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World J Gastroenterol. Dec 7, 2017; 23(45): 7945-7951
Published online Dec 7, 2017. doi: 10.3748/wjg.v23.i45.7945
Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors
Ahmad Al-Hader, Rami N Al-Rohil, Haiyong Han, Daniel Von Hoff
Ahmad Al-Hader, Division of Hematology/Oncology, Indiana University School of Medicine, Indianapolis, IN 46202-3082, United States
Rami N Al-Rohil, Department of Pathology, Vanderbilt University School of Medicine, Nashville, TN 37232, United States
Haiyong Han, Daniel Von Hoff, Molecular Medicine Division, Translational Genomics Research Institute (TGen), Phoenix, AZ 85004, United States
Author contributions: Al-Hader A wrote the paper; Al-Rohil RN provided us with the pathology figures; Han H and Von Hoff D reviewed and edited the paper.
Conflict-of-interest statement: We declare that we have no conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Ahmad Al-Hader, MD, Department of Medicine, Division of Hematology/Oncology, Indiana School of Medicine, Indianapolis, IN 46202, United States. aalhader@iu.edu
Telephone: +1-370-8807078 Fax: +1-370-8800396
Received: July 3, 2017
Peer-review started: July 3, 2017
First decision: July 28, 2017
Revised: September 17, 2017
Accepted: October 26, 2017
Article in press: October 26, 2017
Published online: December 7, 2017
Processing time: 154 Days and 1.4 Hours
Abstract

Pancreatic carcinomas with acinar differentiation are rare, accounting for 1%-2% of adult pancreatic tumors; they include pancreatic acinar cell carcinoma (PACC), pancreatoblastoma, and carcinomas of mixed differentiation. Patients with PACC have a prognosis better than pancreatic ductal adenocarcinomas but worse than pancreatic neuroendocrine tumors. Reports of overall survival range from 18 to 47 mo. A literature review on PACCs included comprehensive genomic profiling and whole exome sequencing on a series of more than 70 patients as well as other diagnostic studies including immunohistochemistry. Surgical resection of PACC is the preferred treatment for localized and resectable tumors. The efficacy of adjuvant treatment is unclear. Metastatic PACCs are generally not curable and treated with systemic chemotherapy. They are moderately responsive to chemotherapy with different regimens showing various degrees of response in case reports/series. Most of these regimens were developed to treat patients with pancreatic ductal adenocarcinomas or colorectal adenocarcinomas. Review of PACC’s molecular profiling showed a number of gene alterations such as: SMAD4, BRAF, BRCA2, TP53, RB1, MEN1, JAK-1, BRCA-1, BRCA-2, and DNA mismatch repair abnormalities. PACCs had multiple somatic mutations with some targetable with available drugs. Therefore, molecular profiling of PACC should be an option for patients with refractory PACC.

Keywords: Pancreatic acinar cell carcinoma; Molecular profiling; Targeted therapy

Core tip: This is a review article on pancreatic acinar cell carcinoma, which is a rare type of pancreatic cancer, with a series of molecularly profiled cases and an insight on how to potentially target specific mutations and genetic abnormalities with different systemic treatments including tyrosine kinase inhibitors, immunotherapy and cytotoxic chemotherapy agents.