Solid pseudopapillary neoplasm

Case contributed by Dr Henry Knipe

Presentation

Work-up of incidental finding on CT chest.

Patient Data

Age: 25 years
Gender: Female
CT

Large mass centred within the pancreatic head.  Internally it is heterogeneous with a few specks of calcification anteriorly, intermediate density (40-50 HU) with some mild enhancement. Atrophic distal pancreas and dilated pancreatic duct.

The portal vein is contacted by the mass and there is a filling defect within the portal vein.

The common bile duct is compressed by the mass and there is extrahepatic and intrahepatic duct dilatation. No enlarged lymph nodes are identified. No focal liver lesion.

MRI

Large pancreatic head mass with heterogeneous T1 and T2 signal, including intrinsic high T1 signal compatible with blood product. There are enhancing internal septations.

Distally the pancreas is atrophied and there is mild pancreatic duct dilatation. Enhancing tissue also extends to the porta hepatis to possibly involve the right hepatic artery without clear involvement of the common or left hepatic arteries. Filling defect within the main portal vein at the porta hepatis with transmural invasion; thrombus extends to the confluence of the left and right portal veins.

The common bile duct is compressed by the mass and there is extrahepatic and intrahepatic duct dilatation with the common hepatic duct measuring 1.3 cm.

No enlarged lymph nodes are identified. No focal liver lesion.

The patient proceeded to EUS with FNA.

Histopathology

MACROSCOPIC DESCRIPTION: EUS-FNA Pancreatic Head Mass:  2 wet-fixed and 2 air-dried smears prepared. Also received in formalin multiple small cores of blood clot processed as a cell block.

MICROSCOPIC DESCRIPTION: The hypercellular smears and cell block section contain abundant neoplastic cells presenting as single cells, loosely cohesive clusters and branching papillary structures with fibrovascular cores. The neoplastic cells demonstrate monotonous round to oval nuclei, coarsely granular chromatin, inconspicuous nuclei and scant pale cytoplasm. Metachromatic hyaline globules are present. Immunohistochemical results show tumour cells stain: CKAE1/3 +/-, Vimentin+, CD56+, Chromogranin-, Synaptophysin+, B-catenin+ (nuclear), alpha-1-antitrypsin+; consistent with pancreatic solid pseudopapillary neoplasm.

DIAGNOSIS: Solid pseudopapillary neoplasm.

Case Discussion

Solid pseudopapillary neoplasm (SPEN) was suspected on the initial imaging, given the appearances of a low-density pancreatic mass in a young female, which is the typical demographic. A small percentage of SPEN are reported to be malignant, and there are features of local invasion and infiltration of the porta hepatis and the portal vein. 

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Case information

rID: 51398
Case created: 14th Feb 2017
Last edited: 17th Mar 2017
Inclusion in quiz mode: Included

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