Extrapancreatic Solid Pseudopapillary Neoplasm

Abstract

Solid pseudopapillary neoplasm, an uncommon, intriguing, low-grade malignant tumor of the pancreas, can exceptionally occur in extrapancreatic sites. We report 1 such case occurring as an ovarian primary in a 25-year-old woman, who presented with abdominal fullness. She remained well 12 years after resection of the 1.7-kg right ovarian tumor. Histologically, the tumor showed a solid, dehiscent, and pseudopapillary growth, traversed by fibrous septa and arborizing blood vessels. The polygonal tumor cells had round nuclei, fine chromatin, and lightly eosinophilic granular cytoplasm. In certain areas, eosinophilic hyaline globules were present. On immunostaining, the tumor cells were positive for β-catenin (nuclear translocation), CD10, CD56, and synaptophysin (focal), and were negative for cytokeratin, E-cadherin, and chromogranin. The morphologic and immunohistochemical features were compatible with those of solid pseudopapillary neoplasm. Review of the literature uncovered 10 cases of extrapancreatic solid pseudopapillary neoplasm with adequate data for analysis. They were seen to occur predominantly in young female patients, who presented with a large tumor mass, similar to their pancreatic counterparts. The most common sites of occurrence were the mesocolon (with ectopic pancreatic tissue) and ovary. Awareness of the existence of this tumor in various extrapancreatic sites is essential to avoid misdiagnosis.