Mitchell Andrew, Scheithauer Bernd W, Doyon Josée, Berthiaume Marie-Josée, Isler Marc
Department of Anatomic Pathology and Cytology, Maisonneuve-Rosemont Hospital, Montreal, Quebec, Canada.
Clin Neuropathol. 2012 Nov-Dec;31(6):424-9. doi: 10.5414/NP300423.
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.
绝大多数恶性外周神经鞘瘤（MPNST）表现出施万细胞分化。近年来，基于其组织学、免疫组织化学和超微结构特征，已鉴定出一部分神经束膜MPNST（恶性神经束膜瘤）。上皮膜抗原（EMA）、葡萄糖转运蛋白1（glut-1）和闭合蛋白1（claudin-1）免疫阳性是其特征。此类肿瘤必须与良性神经束膜瘤以及多种具有EMA阳性特征的非典型或恶性软组织肿瘤相鉴别。在此，我们报告一例累及一名42岁女性臀部的神经束膜MPNST。发现有神经受累。总结了已报道病例的临床病理特征并讨论了关键的鉴别诊断。
