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.
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Epidemiology
This tumor is most common in pediatric (8-15 years) and adult (60 years) populations. There is a male predominance 6.
Clinical presentation
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.
Radiographic features
CT
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
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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 encapsulationÂ
Treatment and prognosis
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.