Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors.

Journal Article (Journal Article;Review)

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.

Full Text

Duke Authors

Cited Authors

  • Al-Hader, A; Al-Rohil, RN; Han, H; Von Hoff, D

Published Date

  • December 7, 2017

Published In

Volume / Issue

  • 23 / 45

Start / End Page

  • 7945 - 7951

PubMed ID

  • 29259370

Pubmed Central ID

  • PMC5725289

Electronic International Standard Serial Number (EISSN)

  • 2219-2840

Digital Object Identifier (DOI)

  • 10.3748/wjg.v23.i45.7945


  • eng

Conference Location

  • United States