Minireviews
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Oct 28, 2022; 28(40): 5827-5844
Published online Oct 28, 2022. doi: 10.3748/wjg.v28.i40.5827
Pancreatic acinar cell carcinoma: A comprehensive review
Luis Fernando Calimano-Ramirez, Taher Daoud, Dheeraj Reddy Gopireddy, Ajaykumar C Morani, Rebecca Waters, Kazim Gumus, Albert Russell Klekers, Priya R Bhosale, Mayur K Virarkar
Luis Fernando Calimano-Ramirez, Department of Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
Taher Daoud, Ajaykumar C Morani, Albert Russell Klekers, Priya R Bhosale, Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
Dheeraj Reddy Gopireddy, Mayur K Virarkar, Department of Diagnostic Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
Rebecca Waters, Department of Pathology and Lab Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
Kazim Gumus, Department of Research and Diagnostic Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
Author contributions: All authors equally contributed to this paper with the conception and design of the study, literature review and analysis, drafting, critical revision, editing, and approval of the final version.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Luis Fernando Calimano-Ramirez, MD, Research Assistant, Department of Radiology, University of Florida College of Medicine Jacksonville, 655 8th St. W 2nd Floor, Jacksonville, FL 32209, United States. luis.calimanoramirez@jax.ufl.edu
Received: August 25, 2022
Peer-review started: August 25, 2022
First decision: September 8, 2022
Revised: September 14, 2022
Accepted: October 14, 2022
Article in press: October 14, 2022
Published online: October 28, 2022
Abstract

Acinar cell carcinoma (ACC) is a rare pancreatic malignancy with distinctive clinical, molecular, and morphological features. The long-term survival of ACC patients is substantially superior to that of pancreatic adenocarcinoma patients. As there are no significant patient series about ACCs, our understanding of this illness is mainly based on case reports and limited patient series. Surgical resection is the treatment of choice for patients with the disease restricted to one organ; however, with recent breakthroughs in precision medicine, medicines targeting the one-of-a-kind molecular profile of ACC are on the horizon. There are no standard treatment protocols available for people in which a total surgical resection to cure the condition is not possible. As a result of shared genetic alterations, ACCs are chemosensitive to agents with activity against pancreatic adenocarcinomas and colorectal carcinomas. The role of neoadjuvant or adjuvant chemoradiotherapy has not been established. This article aims to do a comprehensive literature study and present the most recent information on acinar cell cancer.

Keywords: Acinar cell carcinoma, Pancreas, Imaging, Immunohistochemical stains, Molecular features, Surgery, Chemotherapy

Core Tip: Acinar cell carcinoma (ACC) of the pancreas is a rare, malignant neoplasm that accounts for a small percentage of all pancreatic neoplasms. Our understanding of this disease remains unclear as there are no large series of patients with ACC. This review article aims to conduct a comprehensive literature review and present current knowledge about ACC.