A 41 year-old female presented to her primary care physician with back pain. Abdominal CT revealed a heterogeneous cystic mass in the body of the pancreas measuring 5.8 x 5.3 x 4.9 cm. Further evaluation with endoscopic ultrasound (EUS) revealed a 5cm multiseptated grossly solid cystic lesion consistent with a pseudopapillary neoplasm. After color doppler was used to rule out any overlying vessels, fine needle aspiration (FNA) was performed in the solid portion. FNA cytology aspirate was composed of small clusters of bland, ovoid eithelioid cells. The cell block showed small clusters of epithelioid cells that stained immunohistochemically for beta catenin and vimentin and were negative for synaptophysin and chromogranin, consistent with a pseudopapillary neoplasm.
Distal pancreatectomy was performed and surgical pathology demonstrated a solid and cystic pancreatic mass composed of moderate-sized cells showing solid and papillary growth patterns. The individual tumor cells had moderate amounts of eosinophilic cytoplasm, round nuclei and variable nucleoli.
Solid pseudopapillary tumors (SPT) are rare exocrine pancreatic neoplasms which comprise 1-2% of all pancreatic tumors. They are more common among young women and can grow rapidly leading to abdominal pain and obstructive symptoms due to mass effect. These lesions are now more often incidentally found on cross-sectional abdominal imaging for other indications (1). Endoscopic ultrasound may show a solid or a mixed solid and cystic lesion (2,3). Immunohistochemical stains typically react to Vimentin and beta-catenin. Malignant pseudopapillary tumors occur in up to 15% of cases and metastasis can occur with the liver being the most common metastatic site. Therefore, SPT should be surgically resected soon after diagnosis as complete surgical resection is curative(4).
Our patient did well post-operatively and had no evidence of metastatic disease. She is therefore considered cured from her solid pseudopapillary tumor.
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3. Mergener K, Detweiler SE, Traverso LW. Solid pseudopapillary tumor of the pancreas: Diagnosis by EUS-guided fine needle aspiration. Endoscopy 2003;35:1083–4.
4. Alexandrescu DT, O'Boyle K, Feliz A, et al. Metastatic solid-pseudopapillary tumour of the pancreas: Clinicobiological
correlates and management. Clin Oncol (R Coll Radiol) 2005;17:358–63.
David Arner, MD, University of Virginia
Bryan Sauer, MD, University of Virginia
Vanessa Shami, MD, University of Virginia