Young Female With Pancreatic Mass

Question: A previously healthy, 22-year-old woman underwent computed tomography and magnetic resonance imaging of the abdomen to evaluate acute-onset, self-limited, lower abdominal pain. A 5.8-cm cystic mass with some solid components was seen in the head of the pancreas (Figure A). She had no history of pancreatitis or abdominal trauma and no family history of pancreatitis or malignancy. Her only medication use was oral contraceptive pills. The physical examination was unremarkable. Laboratory studies including pancreas enzymes and liver function tests were normal.

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Endoscopic ultrasound examination (EUS) revealed a heterogeneous, hypoechoic 5.3 × 4.5 cm mass that appeared predominantly solid, with multiple, small anechoic/cystic spaces (Figure B). The lesion was not obstructing the pancreatic or bile duct. The pancreas parenchyma in the body and tail was normal and no lymphadenopathy was seen.

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EUS-guided fine needle aspiration (FNA) was performed with a 22-gauge needle. Cytology identified a 3-layered papillary architecture composed of a central capillary layer, a middle layer of myxoid stroma and an outer layer of monomorphic neoplastic cells (Figure C, papanicolaou stain). Individual cells showed high nuclear/cytoplasmic ratio, scant cytoplasm, and grooved/clefted nuclei. Immunophenotype staining was positive for CD10, β-catenin, vimentin, neuron-specific enolase, progesterone receptor, and CD56, and negative for estrogen receptor and synaptophysin.

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What is the diagnosis?

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Answer to the Clinical Challenges and Images in GI Question: Image 3 (page 791): Solid Pseudopapillary Tumor of the Pancreas 

Solid pseudopapillary tumors (SPT) of the pancreas are rare, indolent, epithelial neoplasms, representing 1%–2% of all exocrine pancreatic tumors. These tumors occur predominantly in young women (mean age, 25–30 years), may be located anywhere in the pancreas, and rarely cause symptoms, hence are often found incidentally on abdominal imaging. The mean tumor size ranges from 4 to 10 cm at the time of presentation. Metastases are infrequent and at presentation 85% of tumors are limited to the pancreas.

EUS-FNA has a diagnostic accuracy of 75% in SPT.1 SPT has a highly characteristic cytomorphology, which includes a 3-layered papillary architecture with a fibrovascular core, a middle layer of myxoid stroma, and an outer layer of monomorphic neoplastic cells with rounded nuclei. Immunostains are very helpful in differentiating SPT from pancreatic neuroendocrine tumors. The expression of vimentin, CD10, and β-catenin is specific to SPT versus chromogranin and synaptophysin in neuroendocrine tumors.2

Complete surgical resection is generally curative. Overall 5-year survival is as high as 97% in patients undergoing surgical resection.3 There is limited experience regarding chemotherapy and radiotherapy with or without the presence of metastatic disease.