Acinar cell carcinoma of the pancreas

Case contributed by Michael P Hartung
Diagnosis certain

Presentation

Abdominal pain. Lipase 8000.

Patient Data

Age: 50 years
Gender: Female

Tumor thrombus expanding the portal and splenic veins. Very large mass arising from the pancreas tail invading into and massively enlarging the spleen. Numerous smaller surrounding tumor nodules along the anterior and inferior aspects. Right adrenal nodule. Small ascites. 

MRI features of the CT findings detailed above. Note of slightly heterogenous hepatobiliary phase activity in the liver parenchyma related to tumor thrombus.

Ultrasound-guided biopsy

Ultrasound-guided biopsy of the spleen showing heterogeneous infiltration of the mass throughout the parenchyma. Two of three total biopsy passes are shown. 

Case Discussion

Ultrasound-guided biopsy of the spleen was performed leading to the rare diagnosis of pancreas acinar cell carcinoma. 

Pancreas acinar cell carcinoma highlights:

  • very rare <1%
  • exocrine tumor
  • larger size at diagnosis and often better prognosis than adenocarcinoma
  • some have paraneoplastic syndrome with hypersecretion of lipase (as in this case), which can lead to fat necrosis of subcutaneous nodules and polyarthralgia

On physical exam, this patient did have subcutaneous nodules in the lower extremities which would be clinically consistent with fat necrosis related to hyperlipasemia. 

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