Pancreatic Acinar Cell Carcinoma
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. 
Pancreatic acinar cell carcinomas most frequently harbor alterations in SMAD4, CDKN2A, CDKN2B, and BRAF .
SMAD4 Mutation, CDKN2B Loss, and CDKN2A Loss are the most common alterations in pancreatic acinar cell carcinoma .
There is 1 clinical trial for pancreatic acinar cell carcinoma, of which 1 is open and 0 are completed or closed. Of the trial that contains pancreatic acinar cell carcinoma as an inclusion criterion, 1 is phase 2 (1 open).
Ipilimumab and nivolumab are the most common interventions in pancreatic acinar cell carcinoma clinical trials.
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 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.