Extrapancreatic solid pseudopapillary neoplasm: report of a case of primary ovarian origin and review of the literature.

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 ß-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.

Intraoral superficial angiomyxoma.

Superficial angiomyxoma is an unusual benign myxoid tumor of the trunk, head and neck, extremities, and genitalia. We report the third case of intraoral superficial angiomyxoma, which occurred within the buccal mucosa of a 37-year-old woman. The symptomless slow-growing mass was present for 2 years. The lobulated, paucicellular myxoid tumor showed prominent vascularity, stromal inflammation, including neutrophils, and immunopositivity for CD34, vimentin, and muscle-specific actin. Cure is effected by localized excision, with follow-up, owing to the high recurrence rate of the cutaneous tumors, which has not been substantiated for the few reported intraoral cases. Superficial angiomyxoma does indeed occur intraorally and should be included in the differential diagnosis of myxoid intraoral soft tissue neoplasms.