuvant radiotherapy (hazard ratio [HR], 0.658 [P<.001]), adjuvant chemoradiotherapy (HR, 0.696 [P = .002]), or neoadjuvant therapy (HR, 0.656 [P = .007]) had improved overall survival. Patients who received radiotherapy alone (HR, 1.294 [P = .001]) or chemotherapy alone (HR, 1.834 [P<.001]) had worse outcomes. These findings were validated in propensity score matching. It is important to note that neoadjuvant chemoradiotherapy was associated with achieving a negative surgical margin (odds ratio, 2.641 [P = .045]).
CONCLUSIONS: Surgery is the mainstay of therapy for patients with sinonasal malignancies, but multimodality therapy is associated with improved overall survival. Cancer 2017;123:3040-49. © 2017 American Cancer Society.

PMID: 28369832 [PubMed - indexed for MEDLINE]

Adenoid Cystic Carcinoma of the Cavernous Sinus - Otolaryngological Sequelae of Therapy: Case Report.

Fetched: September 12th, 2017, 5:00am GMT

Adenoid Cystic Carcinoma of the Cavernous Sinus - Otolaryngological Sequelae of Therapy: Case Report.

Adv Exp Med Biol. 2017 Sep 10;:

Authors: Dzięciołowska-Baran E, Gawlikowska-Sroka A

Abstract
A 60-year-old woman, otherwise in a good health condition, was first admitted to the hospital with a year-long tingling sensations of the right half of the face, which gradually turned into severe neuralgia corresponding to maxillary (V2) and mandibular (V3) branches of the right trigeminal nerve. MRI scans of the head revealed an unusual hyperplastic or inflammatory changes along the brain base, cavernous sinus extending toward the pterygopalatine fossa, and lateral pterygoid on the right side. Meningioma was suspected and neuralgia was treated conservatively. About 2 years later, due to severe facial and eye pain, the patient underwent decompression of trigeminal nerve roots - Janetta's surgery. The following MRI scans revealed a tumor of cavernous sinus, arousing suspicion of malignancy. Histological specimens obtained after a biopsy and then partial transnasal tumor resection yielded a diagnosis of adenoid cystic carcinoma. The patient was treated with proton radiation therapy. The therapy caused burns in the oropharyngeal soft tissues extending from the oral cavity to the throat and esophagus. The additional adverse effect of the therapy was hypoacusis and a damage to the right cornea. A radiation-induced sinusitis appeared that required surgical intervention. The patient suffered a string of further complications, including pneumonia and a transient kidney failure. In the end, the patient survived. The adenoid carcinoma in the currently 64-year-old woman is visibly reduced, but she still suffers from the trigeminal neuralgia. The patient remains under constant laryngological outpatient care as she requires a periodic cleansing of nasal cavities and hypoacusis monitoring.

PMID: 28889233 [PubMed - as supplied by publisher]

Efficacy of intralesional corticosteroids in the treatment of cylindromas.

Fetched: September 10th, 2017, 5:00am GMT

Efficacy of intralesional corticosteroids in the treatment of cylindromas.

Clin Exp Dermatol. 2016 Jul;41(5):578-80

Authors: Mulder KM, Crijns MB, van Doorn R

PMID: 27324556 [PubMed - indexed for MEDLINE]

Adenoid Cystic Carcinoma of the Oral Cavity: Radiology-Pathology Correlation.

Fetched: September 9th, 2017, 5:00am GMT

Adenoid Cystic Carcinoma of the Oral Cavity: Radiology-Pathology Correlation.

Head Neck Pathol. 2017 Sep 06;:

Authors: Uraizee I, Cipriani NA, Ginat DT

Abstract
Adenoid cystic carcinoma in the oral cavity is an uncommon salivary gland malignancy that has a propensity for perineural spread. A high-grade variant is evidenced by an abundance of pleomorphic cells, loss of the classic biphasic epithelial-myoepithelial growth pattern, and comedonecrosis, as well as elevated Ki-67. CT and MRI can both be useful for demonstrating the extent of invasion in oral cavity-associated adenoid cystic carcinoma, which can attain the inferior alveolar nerve for perineural spread by direct invasion through the mandible. Reflecting the aggressive nature of this high-grade malignancy, (18)FDG-PET can demonstrate hypermetabolism and can be useful for staging. These features are exemplified in this sine qua non radiology-pathology correlation article.

PMID: 28879643 [PubMed - as supplied by publisher]

Permalink for 'Advanced Tracheal Adenoid Cystic Carcinoma with Thyroid Invasion Mimicking Thyroid Cancer Treated with Definitive Radiation: Case Report and Review of the Literature.'

Advanced Tracheal Adenoid Cystic Carcinoma with Thyroid Invasion Mimicking Thyroid Cancer Treated with Definitive Radiation: Case Report and Review of the Literature.

Fetched: September 9th, 2017, 5:00am GMT

Advanced Tracheal Adenoid Cystic Carcinoma with Thyroid Invasion Mimicking Thyroid Cancer Treated with Definitive Radiation: Case Report and Review of the Literature.

Case Rep Oncol. 2017 May-Aug;10(2):706-712

Authors: Al Khatib S, Asha W, Khzouz O, Barakat F, Khader J

Abstract
A 54-year-old female patient, a breast cancer survivor and a case of unresectable adenoid cystic carcinoma of the trachea, with thyroid invasion, presented with suprasternal neck swelling mimicking thyroid primary. A literature search was undertaken to highlight this rare presentation. There have been few reports in the literature describing tracheal adenoid cystic carcinoma involving the thyroid.

PMID: 28878654 [PubMed]

Permalink for 'HPV-related Multiphenotypic Sinonasal Carcinoma: An Expanded Series of 49 Cases of the Tumor Formerly Known as HPV-related Carcinoma With Adenoid Cystic Carcinoma-like Features.'

HPV-related Multiphenotypic Sinonasal Carcinoma: An Expanded Series of 49 Cases of the Tumor Formerly Known as HPV-related Carcinoma With Adenoid Cystic Carcinoma-like Features.

Fetched: September 8th, 2017, 5:00am GMT

HPV-related Multiphenotypic Sinonasal Carcinoma: An Expanded Series of 49 Cases of the Tumor Formerly Known as HPV-related Carcinoma With Adenoid Cystic Carcinoma-like Features.

Am J Surg Pathol. 2017 Sep 04;:

Authors: Bishop JA, Andreasen S, Hang JF, Bullock MJ, Chen TY, Franchi A, Garcia JJ, Gnepp DR, Gomez-Fernandez CR, Ihrler S, Kuo YJ, Lewis JS, Magliocca KR, Pambuccian S, Sandison A, Uro-Coste E, Stelow E, Kiss K, Westra WH

Abstract
Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma (HMSC), originally known as HPV-related carcinoma with adenoid cystic carcinoma-like features, is a peculiar neoplasm that is restricted to the sinonasal tract, exhibits features of both a surface-derived and salivary gland carcinoma (particularly adenoid cystic carcinoma), and is associated with high-risk HPV. Given the limited number of published cases, the full clinicopathologic spectrum of this neoplasm is unclear. Here, we present an updated experience of 49 cases. All cases of HMSC were obtained from the authors' files. Immunohistochemistry for p16, c-kit, and myoepithelial cell markers (S100, actin, calponin, p63, and/or p40) was performed along with RNA in situ hybridization for HPV (type 33-specific as well as a high-risk cocktail). Fluorescence in situ hybridization studies for fusions of MYB, NFIB, and MYBL1 was performed on a subset of cases. Clinical follow-up was obtained from medical records. A total of 49 cases of HMSC were collected. Twenty-eight (57%) were from women and 18 (43%) from men, ranging in age from 28 to 90 years (mean, 54 y). Of 40 cases with detailed staging information, 43% of HMSCs presented with a high T-stage (T3 or T4). Histologically, most grew predominantly as solid nests of basaloid cells exhibiting high mitotic rates and frequent necrosis, with histologic and immunohistochemical evidence of myoepithelial differentiation. Most cases also demonstrated foci of cribriform and/or tubular growth, along with an inconspicuous population of ducts. Thirty-four (69%) cases demonstrated an unusual pattern of surface involvement where markedly atypical squamous cells colonized tracts of the sinonasal mucosa. Less consistent histologic features included squamous differentiation within the invasive tumor (n=6), sarcomatoid transformation (n=5) including overt chondroid differentiation (n=3), and prominent epithelial-myoepithelial carcinoma-like growth (n=3). All cases were positive for p16 by immunostaining and HPV by RNA in situ hybridization. Thirty-three (67%) were positive for HPV 33. No cases tested for MYB, MYBL1, or NFIB gene fusions were positive. In the 38 cases with follow-up data, (mean follow-up, 42 mo) 14 recurred locally and 2 metastasized (lung, finger). There were no regional lymph node metastases, and no tumor-related deaths. HMSC is a distinct sinonasal neoplasm characterized by myoepithelial differentiation, frequent surface epithelial involvement, and the presence of high-risk HPV (especially type 33). Although it classically exhibits a cribriforming pattern that closely resembles adenoid cystic carcinoma, our expanded series highlights a histologic spectrum that is much broader than previously recognized, warranting a change in terminology. HMSC usually presents as a large and destructive sinonasal mass with high-grade histologic features, but it paradoxically behaves in a relatively indolent manner, underscoring the importance of distinguishing HMSC from true adenoid cystic carcinoma, squamous cell carcinoma, and other histologic mimickers.

PMID: 28877065 [PubMed - as supplied by publisher]

Optic Disc Pyogenic Granuloma.

Fetched: September 6th, 2017, 5:00am GMT

Optic Disc Pyogenic Granuloma.

Ocul Oncol Pathol. 2017 Jul;3(2):110-112

Authors: Marback EF, Espinheira Fonseca L, Marback RL

Abstract
AIM: The aim of this paper was to report a rare presentation of adenoid cystic carcinoma.
METHOD: This is a case report presenting clinical, radiographic, and histopathologic findings.
RESULTS: A 72-year-old female with no reported ocular symptoms was found to have an incidental right orbital mass on imaging. Additional studies revealed multiple liver lesions, which were biopsied and found to be consistent with adenoid cystic carcinoma. She was then referred to the ophthalmology service, where ocular examination demonstrated 2.5 mm of right proptosis with elevation and abduction deficits. Diplopia could be elicited in extreme upgaze and right lateral gaze. An excisional biopsy of the orbital mass was performed, with histopathology confirming the diagnosis of primary adenoid cystic carcinoma of the lacrimal gland, thereby also supporting the initial suspicion that the hepatic adenoid cystic carcinoma lesions represented metastases.
CONCLUSION: The authors describe a rare presentation of adenoid cystic carcinoma of the lacrimal gland, initially asymptomatic, with metastatic lesions restricted to the liver at the time of diagnosis. Three previous cases of adenoid cystic carcinoma with isolated metastatic hepatic lesions at the time of diagnosis have been reported; all of these cases localized the primary tumor to the salivary glands.

PMID: 28868280 [PubMed]

Adenoid Cystic Carcinoma Presenting as Abducens Nerve Palsy Secondary to Cavernous Sinus Lesion.

Fetched: September 6th, 2017, 5:00am GMT

Adenoid Cystic Carcinoma Presenting as Abducens Nerve Palsy Secondary to Cavernous Sinus Lesion.

Ann Neurosci. 2017 Jul;24(3):187-190

Authors: Donaldson C, Dawes B, Rathi V, Bulluss K

Abstract
BACKGROUND: We report a 61-year-old who presented with a right-sided abducens nerve palsy secondary to a middle cranial fossa adenoid cystic carcinoma (ACC) extending into the cavernous sinus.
PURPOSE: This case represents a unique presentation of intracranial ACC with a large middle cranial fossa mass and only a small extracranial component.
METHODS: Review of the literature was undertaken to identify cases of intracranial ACC and their range of presentations.
RESULTS: Our results show that this is the first reported case of an ACC presenting mostly as an intracranial mass with an isolated cranial nerve lesion.
CONCLUSION: Our case highlights the importance of a broad differential diagnosis, particularly in circumstances where there are atypical features of lesions on radiographic imaging.

PMID: 28867902 [PubMed]

Straightforward Method for Coverage of Major Vessels After Modified Radical Neck Dissection.

Fetched: September 1st, 2017, 5:00am GMT

Straightforward Method for Coverage of Major Vessels After Modified Radical Neck Dissection.

J Oral Maxillofac Surg. 2017 Jun;75(6):1299.e1-1299.e4

Authors: González-García R, Moreno-García C, Moreno-Sánchez M, Román-Romero L

Abstract
A new method for covering the internal jugular vein and carotid artery after exposure of the cervical vascular axis subsequent to neck dissection is presented. To cover the most caudal part of the vascular axis, a platysma coli muscle flap is harvested from its most medial and inferior part of the neck in a caudally based fashion and is slightly rotated posteriorly up to 45°. In addition, a superiorly based sternocleidomastoid muscle flap involving the posterior half of the muscle after detachment of the clavicle head is harvested and rotated 45° anteriorly to cover the upper two thirds of the vascular axis. This technique seems to be a good alternative to the pectoralis major myocutaneous flap for covering cervical major vessels, if no classical radical neck dissection is performed, especially in those oncologic malnourished patients who will undergo adjuvant radiotherapy after surgical treatment.

PMID: 28063277 [PubMed - indexed for MEDLINE]

Adenoid cystic carcinoma with lipometaplasia: a case report with morphoproteomic analysis of lipogenesis.

Fetched: August 24th, 2017, 5:00am GMT

Adenoid cystic carcinoma with lipometaplasia: a case report with morphoproteomic analysis of lipogenesis.

Pathology. 2017 Aug 18;:

Authors: Noh BJ, Kim YW, Park YK, Lee J

PMID: 28826835 [PubMed - as supplied by publisher]

Longitudinal Perioperative Pain Assessment in Head and Neck Cancer Surgery.

Fetched: August 23rd, 2017, 5:00am GMT

Longitudinal Perioperative Pain Assessment in Head and Neck Cancer Surgery.

Ann Otol Rhinol Laryngol. 2017 Sep;126(9):646-653

Authors: Buchakjian MR, Davis AB, Sciegienka SJ, Pagedar NA, Sperry SM

Abstract
OBJECTIVE: To evaluate perioperative pain in patients undergoing major head and neck cancer surgery and identify associations between preoperative and postoperative pain characteristics.
METHODS: Patients undergoing head and neck surgery with regional/free tissue transfer were enrolled. Preoperative pain and validated screens for symptoms (neuropathic pain, anxiety, depression, fibromyalgia) were assessed. Postoperatively, patients completed a pain diary for 4 weeks.
RESULTS: Twenty-seven patients were enrolled. Seventy-eight percent had pain prior to surgery, and for 38%, the pain had neuropathic characteristics. Thirteen patients (48%) completed at least 2 weeks of the postoperative pain diary. Patients with moderate/severe preoperative pain report significantly greater pain scores postoperatively, though daily pain decreased at a similar linear rate for all patients. Patients with more severe preoperative pain consumed greater amounts of opioids postoperatively, and this correlated with daily postoperative pain scores. Patients who screened positive for neuropathic pain also reported worse postoperative pain.
CONCLUSION: Longitudinal perioperative pain assessment in head and neck patients undergoing surgery suggests that patients with worse preoperative pain continue to endorse worse pain postoperatively and require more narcotics. Patients with preoperative neuropathic pain also report poor pain control postoperatively, suggesting an opportunity to identify these patients and intervene with empiric neuropathic pain treatment.

PMID: 28770621 [PubMed - indexed for MEDLINE]

Multicenter experiences in temporal bone cancer surgery based on 89 cases.

Fetched: August 23rd, 2017, 5:00am GMT

Multicenter experiences in temporal bone cancer surgery based on 89 cases.

PLoS One. 2017;12(2):e0169399

Authors: Wierzbicka M, Niemczyk K, Bruzgielewicz A, Durko M, Klatka J, Kopeć T, Osuch-Wójcikiewicz E, Pietruszewska W, Szymański M, Szyfter W

Abstract
OBJECTIVE: To present outcomes of extensive surgery: lateral, subtotal, total petrosectomies in patients with temporal bone invasion resulting from specific primary cancers.
STUDY DESIGN: Retrospective case review.
SETTING: Four tertiary referral centers.
MATERIAL: 89 patients with cancer of the temporal bone treated between January 2006 and December 2010.
INTERVENTION: Multidisciplinary team approach including surgical resection, reconstruction, and postoperative radiotherapy.
MAIN OUTCOME MEASURE: Disease-specific survival, overall survival.
RESULTS: In 27.0% of the patients, relapse was reported, with an average of 6.3 months after surgery; 31 patients (34.8%) died during the follow-up. The average mortality was 22.1 months. Fifty-four patients (58.7%) stayed alive during the time of observation. The average survival time was 42.0 months. The median time of survival with relapse was 12 months (range: 1-51 months). The three-year disease-free rate was 38.0% and the overall survival rate was 58.7%.
CONCLUSIONS: Petrosectomy is an effective treatment for malignant temporal bone invasion. The probability of a good outcome was statistically decreased with a high T grade, positive margins, and salvage surgery. Younger age is connected with better prognosis. One of the major tasks remains to improve detection and to shorten the time to diagnosis, keeping in mind that symptoms are insidious and in younger people, the time before diagnosis was longer.

PMID: 28225795 [PubMed - indexed for MEDLINE]

Whole Exome Sequencing of Lacrimal Gland Adenoid Cystic Carcinoma.

Fetched: August 20th, 2017, 5:00am GMT

Whole Exome Sequencing of Lacrimal Gland Adenoid Cystic Carcinoma.

Invest Ophthalmol Vis Sci. 2017 May 01;58(6):BIO240-BIO246

Authors: Sant DW, Tao W, Field MG, Pelaez D, Jin K, Capobianco A, Dubovy SR, Tse DT, Wang G

Abstract
Purpose: To identify genomic mutations in lacrimal gland adenoid cystic carcinoma (LGACC) samples from patients.
Methods: Genomic DNA was extracted from LGACC specimens. Whole exome sequencing (exome-seq) was conducted to screen for mutations. Capillary sequencing was performed to verify mutations in genes shared by multiple samples. Luciferase assays were used to evaluate functional consequences of NOTCH1 mutations.
Results: The mutation profile of LGACC was complicated. The most frequently mutated gene observed (28.6%) was bromodomain PHD finger transcription factor (BPTF). No mutation was identified in common cancer genes such as TP53, KRAS, and BRAF. However, mutations predicted to be functionally severe were accumulated in the Notch signaling pathway including NOTCH1 and NOTCH2, of which mutations have been reported in head/neck adenoid cystic carcinoma (ACC). Of 14 LGACC samples, five samples carry mutations in Notch pathway genes. Capillary sequencing verified all the mutations in the two NOTCH genes identified by exome-seq. Compared to the wild-type NOTCH1, three frame shifting mutations and two missense mutations (C387W and L1600Q) increased luciferase activity approximately 10- to 25-fold.
Conclusions: Major genomic mutation profiles in LGACC were uncovered by exome-seq. Although preliminary in nature, the Notch pathway could be a potential therapeutic target for LGACC.

PMID: 28820917 [PubMed - in process]

Serum Levels of Cyfra 21 in Patients with Benign and Malignant Salivary Gland Tumors.

Fetched: August 20th, 2017, 5:00am GMT

Serum Levels of Cyfra 21 in Patients with Benign and Malignant Salivary Gland Tumors.

Iran J Otorhinolaryngol. 2017 Jul;29(93):203-208

Authors: Jeiroodi N, Aslani SM, Khademi B, Malekzadeh M, Jaafari-Ashkavandi Z

Abstract
INTRODUCTION: Cyfra 21 is a serum-soluble fragment of cytokeratin19. Increased Cyfra 21 serum levels and their benefit as a tumor marker have been shown in some malignancies. This study aimed to evaluate the serum levels of Cyfra 21 in patients with benign and malignant salivary gland tumors.
MATERIALS AND METHODS: In this cross-sectional study, the serum level of Cyfra 21 in 44 patients with malignant salivary gland tumors and 16 cases of pleomorphic adenoma were compared with 28 healthy controls using enzyme-linked immunosorbent assay (ELISA). Data were analyzed statistically using the Kruskal Wallis test, analysis of variance (ANOVA) and Spearman's correlation tests.
RESULTS: Mean serum levels of Cyfra 21 were 0.135 ± 0.285 ng/ ml in the control group, 0.167 ± 0.142 ng/ ml in patients with pleomorphic adenoma and 1.059 ± 3.251 ng/ml in patients with malignant salivary gland tumors. There was no significant difference among groups. Cyfra 21 levels did not correlate with location of tumor, clinical stage or cigarette smoking.
CONCLUSION: Results of the present study showed no significant difference in Cyfra 21 serum level in salivary gland tumors compared with normal individuals. In addition, Cyfra 21 serum level was not sufficiently sensitive to function as a tumor marker in salivary gland tumors.

PMID: 28819618 [PubMed]

Ceruminous Adenoid Cystic Carcinoma of External Auditory Canal.

Fetched: August 19th, 2017, 5:00am GMT

Ceruminous Adenoid Cystic Carcinoma of External Auditory Canal.

J Int Adv Otol. 2017 Aug;13(2):292-294

Authors: Ebelhar AE, West DS, Aouad RK

Abstract
An adenoid cystic carcinoma (AdCC) of the ceruminous glands is very rare; its diagnosis is most often challenging, and simple biopsies may be misleading. Our paper describes a case of a circumferential mass of the left ear canal that was initially reported as a basal cell carcinoma on biopsies in the clinic and on frozen sections intraoperatively. The final pathology was an AdCC of the ceruminous glands of the external auditory canal. Our case reflects the difficulty in the diagnosis of an AdCC of the ceruminous gland and the importance of keeping broad differential diagnoses in mind when counseling patients with masses in the ear canals until final pathology is obtained.

PMID: 28816699 [PubMed - in process]

Bartholin gland cancer.

Fetched: August 18th, 2017, 5:00am GMT

Bartholin gland cancer.

Crit Rev Oncol Hematol. 2017 Sep;117:1-11

Authors: Di Donato V, Casorelli A, Bardhi E, Vena F, Claudia M, Muzii L, Benedetti Panici P

Abstract
INTRODUCTION: Bartholin gland carcinoma is an extremely rare condition. Because of its, phase III trials have not been carried out, there exists no unanimous consensus on treatment and guidelines are missing.
METHODS: All studies reporting cases of Bartholin cancer were collected and screened for the evaluations. Baseline characteristics of studies were extracted and were queried in a database.
RESULTS: A total number of 133 manuscripts collected were available for the review process, representing a total number of 275 reported cases. The histological type of Bartholin gland cancer was specified in 90.4% cases: 30.7% cases were squamous cell carcinoma, 29.6% adenoid cystic carcinoma, 25% adenocarcinomas. At multivariate analysis adenocarcinoma histotype and positive lymph node were statistical correlated with worse prognosis.
CONCLUSION: Bartholin gland cancer remains a challenge for gynecologic oncologists. To better understand and treat this disease, centralization to referral centers and design of multi institutional trials is crucial.

PMID: 28807231 [PubMed - in process]

Doxorubicin-loaded environmentally friendly carbon dots as a novel drug delivery system for nucleus targeted cancer therapy.

Fetched: August 16th, 2017, 5:00am GMT

Doxorubicin-loaded environmentally friendly carbon dots as a novel drug delivery system for nucleus targeted cancer therapy.

Colloids Surf B Biointerfaces. 2017 Jul 10;159:349-359

Authors: Yuan Y, Guo B, Hao L, Liu N, Lin Y, Guo W, Li X, Gu B

Abstract
Chemotherapy is widely applied against various kinds of carcinoma. Generally, chemotherapeutic agents, such as Doxorubicin (DOX), Paclitaxel (PTX), 5-Fluorouracil (5-FU), Methotrexate (MTX), and Vinblastine (VLB) are combined with a view to maximizing their efficacy. Unfortunately, chemotherapeutics are indiscriminate and also kill normal healthy cells, resulting in serious side effects. This non-productive and destructive distribution of chemotherapeutics is regarded as one of the largest problems associated with chemotherapy. Recently, the application of carbon dots (CDs) in cancer therapy has attracted considerable attention due to their attractive properties, such as biocompatibility and low toxicity. We report herein on the fabrication of CD-DOX antitumor drug complexes, from the combination of CDs and DOX, with a view to providing a novel and efficient strategy for cancer treatment. CDs were synthesized by hydrothermal treatment of milk, a simple and environmentally friendly synthetic process. DOX was conjugated to the CDs through electrostatic interactions via the multiple surface CD functional groups. The CD-DOX complexes exhibited pH-dependent DOX release behavior. A cytotoxicity study demonstrated that the CDs were non-cytotoxic in the range of concentrations used. Compared to free DOX, the CD-DOX complexes were significantly more destructive to the adenoid cystic carcinoma cell line (ACC-2), but exhibited lower toxicity to a mouse fibroblast cell line (L929). Confocal microscopy and flow cytometry confirmed that CD-DOX complexes increased cancer therapy efficiency through the localization of a much higher quantity of drugs in the nuclei of tumor cells and induced a higher rate of apoptosis in ACC-2 cells, compared to DOX alone.

PMID: 28806666 [PubMed - as supplied by publisher]

Cutaneous Sweat Gland Carcinomas with Basaloid Differentiation: An Update with Emphasis on Differential Diagnoses.

Fetched: August 16th, 2017, 5:00am GMT

Cutaneous Sweat Gland Carcinomas with Basaloid Differentiation: An Update with Emphasis on Differential Diagnoses.

Clin Lab Med. 2017 Sep;37(3):587-601

Authors: Flux K, Brenn T

Abstract
This article focuses on primary cutaneous sweat gland carcinomas with basaloid differentiation, including cribriform apocrine carcinoma, endocrine mucin-producing sweat gland carcinoma, mucinous carcinoma, adenoid cystic carcinoma, spiradenocarcinoma, and digital papillary adenocarcinoma. These tumors are rare and pose a significant diagnostic challenge. Their clinical presentation is nonspecific and there is significant overlap of their histologic features. Confident diagnosis is necessary because their clinical behavior ranges from indolent, nonrecurring, nonmetastasizing tumors to those with potential for disseminated disease and mortality. They should be separated from cutaneous metastases of primary visceral adenocarcinomas. Salient features of these rare entities are discussed with emphasis on distinguishing features and diagnostic clues.

PMID: 28802502 [PubMed - in process]

Breast Adenoid Cystic Carcinoma: A Rare Case Report and Review of the Literature.

Fetched: August 13th, 2017, 5:00am GMT

Breast Adenoid Cystic Carcinoma: A Rare Case Report and Review of the Literature.

Gulf J Oncolog. 2017 May;1(24):66-69

Authors: El Amarti L, Raiss H, Layachi M, Baldé S, Ettahri H, Elghissassi I, Mrabti H, Errihani H

Abstract
BACKGROUND: Primary adenoid cystic carcinoma (ACC) of the breast is a rare subtype of invasive breast cancer. It has a particular interest because of its excellent prognosis conversely to other triple-negative breast cancers that are associated with poor prognosis. The place of chemotherapy and radiotherapy remains controversial and there is no consensus on optimal management of the ACC of the breast.
CASE REPORT: A 50-year-old woman, presented with a palpable right breast lump. Core biopsy of the lump revealed an adenoid cystic carcinoma. A lumpectomy with axillary lymph node dissection was performed and completed by a mastectomy because of the positive surgical margins. The histopathological examination revealed an adenoid cystic carcinoma, with negative expression of hormone receptors and human epidermal growth factor receptor 2 HER2 and with no lymph node involvement. The patient underwent adjuvant sequential chemotherapy with anthracycline and taxane followed by radiotherapy. We discuss diagnosis, prognostic, and treatment options for ACC of the breast in light of existing literature.
CONCLUSION: Adenoid cystic carcinoma of the breast is a rare variant of triple negative breast cancer with excellent prognosis. Surgical treatment is the mainstay with no clear consensus for radiotherapy and chemotherapy.

PMID: 28798006 [PubMed - in process]

MYPHISMO: MYB and PD-1 Immunotherapies Against Multiple Oncologies Trial

Posted: September 12th, 2017, 2:00pm GMT

Conditions: Colorectal Cancer; Adenoid Cystic Carcinoma
Interventions: Drug: TetMYB Vaccine; Drug: BGB-A317
Sponsor: Peter MacCallum Cancer Centre, Australia
Not yet recruiting - verified September 2017

Permalink for 'A Clinical Trial of Endoscopic Surgery Followed by Chemotherapy and Proton Radiation for the Treatment of Tumors in the Sinus and Nasal Passages'

A Clinical Trial of Endoscopic Surgery Followed by Chemotherapy and Proton Radiation for the Treatment of Tumors in the Sinus and Nasal Passages

Posted: September 5th, 2017, 2:00pm GMT

Conditions: Paranasal Sinus Cancer; Nasal Cavity Tumor; Nasal Cavity Adenocarcinoma
Interventions: Drug: Cisplatin; Radiation: Adjuvant Proton Radiotherapy; Procedure: Endoscopic Resection
Sponsor: Memorial Sloan Kettering Cancer Center
Recruiting - verified September 2017

Study of Nivolumab Plus Ipilimumab in Patients With Salivary Gland Cancer

Posted: May 30th, 2017, 2:00pm GMT

Condition: Salivary Gland Cancer
Interventions: Drug: Nivolumab; Drug: Ipilimumab
Sponsors: Memorial Sloan Kettering Cancer Center; Bristol-Myers Squibb
Recruiting - verified August 2017

Pembrolizumab With or Without Radiation in Patients With Recurrent or Metastatic Adenoid Cystic Carcinoma

Posted: March 13th, 2017, 2:00pm GMT

Condition: Adenoid Cystic Carcinoma
Interventions: Radiation: Radiation; Drug: Pembrolizumab
Sponsors: Dana-Farber Cancer Institute; Merck Sharp & Dohme Corp.
Recruiting - verified August 2017

Chidamide for Advanced Cephalic and Cervical Adenocystic Carcinoma: Evaluation of Efficiency and Safety

Posted: August 25th, 2016, 2:00pm GMT

Condition: Adenocystic Carcinoma
Intervention: Drug: Chidamide
Sponsor: Dong mei
Recruiting - verified August 2017

Study of Axitinib in Patients With Recurred or Metastatic Adenoid Cystic Carcinoma

Posted: July 28th, 2016, 2:00pm GMT

Condition: Recurrent ACC, metastaticACC, Unreaectable ACC
Interventions: Drug: Axitinib; Other: Observation
Sponsor: Seoul National University Hospital
Recruiting - verified September 2017

Randomized Carbon Ions vs Standard Radiotherapy for Radioresistant Tumors

Posted: July 18th, 2016, 2:00pm GMT

Condition: Malignant Tumors as Chordoma, Adenoid Cystic Carcinoma and Sarcoma
Interventions: Radiation: Carbon ions therapy; Radiation: Advanced external radiotherapy by Xrays or protons
Sponsor: Hospices Civils de Lyon
Not yet recruiting - verified August 2017

A Study of Apatinib in Recurrent/Metastatic Adenoid Cystic Carcinoma of the Head and Neck

Posted: May 12th, 2016, 2:00pm GMT

Condition: Adenoid Cystic Carcinoma
Intervention: Drug: Apatinib Mesylate
Sponsor: Shanghai Ninth People's Hospital Affiliated to Shanghai Jiao Tong University
Recruiting - verified August 2017

Childhood Cancer Awareness Month - WHEC News - 10NBC

Posted: September 13th, 2017, 9:45am GMT

Childhood Cancer Awareness Month
WHEC News - 10NBC
Hailey survived Adrenocortical Carcinoma, a rare form of cancer that affects the adrenal glands. Every Monday morning, Hailey goes into Golisano Children's Hospital for a weekly checkup. "Every Monday I come in to get my blood checked because I have ...

Genetic disruption of 21-hydroxylase in zebrafish causes interrenal hyperplasia.

Fetched: September 24th, 2017, 5:00am GMT

Genetic disruption of 21-hydroxylase in zebrafish causes interrenal hyperplasia.

Endocrinology. 2017 Sep 13;:

Authors: Eachus H, Zaucker A, Oakes JA, Griffin A, Weger M, Güran T, Taylor A, Harris A, Greenfield A, Quanson JL, Storbeck KH, Cunliffe VT, Müller F, Krone N

Abstract
Congenital adrenal hyperplasia is a group of common inherited disorders leading to glucocorticoid deficiency. Most cases are caused by 21-hydroxylase deficiency (21OHD). The systemic consequences of imbalanced steroid hormone biosynthesis due to severe 21OHD remains poorly understood. Therefore, we have developed a zebrafish model for 21OHD, which focuses on the impairment of glucocorticoid biosynthesis. A single 21-hydroxylase gene (cyp21a2) is annotated in the zebrafish genome based on sequence homology. Our in silico analysis of the Cyp21a2 protein sequence suggests a sufficient degree of similarity for the usage of zebrafish cyp21a2 to model aspects of human 21OHD in vivo. We determined the spatio-temporal expression patterns of cyp21a2 by whole mount in situ hybridisation and RT-PCR throughout early development. Early cyp21a2 expression is restricted to the interrenal gland (zebrafish adrenal counterpart) and the brain. To further explore the in vivo consequences of 21-hydroxylase deficiency we created several cyp21a2 null-allele zebrafish lines employing a transcription activator-like effector nuclease genomic engineering strategy. Homozygous mutant zebrafish larvae showed an upregulation of the hypothalamic-pituitary-interrrenal axis and interrenal hyperplasia. Furthermore, Cyp21A2-deficient larvae had a typical steroid profile with reduced concentrations of cortisol and increased concentrations of 17-hydroxyprogesterone and 21-deoxycortisol. Affected larvae showed an upregulation of the hypothalamic-pituitary-interrrenal axis and interrenal hyperplasia. Downregulation of the glucocorticoid-responsive genes pck1 and fkbp5 indicated systemic glucocorticoid deficiency. Our work demonstrates the crucial role of Cyp21a2 in glucocorticoid biosynthesis in zebrafish larvae and establishes a novel in vivo model allowing for studies of systemic consequences of altered steroid hormone synthesis.

PMID: 28938470 [PubMed - as supplied by publisher]

Permalink for 'Critical Illness-Related Corticosteroid Insufficiency (CIRCI): A Narrative Review from a Multispecialty Task Force of the Society of Critical Care Medicine (SCCM) and the European Society of Intensive Care Medicine (ESICM).'

Critical Illness-Related Corticosteroid Insufficiency (CIRCI): A Narrative Review from a Multispecialty Task Force of the Society of Critical Care Medicine (SCCM) and the European Society of Intensive Care Medicine (ESICM).

Fetched: September 24th, 2017, 5:00am GMT

Critical Illness-Related Corticosteroid Insufficiency (CIRCI): A Narrative Review from a Multispecialty Task Force of the Society of Critical Care Medicine (SCCM) and the European Society of Intensive Care Medicine (ESICM).

Crit Care Med. 2017 Sep 21;:

Authors: Annane D, Pastores SM, Arlt W, Balk RA, Beishuizen A, Briegel J, Carcillo J, Christ-Crain M, Cooper MS, Marik PE, Meduri GU, Olsen KM, Rochwerg B, Rodgers SC, Russell JA, Van den Berghe G

Abstract
OBJECTIVE: To provide a narrative review of the latest concepts and understanding of the pathophysiology of critical illness-related corticosteroid insufficiency (CIRCI).
PARTICIPANTS: A multi-specialty task force of international experts in critical care medicine and endocrinology and members of the Society of Critical Care Medicine and the European Society of Intensive Care Medicine.
DATA SOURCES: Medline, Database of Abstracts of Reviews of Effects (DARE), Cochrane Central Register of Controlled Trials (CENTRAL) and the Cochrane Database of Systematic Reviews.
RESULTS: Three major pathophysiologic events were considered to constitute CIRCI: dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis, altered cortisol metabolism, and tissue resistance to glucocorticoids. The dysregulation of the HPA axis is complex, involving multidirectional crosstalk between the CRH/ACTH pathways, autonomic nervous system, vasopressinergic system, and immune system. Recent studies have demonstrated that plasma clearance of cortisol is markedly reduced during critical illness, explained by suppressed expression and activity of the primary cortisol-metabolizing enzymes in the liver and kidney. Despite the elevated cortisol levels during critical illness, tissue resistance to glucocorticoids is believed to occur due to insufficient glucocorticoid alpha-mediated anti-inflammatory activity.
CONCLUSIONS: Novel insights into the pathophysiology of CIRCI add to the limitations of the current diagnostic tools to identify at-risk patients and may also impact how corticosteroids are used in patients with CIRCI.

PMID: 28938251 [PubMed - as supplied by publisher]

[One Case about Primary Pulmonary Squamous Cell Carcinoma  with Retinal Metastasis].

Fetched: September 24th, 2017, 5:00am GMT

[One Case about Primary Pulmonary Squamous Cell Carcinoma  with Retinal Metastasis].

Zhongguo Fei Ai Za Zhi. 2017 Sep 20;20(9):652-656

Authors: Liu J, Gao X, Sun M

Abstract
Lung cancer is a common malignant tumor in clinic. Morbidity and mortality are the first place in cancer, which seriously threaten the health of human beings. Due to the lack of effective and specific screening methods, most patients were found already at an advanced stage, and often combined with bone, brain, liver, adrenal and other parts of the metastasis. Symptoms of patient resulted from the primary tumor, metastatic spread, or paraneoplastic syndrome commonly cause the medical attention. For the syndrome related to the distant metastatic spread as the first manifestation, diagnosis would be a challenge. Retinal metastasis is very rare as the first symptom of squamous cell carcinoma of the lung. This paper reviewed a case, in which a patient developed the retinal metastasis as the inital presentation. After systemic evaluation, our patient was managed in multidisciplinary treatments, including surgery and chemotherapy. Now, the patient has a good short-term prognosis and is still in fellow-up condition. Later, we summarized the characteristics of the diagnosis and treatment of this case and provided experience for clinicians. At the same time, reviewing the relevant literature on tumor metastasis provides a window for our understanding of the rare manifestations of lung cancer.

PMID: 28935021 [PubMed - in process]

Permalink for 'Corticosteroids and infliximab impair the performance of interferon-γ release assays used for diagnosis of latent tuberculosis.'

Corticosteroids and infliximab impair the performance of interferon-γ release assays used for diagnosis of latent tuberculosis.

Fetched: September 24th, 2017, 5:00am GMT

Corticosteroids and infliximab impair the performance of interferon-γ release assays used for diagnosis of latent tuberculosis.

Thorax. 2017 Oct;72(10):946-949

Authors: Edwards A, Gao Y, Allan RN, Ball D, de Graaf H, Coelho T, Clifford V, Curtis N, Williams A, Faust SN, Mansour S, Marshall B, Elkington P, Tebruegge M

Abstract
The impact of immunosuppression on interferon-γ release assays and novel cytokine biomarkers of TB infection, mycobacteria-specific IL-2, IP-10 and TNF-α responses was investigated in an ex vivo model. Cytokine responses in standard QuantiFERON-TB Gold in-Tube (QFT-GIT) assays were compared with duplicate assays containing dexamethasone or infliximab. Dexamethasone converted QFT-GIT results from positive to negative in 30% of participants. Antigen-stimulated interferon-γ, IL-2 and TNF-α responses were markedly reduced, but IP-10 responses were preserved. Infliximab caused QFT-GIT result conversion in up to 30% of participants and substantial reductions in all cytokine responses. Therefore, corticosteroids and anti-TNF-α agents significantly impair interferon-γ release assay performance. IP-10 may be a more robust TB biomarker than interferon-γ in patients receiving corticosteroids.

PMID: 28159773 [PubMed - indexed for MEDLINE]

Clinical characteristics of PRKACA mutations in Chinese patients with adrenal lesions: a single-centre study.

Fetched: September 24th, 2017, 5:00am GMT

Clinical characteristics of PRKACA mutations in Chinese patients with adrenal lesions: a single-centre study.

Clin Endocrinol (Oxf). 2016 Dec;85(6):954-961

Authors: Li X, Wang B, Tang L, Lang B, Zhang Y, Zhang F, Chen L, Ouyang J, Zhang X

Abstract
CONTEXT: Recent studies have identified that the somatic PRKACA L206R mutation can cause cortisol-producing adenomas (CPAs). This study investigated the prevalence and characteristics of PRKACA, GNAS and CTNNB1 mutations in adrenal lesions in patients from a single centre in China.
DESIGN, PATIENTS AND MEASUREMENTS: We sequenced PRKACA, GNAS and CTNNB1 genes in 108 patients, including 60 patients with CPAs (57 with unilateral and three with bilateral adenomas), 13 with nonfunctional adenomas, 12 with adrenocortical carcinomas (ACCs), 15 with primary bilateral macronodular hyperplasia (PBMAH) and eight with aldosterone and cortisol cosecreting adenomas. Mutations in PRKACA, GNAS and CTNNB1 were examined, and clinical characteristics were compared.
RESULTS: Among the unilateral CPAs, we identified somatic mutations in PRKACA (L206R) in 23 cases (40·4%), GNAS (R201C and R201H) in six cases (10·5%), CTNNB1 (S45C, L46P and S45P) in six cases (10·5%) and CTNNB1 plus GNAS in two cases (3·5%). PRKACA and GNAS mutations were mutually exclusive. Among the patients with nonfunctional adenoma, two carried CTNNB1 mutations. Among the patients with ACC, two carried GNAS and CTNNB1 mutations but none carried PRKACA mutations. One patient showed bilateral CPA, and one PBMAH patient carried PRKACA mutations. No mutations in PRKACA, GNAS or CTNNB1 were identified in the eight patients with aldosterone and cortisol cosecreting adenomas. PRKACA-mutant adenomas were associated with young age, overt Cushing's syndrome and high cortisol levels compared with non-PRKACA-mutant or CTNNB1-mutant lesions.
CONCLUSIONS: PRKACA mutations are present in CPAs and bilateral adrenal macronodular hyperplasia. PRKACA mutation is associated with more severe autonomous cortisol secretion.

PMID: 27296931 [PubMed - indexed for MEDLINE]

Procedural and clinical outcomes of percutaneous adrenal biopsy in a high-risk population for adrenal malignancy.

Fetched: September 24th, 2017, 5:00am GMT

Procedural and clinical outcomes of percutaneous adrenal biopsy in a high-risk population for adrenal malignancy.

Clin Endocrinol (Oxf). 2016 Nov;85(5):710-716

Authors: Delivanis DA, Erickson D, Atwell TD, Natt N, Maraka S, Schmit GD, Eiken PW, Nathan MA, Young WF, Bancos I

Abstract
OBJECTIVE: The role of percutaneous adrenal biopsy in a high-risk population for adrenal malignancy has not been fully investigated. Our aim was to describe the clinical presentation leading to the adrenal biopsy and evaluate the diagnostic performance, complications and non diagnostic rate of adrenal biopsy.
DESIGN: Single-centre, retrospective cohort study.
PATIENTS AND MEASUREMENTS: Medical records of patients who underwent adrenal biopsy between 1994 and 2014 were reviewed. Adrenal biopsy outcome was compared to a predefined reference standard.
RESULTS: Biopsy was performed in 418 patients [62% men, median age 69 years (range, 15-91)] on 419 adrenal lesions, median size 3·1 cm (range, 0·6-24). The main indication for adrenal mass biopsy was (349/419, 83%) suspected adrenal metastasis from a known or suspected extra-adrenal primary source. Only 116 of 419, 28% of cases had prebiopsy biochemical testing for pheochromocytoma. Biopsy-related complications occurred in 4% of the patients. Histology revealed a metastasis in 231 of 419 (55%), benign adrenal tissue in 137 of 419 (33%), adrenocortical carcinoma in eight of 419 (2%), other lesions in 23 of 419 (5%) including seven cases of pheochromocytoma and six cases of infectious process. Biopsy was nondiagnostic in 20 of 419 (5%). All adrenal masses with unenhanced radiodensity ≤10 HU (42/137, 31%) proved to be benign adrenal adenomas. Adrenal biopsy diagnosed malignancy with a sensitivity of 88·5%, specificity of 91·5%, positive predictive value of 93·4% and negative predictive value of 85·5%.
CONCLUSION: When used in the appropriate clinical setting, adrenal biopsy is a powerful tool in the diagnostic algorithm of the evaluation of adrenal masses with features suspicious for malignancy. Efforts to increase awareness to perform biochemical testing for pheochromocytoma prior to adrenal biopsy are needed.

PMID: 27248805 [PubMed - indexed for MEDLINE]

Permalink for 'Bilateral aldosterone suppression and its resolution in adrenal vein sampling of patients with primary aldosteronism: analysis of data from the WAVES-J study.'

Bilateral aldosterone suppression and its resolution in adrenal vein sampling of patients with primary aldosteronism: analysis of data from the WAVES-J study.

Fetched: September 24th, 2017, 5:00am GMT

Bilateral aldosterone suppression and its resolution in adrenal vein sampling of patients with primary aldosteronism: analysis of data from the WAVES-J study.

Clin Endocrinol (Oxf). 2016 Nov;85(5):696-702

Authors: Shibayama Y, Wada N, Umakoshi H, Ichijo T, Fujii Y, Kamemura K, Kai T, Sakamoto R, Ogo A, Matsuda Y, Fukuoka T, Tsuiki M, Suzuki T, Naruse M

Abstract
CONTEXT: In adrenal vein sampling (AVS) for patients with primary aldosteronism, the contralateral ratio of aldosterone/cortisol (A/C) between the nondominant adrenal vein and the inferior vena cava is one of the best criteria for determining lateralized aldosterone secretion. Despite successful cannulation in some patients, the A/C ratios in the adrenal veins are bilaterally lower than that in the inferior vena cava (bilateral aldosterone suppression; BAS).
OBJECTIVES: To investigate the prevalence of BAS in AVS and how to resolve this condition.
DESIGN AND SETTING: Retrospective study involving nine referral centres.
PATIENTS: Four hundred and ninety-one patients who were confirmed as having primary aldosteronism and had an AVS between January 2006 and December 2013.
MEASUREMENTS: The prevalence of BAS before and after ACTH stimulation was compared. In addition, we investigated other methods for overcoming BAS.
RESULTS: In 304 patients with successful AVS before ACTH stimulation, BAS was observed in 29 (9·5%). BAS was resolved after ACTH stimulation in 22 patients. In 276 patients with successful AVS both before and after ACTH stimulation, the frequency of BAS was significantly reduced after ACTH (8·7% vs 2·5%, P < 0·01). In a few patients, BAS was also resolved by adding a sampling point at the common trunk of the left adrenal vein and by an alternative drainage vein from the adrenal tumour.
CONCLUSIONS: BAS sometimes occurs in AVS without ACTH stimulation. ACTH stimulation significantly reduces BAS with a single AVS procedure.

PMID: 27128234 [PubMed - indexed for MEDLINE]

[Carney triad].

Fetched: September 23rd, 2017, 5:00am GMT

[Carney triad].

Rozhl Chir. 2017;96(6):267-272

Authors: Fiala L, Kocáková I, Šimůnek R, Krejčí E, Babánková I, Šefr R

Abstract
Carney triad is a synchronous or metachronous association of gastric gastrointestinal stromal tumors (GIST), pulmonary chondroma and extra-adrenal paraganglioma. The majority of patients have only one or two components of the triad, all three tumors being found in only about 2% of the patients at the time of the first diagnosis. The most common combination is gastric and pulmonary tumors. We report a case of Carney triad which was diagnosed at Masaryk Memorial Cancer Institute. A 57-year-old female patient with a history of gastric resection for leiomyosarcoma at the age of 14 and with an unclear pulmonary lesion evident on chest X-ray since as early as 2003. She was referred to our Clinic of Comprehensive Cancer Care after being diagnosed with unspecified tumors of the stomach, the left retroperitoneum and two liver metastases. Biopsy of the retroperitoneal mass was performed and histological examination showed pheochromocytoma. The patient underwent resection of the retroperitoneal tumor and wedge resection of the gastric tumor, left hemihepatectomy and left adrenalectomy (in two separate operations). The excised gastric tumor was a gastrointestinal stromal tumor (GIST) with a low risk of malignancy. Analysis of a liver specimen, however, showed two GIST metastases. No pathology was found in the left adrenal gland and the retroperitoneal tumor was positive for chromogranin A. Paraganglioma was thus diagnosed. Subsequently, mutational analysis of genes coding for succinate dehydrogenase subunits B, C and D (SDHB, SDHC, SDHD) and analysis of DNA methylation at the gene locus of SDHC was made. Carney triad was thus confirmed and the unclear pulmonary lesion could be described as benign chondroma. This report demonstrates the difficulty in distinguishing between Carney triad and Carney-Stratakis syndrome. Molecular information should improve the diagnosis of Carney triad.Key words: Carney triad - GIST pulmonary chondroma extraadrenal paragangliomaCarney-Stratakis syndrome.

PMID: 28931294 [PubMed - in process]

Adrenal Lesions in Patients With (Attenuated) Familial Adenomatous Polyposis and MUTYH-Associated Polyposis.

Fetched: September 23rd, 2017, 5:00am GMT

Adrenal Lesions in Patients With (Attenuated) Familial Adenomatous Polyposis and MUTYH-Associated Polyposis.

Dis Colon Rectum. 2017 Oct;60(10):1057-1064

Authors: Kallenberg FGJ, Bastiaansen BAJ, Nio CY, Soeters MR, Boermeester MA, Aalfs CM, Bossuyt PMM, Dekker E

Abstract
BACKGROUND: The reported proportion of patients with familial adenomatous polyposis who have adrenal lesions varies between 7% and 13% compared with 4% in the general population; the prevalence of adrenal lesions in patients with attenuated familial adenomatous polyposis and MUTYH-associated polyposis is unknown. Data on the clinical relevance and clinical course are limited.
OBJECTIVE: We aimed to report on the frequency, characteristics, and progression of adrenal lesions in polyposis patients.
DESIGN: This was a historical cohort study.
SETTINGS: The study was performed at the Academic Medical Center, Amsterdam.
PATIENTS: All of the patients with familial adenomatous polyposis, attenuated familial adenomatous polyposis, and MUTYH-associated polyposis were included. Medical charts and imaging reports were analyzed for data on adrenal lesions. A radiologist reassessed all of the images. Patients had not routinely been screened for adrenal lesions.
MAIN OUTCOME MEASURES: The frequency, characteristics, and progression of adrenal lesions in patients with polyposis who underwent abdominal imaging were assessed. Findings were compared with a reference.
RESULTS: A total of 39 adrenal lesions were identified in 23 (26%) of 90 patients with familial adenomatous polyposis, 2 (18%) of 11 with attenuated familial adenomatous polyposis, and 5 (24%) of 21 with MUTYH-associated polyposis. Mean age at time of detection was 50.7 years (range, 17.1-83.3 y). Median lesion size at baseline was 1.4 cm (range, 1.0-5.0 cm) versus 1.7 cm (range, 1.0-5.7 cm) after a median of 3.5 years (range, 1.0-11.4 y). Two patients were diagnosed with a hyperfunctioning lesion, and 4 underwent adrenalectomy: 3 lesions appeared benign, and 1 was oncocytic of uncertain malignant potential. The OR for detecting at least 1 lesion in a patient with polyposis versus reference was 6.2 (95% CI, 3.2-12.3), with no significant differences in ORs among the 3 syndromes.
LIMITATIONS: The study was limited by its retrospective design.
CONCLUSIONS: Adrenal lesions are frequent in patients with polyposis who undergo abdominal imaging. They appear to follow a benign and slowly progressive course and are mostly nonhyperfunctioning. See Abstract Video at [links.lww.com]

PMID: 28891849 [PubMed - indexed for MEDLINE]

EZH2 is overexpressed in adrenocortical carcinoma and is associated with disease progression.

Fetched: September 23rd, 2017, 5:00am GMT

EZH2 is overexpressed in adrenocortical carcinoma and is associated with disease progression.

Hum Mol Genet. 2016 Jul 01;25(13):2789-2800

Authors: Drelon C, Berthon A, Mathieu M, Ragazzon B, Kuick R, Tabbal H, Septier A, Rodriguez S, Batisse-Lignier M, Sahut-Barnola I, Dumontet T, Pointud JC, Lefrançois-Martinez AM, Baron S, Giordano TJ, Bertherat J, Martinez A, Val P

Abstract
Adrenal Cortex Carcinoma (ACC) is an aggressive tumour with poor prognosis. Common alterations in patients include constitutive WNT/β-catenin signalling and overexpression of the growth factor IGF2. However, the combination of both alterations in transgenic mice is not sufficient to trigger malignant tumour progression, suggesting that other alterations are required to allow development of carcinomas. Here, we have conducted a study of publicly available gene expression data from three cohorts of ACC patients to identify relevant alterations. Our data show that the histone methyltransferase EZH2 is overexpressed in ACC in the three cohorts. This overexpression is the result of deregulated P53/RB/E2F pathway activity and is associated with increased proliferation and poorer prognosis in patients. Inhibition of EZH2 by RNA interference or pharmacological treatment with DZNep inhibits cellular growth, wound healing and clonogenic growth and induces apoptosis of H295R cells in culture. Further growth inhibition is obtained when DZNep is combined with mitotane, the gold-standard treatment for ACC. Altogether, these observations suggest that overexpression of EZH2 is associated with aggressive progression and may constitute an interesting therapeutic target in the context of ACC.

PMID: 27149985 [PubMed - indexed for MEDLINE]

Local Control and Survival after Image-Guided Percutaneous Ablation of Adrenal Metastases.

Fetched: September 22nd, 2017, 5:00am GMT

Local Control and Survival after Image-Guided Percutaneous Ablation of Adrenal Metastases.

J Vasc Interv Radiol. 2017 Sep 16;:

Authors: Frenk NE, Daye D, Tuncali K, Arellano RS, Shyn PB, Silverman SG, Fintelmann FJ, Uppot RN

Abstract
PURPOSE: To evaluate local control and survival after image-guided ablation of adrenal gland metastases.
MATERIALS AND METHODS: Image-guided ablations of adrenal metastases measuring < 5 cm performed at 2 academic medical centers between July 2002 and June 2016 were analyzed. There were 51 procedures performed on 46 tumors (mean diameter 2.8 cm ± 1.1; range, 0.7-4.9 cm) in 38 patients (mean age 66 y; range, 41-80 y) with renal cell carcinoma (n = 17 patients; 45%), non-small cell lung cancer (n = 10 patients; 26%), and other primary malignancies (n = 11 patients; 29%). Treatment modalities included cryoablation (n = 30 procedures; 59%), radiofrequency ablation (n = 12 procedures; 24%) and microwave ablation (n = 9 procedures; 18%). Technical success, primary and secondary efficacy, local progression rate, local progression-free survival, and overall survival were assessed. Mean follow-up was 37 months (range, 2-128 months). Statistical analysis was performed with univariate Cox hazards regression and Kaplan-Meier analyses.
RESULTS: Technical success, primary efficacy, and secondary efficacy were 96%, 72%, and 76%. Local progression rate during all follow-up was 25%. Local tumor progression-free survival at 1, 3, and 5 years was 82%, 69%, and 55%. Overall survival at 1, 3, and 5 years was 82%, 44%, and 34%. In 16 patients with isolated adrenal metastasis, median disease-free survival was 8 months; 4 patients had no evidence of disease during follow-up. Lung cancer was associated with decreased survival (hazard ratio 4.41, P = .002).
CONCLUSIONS: Image-guided ablation can achieve local control for adrenal metastases < 5 cm.

PMID: 28927661 [PubMed - as supplied by publisher]

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