Published online Oct 28, 2022. doi: 10.3748/wjg.v28.i40.5827
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
Processing time: 63 Days and 21.8 Hours
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.
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.