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.
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