Pancreatic acinar cell carcinoma

Last revised by Arlene Campos on 6 Mar 2024

Pancreatic acinar cell carcinomas are rare exocrine neoplasms that comprise ~1% of all pancreatic tumors. This tumor shows more aggressive behavior than the far more common adenocarcinoma 1,3,4.

This tumor is most common in pediatric (8-15 years) and adult (60 years) populations. There is a male predominance 6.

Presenting symptoms are non-specific including weight loss, abdominal and/or back pain, and nausea and vomiting 6. Pancreatic acinar cell carcinoma can present with lipase hypersecretion syndrome (serum lipase levels >10,000 U/dL), which can result in presentation of Schmid's triad with subcutaneous fat necrosis, polyarthropathy, and serum eosinophilia 2,3,5,6.

  • on contrast-enhanced CT, generally presents as an enhancing, ovoid, solid pancreatic tumor

  • variable hypo/hyperattenuating mass relative to the pancreas, with well-distinguished margins

  • non-biliary duct dilation

    • ~15 (range 12-17%) of cases demonstrate bile duct dilatation 6

    • this is one of the differentiating features from adenocarcinoma 1

  • hypovascular tumor with adjacent organ invasion

  • internal calcification (50% of cases)

  • tumor encapsulation 

  • peripancreatic lymph nodes

In cases of disease confinement to the pancreas, surgical resection is curative and in cases of distant metastasis, surgery followed by chemotherapy and radiotherapy is the treatment of choice 1,2,4.

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