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Hum Pathol. 2023 Jan 23:S0046-8177(23)00026-6. doi: 10.1016/j.humpath.2023.01.007. Online ahead of print.
ABSTRACT
This study assessed the expression of GATA3 in primary lung carcinomas and correlated it with tumor histology and immunostains routinely utilized in the work up of primary lung cancers. Tissue microarrays (TMAs) were constructed from a cohort of 184 non-small cell carcinomas, stained with GATA3, p40, TTF-1, and napsin A, and analyzed semi-quantitatively. All TMA cases with GATA3 expression were further analyzed using corresponding whole slide sections. Positive GATA3 staining was present in sixteen cases (9%), including 7 squamous cell carcinomas (SqCCs) (4%), 4 adenocarcinomas (AdCs) (2%), 2 adenosquamous carcinomas (AdSqCs) (1%), 2 large cell carcinomas (LCCs) (1%), and 1 sarcomatoid carcinoma (SC) (<1%). Among tumor histotypes, SqCC was more likely to stain with GATA3 (7/49, 14%), while AdC was less likely (4/111, 4%) (p = 0.04). In GATA3-positive cases, high-level expression was observed in 9 cases (56%), including 5 p40-positive SqCCs (3 were nonkeratinizing), 1 p40-positive AdSqC negative for TTF-1 and napsin A, and 1 AdC (solid), 1 LCC, and 1 SC, each negative for p40, TTF-1, and napsin A. Low-level GATA3 expression was found in 3 AdCs (1 was lepidic and 2 were acinar predominant), 2 SqCCs (keratinizing), 1 AdSqC, and 1 LCC. These findings indicate that GATA3 expression occurs in a minor but significant proportion of primary non-small cell lung carcinomas, most often involves SqCC, and tends to show increasing levels of expression in more poorly differentiated subtypes. Caution should be exercised when interpreting GATA3 expression, and a panel of immunostains should be utilized when assigning tumor origin.
PMID:36702357 | DOI:10.1016/j.humpath.2023.01.007
Cancer Med. 2023 Jan 23. doi: 10.1002/cam4.5629. Online ahead of print.
ABSTRACT
BACKGROUND: Our objective was to correlate cytomorphological features of metastatic non-small cell lung carcinoma (mNSCLC) with maximal standardized uptake value (mSUV) of positron emission tomography (PET) in Lymph nodes (LNs).
METHODS: Positive cytology slides of 114 LNs were reviewed from 100 patients with mNSCLC who had undergone PET study. Student's t-test was used for statistical comparisons.
RESULTS: Mean patients' age: 68.5, 54% male. LNs locations were: mediastinum: 99, lung hilum: 13, peribronchial: 1, axilla: 1. Final diagnoses were: Adenocarcinoma: 86, squamous cell carcinoma: 28 LNs. Within the adenocarcinoma subgroup, histological patterns correlate with mSUV. Acinar and papillary patterns were associated with significantly lower mSUVs (mean ± standard error (SE): 7.9 ± 0.9 and 9.2 ± 0.8, respectively) than solid pattern (13.0 ± 1.2; p values: 0.001 and 0.009, respectively). Similar difference exists between patterns associated with low- and high-grade adenocarcinoma (Mean ± SE: 9.2 ± 0.8 and 12.0 ± 1.0, respectively. p value: 0.02). Interestingly, micropapillary pattern was associated with the lowest mSUV amongst all patterns (Mean ± SE: 5.4 ± 1.1). Other features that correlated with higher mSUV were necrosis, moderate/severe nuclear atypia, lower lymphoid tissue yield, and contralateral LN involvement.
CONCLUSIONS: In LNs with mNSCLC, certain cytomorphological features are associated with higher mSUV. Micropapillary, a pattern considered as high-grade, is associated with lower SUV values; hence, a lower SUV threshold may raise concern for metastasis. Although high SUV is associated with LN metastasis, lower SUV levels in certain adenocarcinomas suggest correlation with clinical and morphological characteristics could be valuable in tailoring therapeutic management.
PMID:36691354 | DOI:10.1002/cam4.5629
Otolaryngol Pol. 2022 Sep 9;76(5):29-36. doi: 10.5604/01.3001.0015.9816.
ABSTRACT
INTRODUCTION Tumors of the salivary glands account for approximately 3 to 4% of all head and neck neoplasms. It is estimated that 10-15% of them are malignant. The most common benign tumor is pleomorphic adenoma, while the most common malignant tumors are adenoid cystic carcinoma and mucoepidermoid carcinoma. Neoplasms of the salivary glands are extremely histologically diverse, which results from the complex embryogenesis of the salivary glands. The identified risk factors for tumors of the salivary glands are: ultraviolet radiation, ionizing radiation, viral infections, nicotine and alcohol. MATERIAL AND METHOD The aim of the study was an epidemiological analysis of patients with salivary gland neoplasms, the distribution and histopathological characteristics of individual neoplasms treated at the Department of Otorhinolaryngology of the Medical University of Warsaw in 2010-2020. The diagnoses were analyzed according to the latest WHO 2017 histological classification of salivary gland tumors. In addition, the material was supplemented with data on the 5-year survival rates of patients with malignant neoplasms obtained from the Registry of Marital Status. RESULTS AND DISCUSSION The material contained 407 neoplasms of the salivary glands over a 11-year period, of which malignant neoplasms accounted for 17.4%. The malignant tumors were dominated by: adenoid cystic carcinoma (28,2%), mucoepidermoid carcinoma (12,7%), and acinic cell carcinoma (9,9%). Lymphomas (15,5%) were also a large group. The benign neoplasms were dominated by pleomorphic adenoma (54.1%) and Warthin's tumor (36%). Tumors of the salivary gland the most often affected the parotid gland (92%). CONCLUSIONS The obtained data are consistent with the general epidemiological data described in the current literature.
PMID:36622126 | DOI:10.5604/01.3001.0015.9816
Vet Pathol. 2023 Jan;60(1):8-20. doi: 10.1177/03009858221122517. Epub 2022 Sep 13.
ABSTRACT
Feline pulmonary carcinoma (FPC) is an uncommon neoplasm with unique morphological features. We describe the gross, histological, metastatic, and immunohistochemical aspects of FPC, based on postmortem examinations from an 11-year retrospective study. Thirty-nine cases were selected. Predispositions were observed in senior (P < .001) and Persian (P = .039) cats. There were three gross patterns of the pulmonary tumors: (a) a large nodule and additional smaller nodules, (b) a solitary nodule, and (c) small, multifocal to coalescent nodules. Extrapulmonary metastases were present in 22/39 cases (56.4%), mainly in the regional lymph nodes (17/39, 43.5%), skeletal muscles (9/39, 23%), kidneys (6/39, 15.3%), and parietal pleura (4/39, 10.2%). The primary tumor size was correlated with the occurrence of extrapulmonary metastases (P = .002). Histologically, the tumors were classified as papillary adenocarcinoma (19/39, 48.7%), adenosquamous carcinoma (ADS) (8/39, 20.5%), acinar adenocarcinoma (6/39, 15.3%), solid adenocarcinoma (3/39, 7.6%), lepidic adenocarcinoma (2/39, 5.1%), and micropapillary adenocarcinoma (1/39, 2.5%). By immunohistochemistry, 39/39 cases (100%) were positive for pancytokeratin, 34/39 (87.1%) for thyroid transcription factor-1, and 8/39 (20.5%) for vimentin. Immunoreactivity for p40 was detected in the squamous component of all ADSs (8/8, 100%) and occasionally in the glandular component of adenocarcinomas (10/31, 32.2%). Napsin A expression was absent in all feline tissue tested. The results indicate that a modified and simplified histological classification based on current human and domestic animal systems is appropriate for cats. Additionally, this study highlights the utility of p40 as an immunohistochemical marker for the diagnosis of FPC with squamous differentiation.
PMID:36112908 | DOI:10.1177/03009858221122517
Acta Cytol. 2023 Jan 2. doi: 10.1159/000528882. Online ahead of print.
ABSTRACT
INTRODUCTION: This study aimed to clarify the diagnostic structural features in cytology specimens that are useful in subtyping non-small cell lung carcinoma (NSCLC) into adenocarcinoma (ADC) and squamous cell carcinoma (SQCC).
METHODS: Cytology specimens (n = 233) of NSCLCs, which included ADCs (n = 149) and SQCCs (n = 84), were analyzed. The following cytological features were evaluated: isolated cell, flat sheet, three-dimensional cluster with irregular arrangement, papillary-like structure, micropapillary-like structure, acinar-like structure, palisading pattern, protrusion of nuclei at the periphery of the cluster, honeycomb pattern, streaming arrangement, three-dimensional sheets with regular arrangement, flattening at the periphery of the cluster, fuzzy pattern at the periphery of the cluster, and mutual inclusion.
RESULTS: ADCs exhibited significantly higher frequencies of flat sheet (p < 0.001), papillary-like structure (p < 0.001), micropapillary-like structure (p = 0.028), acinar-like structure (p < 0.001) and protrusion of nuclei at the periphery of the cluster (p < 0.001) than SQCCs. The latter exhibited significantly higher frequencies of streaming arrangement (p < 0.001), three-dimensional sheets with regular arrangement (p < 0.001), flattening at the periphery of the cluster (p < 0.001), fuzzy pattern at the periphery of the cluster (p < 0.001), and mutual inclusion (p < 0.001) than ADCs.
DISCUSSION/CONCLUSION: Cytological structural features, such as flat sheet, papillary-like structure, micropapillary-like structure, acinar-like structure, and protrusion of nuclei at the periphery of the cluster, indicated ADC, whereas streaming arrangement, three-dimensional sheets with regular arrangement, flattening at the periphery of the cluster, fuzzy pattern at the periphery of the cluster, and mutual inclusion indicated SQCC. Paying attention to these cytological structural features can enable the accurate subtyping of NSCLC into ADC and SQCC.
PMID:36592626 | DOI:10.1159/000528882
BMJ Case Rep. 2022 Dec 30;15(12):e252910. doi: 10.1136/bcr-2022-252910.
ABSTRACT
A woman in her 80s was evaluated for a liver mass. She had a history of what was reported as pancreatic acinar cell carcinoma resected with a pancreatoduodenectomy 10 years prior at another institution. Liver biopsy showed metastatic pancreatoblastoma (PB) and staging imaging showed no evidence of additional metastatic disease. She underwent laparoscopic non-anatomic partial hepatectomy and recovered uneventfully. The liver pathology was reviewed along with slides from her initial pancreatoduodenectomy, and both were noted to be consistent with PB. PB contains similar histological characteristics to pancreatic acinar cell carcinoma. Distinguishing between the two diagnoses is critical for accurately defining the prevalence, clinical course and prognosis associated with PB.
PMID:36585053 | PMC:PMC9809231 | DOI:10.1136/bcr-2022-252910
Pathol Int. 2022 Dec 28. doi: 10.1111/pin.13302. Online ahead of print.
ABSTRACT
Acinic cell carcinoma (ACC) is an exceptionally rare type of breast carcinoma with a low-grade morphology and a favorable prognosis. It is postulated to be a type of invasive carcinoma arising in microglandular adenosis (MGA). We report a case of extensively spreading ACC of the breast with MGA-like features. Macroscopically, yellowish nodules were widely distributed throughout the right breast, up to the axilla, without mass formation. Microscopically, the tumor consisted of two distinct carcinoma components: one was multiple nodular lesions showing invasive carcinoma with fused solid nests, and the other was a widely spreading lesion exhibiting MGA-like features with uniform small single glands. Immunohistochemically, both components were negative for ER, PR, and HER2, and expressed EMA, S100 and lysozyme. The distinct morphology and molecular expression indicated ACC. The single glands in the MGA-like area lacked myoepithelial cells but were linearly surrounded by type IV collagen, a basement membrane component. This case supports the hypothesis that ACC and MGA have the same lineage and indicates that ACC is not necessarily a low-grade malignancy and can be aggressive.
PMID:36579416 | DOI:10.1111/pin.13302
Int J Surg Pathol. 2022 Dec 26:10668969221133351. doi: 10.1177/10668969221133351. Online ahead of print.
ABSTRACT
We report the histopathological, immunohistochemical (IHC), and molecular findings in 3 patients with adult pancreatoblastoma, including 2 with autopsy features. The tumors were located in the tail and body of the pancreas, and the 2 autopsy examinations revealed liver and lung metastases. Histopathologically the neoplasms were composed of solid epithelial elements with nested or trabecular growth patterns, fibrous stroma, and squamoid clusters. Keratin 19 was positive mainly in squamoid corpuscles, and trypsin or chymotrypsin was positive in the acinar component. Neuroendocrine differentiation was observed in all tumors, and nuclear β-catenin expression in 2 tumors. Despite nuclear β-catenin expression, CTNNB1 mutation was found only in tumor 2. APC mutation was detected in tumor 1, and SMAD4 as well as MEN1 mutations in tumor 3. This last tumor also revealed chromosomal instability with many chromosomal losses and gains. The follow-up showed regional or distant metastases in all patients. Two patients died of disease after 3 and 26 months of follow-up and 1 patient is alive with no evidence of disease 6 years and 2 months after surgery. Adult pancreatoblastoma can display genetic heterogeneity, diverse histological appearance, and overlapping IHC findings. As a result, the differential diagnosis with other adult pancreatic tumors, such as acinar cell carcinoma, neuroendocrine neoplasm, solid pseudopapillary neoplasm, and mixed tumors may be challenging, especially when dealing with limited tumor tissue. The identification of squamoid corpuscles is essential for diagnosis. Although molecular findings might provide useful information, the integration of clinical, radiological, and histopathological findings is essential in pancreatoblastoma diagnosis.
PMID:36573045 | DOI:10.1177/10668969221133351
Cell Rep. 2022 Dec 20;41(12):111837. doi: 10.1016/j.celrep.2022.111837.
ABSTRACT
SAG/RBX2 is an E3 ligase, whereas SHOC2 is a RAS-RAF positive regulator. In this study, we address how Sag-Shoc2 crosstalk regulates pancreatic tumorigenesis induced by KrasG12D. Sag deletion increases the size of pancreas and causes the conversion of murine pancreatic intraepithelial neoplasms (mPanINs) to neoplastic cystic lesions with a mechanism involving Shoc2 accumulation, suggesting that Sag determines the pathological process via targeting Shoc2. Shoc2 deletion significantly inhibits pancreas growth, mPanIN formation, and acinar cell transdifferentiation, indicating that Shoc2 is essential for KrasG12D-induced pancreatic tumorigenesis. Likewise, in a primary acinar 3D culture, Sag deletion inhibits acinar-to-ductal transdifferentiation, while Shoc2 deletion significantly reduces the duct-like structures. Mechanistically, SAG is an E3 ligase that targets SHOC2 for degradation to affect both Mapk and mTorc1 pathways. Shoc2 deletion completely rescues the phenotype of neoplastic cystic lesions induced by Sag deletion, indicating physiological relevance of the Sag-Shoc2 crosstalk. Thus, the Sag-Shoc2 axis specifies the pancreatic tumor types induced by KrasG12D.
PMID:36543126 | DOI:10.1016/j.celrep.2022.111837
BMJ Case Rep. 2022 Dec 22;15(12):e251400. doi: 10.1136/bcr-2022-251400.
ABSTRACT
Pancreatic acinar cell carcinoma is a rare type of pancreatic malignancy, which can be confused with pancreatic neuroendocrine neoplasm. Here, we describe a woman in her 80s who presented with abdominal pain and bilateral lower extremity panniculitis. She underwent surgery for a presumed diagnosis of neuroendocrine tumour with PTEN and PRKAR1A alterations; 19 months, later, a recurrence of her pancreatic malignancy was discovered. The patient underwent repeat resection and this time immunohistochemical staining confirmed the diagnosis of acinar cell carcinoma. Staining for acinar cell carcinoma should be prompted based on clinical suspicion in context of PTEN or PRKAR1A mutation when appropriate.
PMID:36549753 | PMC:PMC9791417 | DOI:10.1136/bcr-2022-251400
Cytopathology. 2022 Dec 22. doi: 10.1111/cyt.13203. Online ahead of print.
ABSTRACT
Pancreatoblastoma (PB), a rare malignant epithelial neoplasm, is the most common pancreatic neoplasm of childhood. It is exceptionally rare in the adult population and its occurrence is limited to case reports. Although the neoplastic cells of PB can have a number of different directions of differentiation, PB is defined by the combination of neoplastic cells with acinar differentiation and squamoid morules. We report a case of a female patient in her 70s who presented with elevated creatinine level, concerning a kidney disorder, and was found to have an abdominal mass on CT scan. Fine needle aspiration (FNA) showed cellular smears with numerous 3-dimentional clusters of acinar cells and scattered squamoid morules. A cell block showed sheets of cells, some of which formed acini. Numerous squamoid morules were noted and were highlighted by nuclear labelling with antibodies to B-catenin in the cell block. The FNA diagnosis was rendered as "carcinoma with acinar differentiation, favour pancreatoblastoma." Subsequent histological findings confirmed the PB diagnosis. Next generation sequencing detected a CTNNB1 mutation. Given the wide usage of FNA in the preoperative diagnosis of pancreatic masses, the cytopathologist needs to be aware of the morphological features of PB and its cytological differential diagnosis, even in an elderly patient. The differential diagnosis includes acinar cell carcinoma, pancreatic neuroendocrine tumour, and solid pseudopapillary neoplasm. In conclusion, the cytological finding of neoplastic cells with acinar differentiation combined with squamous morules and/or mesenchymal elements in the smears and more commonly in the cell blocks appears to be the most specific finding for the diagnosis of PB.
PMID:36546760 | DOI:10.1111/cyt.13203
Biomater Sci. 2022 Dec 20;11(1):208-224. doi: 10.1039/d2bm00881e.
ABSTRACT
Pancreatic ductal adenocarcinoma (PDAC) mainly develops in the head of the pancreas, within the acino-ductal unit composed of acinar and ductal cells surrounded by pancreatic stellate cells (PSCs). PSCs strongly influence the tumor microenvironment by triggering an intense stromal deposition, which plays a key role in tumor progression and limits drug perfusion. We have developed a microfluidic in vitro model recreating the in vivo tumor-stroma crosstalk to replicate the steps of PDAC evolution towards the establishment of an efficient in vitro platform for innovative therapy validation. The multilayer PDAC-on-chip was designed to culture the PDAC cells and the PSCs embedded in a type I collagen gel in the top and bottom layers, respectively. The presence of a biomimetic nanofibrous membrane in the middle of the chip permits the control of interactions between the two cell lines and the easy analysis of the effects of the crosstalk on cell behavior. First, the PDAC-stromal cell relationship was evaluated under co-culture conditions on 24-well inserts including the PCL/Gel electrospun membrane. This simplified model shows that human fibroblasts change their morphology and secrete larger amounts of IL-6 cytokines in the presence of tumor cells, confirming the activation of stromal cells under co-culture. Then, the PDAC-on-chip system was validated by demonstrating that human fibroblasts seeded in a 3D collagen matrix in the bottom microchannel also change to a myofibroblast-like shape with increased expression of α-SMA and secrete larger amounts of IL-6 cytokines. This microfluidic system is suitable for the evaluation of drug efficacy and serves as a powerful tool for understanding the early evolution steps of PDAC.
PMID:36420859 | DOI:10.1039/d2bm00881e
Laryngorhinootologie. 2022 Dec 21. doi: 10.1055/a-1976-9694. Online ahead of print.
ABSTRACT
OBJECTIVE: Salivary gland carcinomas are rare and heterogeneous. More than 20 subtypes are recognized and risk factors are diverse. The aim of this work was to evaluate the subtype and other risk factors in a monocentric population from more than four decades.
MATERIAL AND METHODS: 205 cases (diagnosis period 1972-2014) were retrospectively collected and analyzed with regard to the distribution of risk factors and their influence on overall survival (OS).
RESULTS: 19/24 (79.2%) of the subtypes listed in the WHO classification occurred rarely in the cohort (< 5%). 10/24 (41.7%) of all subtypes were never diagnosed. With a total of 145/205 cases (70.7%), squamous cell carcinoma (PEC), adenocarcinoma (AdenoCa), acinar cell carcinoma (AcinarCa), mucoepidermoid carcinoma (MEC), and adenoid cystic carcinoma (ACC) were by far the most common subtypes. Risk factors are significantly different in these groups (e.g., lymphogenic metastasis and degree of differentiation in AdenoCa and age, T and UICC stage in PEC). The 5-year overall survival of all patients was 66.9% and differed significantly within the most common subtypes. An independent impact on overall survival was detectable for patient age (p<0.001), and T- (p=0.003) and N-stage (p=0.046) in multivariate analysis.
CONCLUSIONS: Most subtypes occurred markedly rarely or not at all within decades. The most common diagnoses differ with respect to risk factors as well as OS and 3 risk groups can be defined based on histology. In conclusion, considering TNM alone is insufficient for prognosis estimation in salivary gland carcinoma.
PMID:36543220 | DOI:10.1055/a-1976-9694
J Postgrad Med. 2023 Jan-Mar;69(1):50-52. doi: 10.4103/jpgm.jpgm_136_22.
ABSTRACT
Pancreatic panniculitis is a rare disease characterized by subcutaneous fat necrosis. It could be the result of an associated pancreatic tumor. Herein, we reported a 63-year-old man who presented with progressive bilateral lower limb edema accompanied with nodule-like lesions for 1 month. His serum lipase was 3,927 U/L (normal, 0-160 U/L). Histopathology of the skin specimen revealed lobular panniculitis, favoring a diagnosis of pancreatic panniculitis. Abdominal computed tomography (CT) scan with contrast showed a huge mass in his left upper quadrant. Endoscopic ultrasound showed a mixed echoic tumor, measuring 11.9 × 7.8 cm in dimensions, originating from the pancreatic tail. Biopsy performed via an endoscopic ultrasound showed a poorly differentiated acinar cell carcinoma. Because of the unresectable status of the tumor, the patient underwent chemotherapy with paclitaxel and gemcitabine. After chemotherapy, his skin lesions improved progressively. It is important to treat pancreatic panniculitis with its underlying pancreatic disease.
PMID:36537394 | DOI:10.4103/jpgm.jpgm_136_22
Am J Otolaryngol. 2022 Dec 8;44(2):103735. doi: 10.1016/j.amjoto.2022.103735. Online ahead of print.
ABSTRACT
BACKGROUND: The aim of the study was to investigate the oncological and functional outcome following extracapsular dissection as the sole form of treatment in locally limited low-grade malignant parotid tumours in the long term.
METHODS: The records of all patients treated for T1-T2 low-grade malignant tumours of the parotid gland solely by means of extracapsular dissection between 2005 and 2017 were studied retrospectively.
RESULTS: A total of 16 cases formed our study sample (7 men, 9 women). Their mean age was 50.2 years (21-84 years). Mean follow-up was 107 months (60-201 months). In 6 cases the tumour was an acinic cell carcinoma, in 9 cases a mucoepidermoid carcinoma and in one case a basal cell adenocarcinoma. Regarding the T category, the tumour was Tis in one case, T1 in 12 cases and T2 in three cases. The five-year disease-specific survival rate was 100 %, as was local disease control. Facial nerve function was House-Brackmann grade I without exception.
CONCLUSIONS: Our study showed very encouraging long-term results following primary extracapsular dissection as the sole surgical therapy for carefully selected low-stage, low-grade, inferiorly located lesions in patients with high compliance.
LAY SUMMARY: The "one-size-fits-all" strategy of complete parotidectomy with neck dissection might be tantamount to overtreatment by less aggressive cases of parotid cancer. Extracapsular dissection seems to be oncologically sufficient for carefully selected T1-T2 low-grade cases in the long-term in patients with ensured follow-up.
PMID:36535225 | DOI:10.1016/j.amjoto.2022.103735
World J Gastroenterol. 2022 Dec 7;28(45):6421-6432. doi: 10.3748/wjg.v28.i45.6421.
ABSTRACT
BACKGROUND: Pancreatic acinar cell carcinoma (PACC) is a rare tumor. Up to 45% of PACCs have alterations in the DNA damage repair pathway and 23% harbor rearrangements in the BRAF or RAF1 genes. We present a PACC case with a germline BRCA2 likely pathogenic variant (LPV) to highlight the impact of genomic testing on treatment decisions and patient outcomes. In our larger case series, we provide clinic-based information on additional 10 PACC patients treated in our center.
CASE SUMMARY: A 70-year-old male was diagnosed with advanced PACC. At presentation, he was cachectic with severe arthralgia despite prednisolone and a skin rash that was later confirmed to be panniculitis. He was treated with modified FOLFIRINOX (mFFX) with the knowledge of the germline BRCA2 LPV. Following 11 cycles of mFFX, a computed tomography (CT) scan demonstrated significant tumor response in the pancreatic primary and hepatic metastases, totaling 70% from baseline as per Response Evaluation Criteria in Solid Tumors. Resolution of the skin panniculitis was also noted. We identified two additional PACCs with druggable targets in our case series. Our data contribute to practical evidence for the value of germline and somatic profiling in the management of rare diseases like PACC.
CONCLUSION: This patient and others in our larger case series highlight the importance of genomic testing in PACC with potential utility in personalized treatment.
PMID:36533108 | PMC:PMC9753052 | DOI:10.3748/wjg.v28.i45.6421
Clin Case Rep. 2022 Dec 12;10(12):e6718. doi: 10.1002/ccr3.6718. eCollection 2022 Dec.
ABSTRACT
This 38-year-old man has a familial BRCA2 mutation. He presented with skin erythema, polyarthritis, dactylitis, and febrile erythema nodosum; a biopsy of a liver metastase revealed acinar cell carcinoma of the pancreas. After FOLFIRINOX, olaparib was initiated, and 24 months after, the patient was PS 0 and asymptomatic.
PMID:36523391 | PMC:PMC9744715 | DOI:10.1002/ccr3.6718