title: Malignant perineurioma (malignant peripheral nerve sheath tumor with perineural differentiation).
abstract: The great majority of malignant peripheral nerve sheath tumors (MPNST) exhibit Schwannian differentiation. In recent years, a subset of perineurial MPNST (malignant perineurioma) has been identified based on their histologic, immunohistochemical and ultrastructural features. Immunopositivity for epithelial membrane antigen (EMA), glut-1 and claudin-1, is characteristic. Such tumors must be distinguished from benign perineurioma and a variety of atypical or malignant soft tissue tumors featuring EMA positivity. Herein, we report a perineurial MPNST involving the buttock of a 42-year-old woman. Nerve involvement was noted. The clinicopathologic features of reported examples are ummarized and key differential diagnoses are discussed.
authors: Department of Anatomic Pathology and Cytology, Maisonneuve-Rosemont Hospital, Montreal, Quebec, Canada. plaines@mac.com
authors: Andrew
authors: A
authors: Mitchell
authors: Bernd W
authors: BW
authors: Scheithauer
authors: Josée
authors: J
authors: Doyon
authors: Marie-Josée
authors: MJ
authors: Berthiaume
authors: Marc
authors: M
authors: Isler
mesh_terms: D000328:Adult; D002081:Buttocks / Q000473:pathology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D009364:Neoplasm Recurrence, Local / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D018317:Nerve Sheath Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000628:therapy