Overview

NCI Definition: An adenocarcinoma arising from the pancreas. It is characterized by the presence of relatively uniform malignant cells which form acinar patterns. It usually occurs during adulthood. Signs and symptoms include abdominal pain, weight loss, nausea, and diarrhea. It may metastasize to regional lymph nodes and the liver. A minority of patients develop lipase hypersecretion syndrome. This syndrome may be seen in patients with liver metastases and it is characterized by excessive secretion of lipase in the serum, polyarthralgia, and subcutaneous fat necrosis. [1]

Pancreatic acinar cell carcinomas most frequently harbor alterations in CDKN2A, SMAD4, BRAF, CDKN2B, and AXIN1 [2].

Most Commonly Altered Genes in Pancreatic Acinar Cell Carcinoma

CDKN2A Loss, CDKN2B Loss, SMAD4 Mutation, CTNNB1 Mutation, and BRAF Fusion are the most common alterations in pancreatic acinar cell carcinoma [2].

Top Alterations in Pancreatic Acinar Cell Carcinoma

Significant Genes in Pancreatic Acinar Cell Carcinoma

CD274 +

Disease Details

Synonyms
Pancreatic Acinar Cell Adenocarcinoma, Acinar Cell Carcinoma of the Pancreas, Acinar Cell Carcinoma of Pancreas, Carcinoma, favor acinar cell carcinoma, Acinar Cell Adenocarcinoma of Pancreas, Pancreas Acinar Cell Adenocarcinoma, Acinar Cell Adenocarcinoma of the Pancreas
Parent(s)
Pancreatic Adenocarcinoma
OncoTree Name
Acinar Cell Carcinoma of the Pancreas
OncoTree Code
PAAC

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.