Pancreatic acinar cell carcinoma: A comprehensive review

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 und...

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Published inWorld journal of gastroenterology : WJG Vol. 28; no. 40; pp. 5827 - 5844
Main Authors Calimano-Ramirez, Luis Fernando, Daoud, Taher, Gopireddy, Dheeraj Reddy, Morani, Ajaykumar C, Waters, Rebecca, Gumus, Kazim, Klekers, Albert Russell, Bhosale, Priya R, Virarkar, Mayur K
Format Journal Article
LanguageEnglish
Published United States Baishideng Publishing Group Inc 28.10.2022
Subjects
Carcinoma, Acinar Cell - genetics
Carcinoma, Acinar Cell - pathology
Carcinoma, Acinar Cell - therapy
Humans
Immunohistochemistry
Minireviews
Mutation
Pancreatic Neoplasms
Pancreatic Neoplasms - genetics
Pancreatic Neoplasms - pathology
Pancreatic Neoplasms - therapy
Acinar cell carcinoma
Chemotherapy
Imaging
Surgery
Immunohistochemical stains
Pancreas
Molecular features
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Summary: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.
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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
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.
ISSN:1007-9327
2219-2840
DOI:10.3748/wjg.v28.i40.5827