Clinico-pathological and Molecular Features of Pancreatic Acinar Cell Carcinoma: Association with BRCA2 Mutation

Abstract

Abstract Introduction/Objective Pancreatic acinar cell carcinoma (ACC) is a rare neoplasm of exocrine epithelial origin and accounts to <1% of primary neoplasms of pancreas. Methods/Case Report The cases diagnosed as ACC were retrieved from Pathology data base between January 2010 to July 2021. A retrospective review of clinical, pathological, molecular findings along with follow up was performed. Results (if a Case Study enter NA) Eight patients with resection specimens were identified over a period of 11 years. Median age of diagnosis was 74.5 yrs with M: F ratio of 3:1 and size range of 1.8 to 10.9 cm. The tumor was located in body/tail (5) and head of pancreas (3). Immunohistochemical stains for BCL10 and trypsin were utilized in 4 cases. One patient had lymph node metastasis and two patients had liver metastasis at the time of presentation. One patient presented with lipase hypersecretion syndrome. Four cases (50%) had pT3 stage. Follow-up period was available in 7 patients and ranged from 1-3 yrs, of which 3 died within 2 yrs of surgery while 4 patients were disease free. Three patients (35%) were found to have germline (1) and somatic BRCA2 mutations (2). These patients received platinum- based chemotherapy and were free from recurrence upon follow-up of 3 yrs. Conclusion Pancreatic ACC is a rare entity. In our study, 35% (3/8) cases were associated with somatic/germline BRCA2 mutations. Our results suggest that patients with ACC should undergo genetic testing for BRCA 1/2 mutations. This would not only enable treatment of these patients with specific targeted therapies, but also aid in screening of their families.