Acinar cell carcinoma of the pancreas

Pancreatic acinar cell carcinoma is a rare exocrine neoplasm that comprises ~1% of all pancreatic tumors. This tumor shows more aggressive behavior than the far more common adenocarcinoma ^1,3,4.

Clinical presentation

High levels of serum lipase, due to hypersecretion syndrome, resulting in subcutaneous fat necrosis form part of its presentation ^2,3,5.

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

• non-biliary duct dilation

  • only 17% of cases demonstrate bile duct dilatation

  • 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

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.