title: Intraneural malignant perineurioma: a case report and review of literature.
abstract: The great majority of malignant peripheral nerve sheath tumors (MPNSTs) exhibit Schwann differentiation. Few MPNSTs with perineurial differentiation are also named malignant perineuriomas. Benign perineurioms were classified as intraneural, extraneural (soft tissue), sclerosing, and reticular variant. Histopathological features of intraneural perineurioma are individual nerve axons surrounded by whorls of spindle-shaped cells arranged in an onion bulb-like pattern. However, intraneural malignant perineurioma is uncommon, its characteristic histological features were not clearly described yet. Positive for epithelial membrane antigen (EMA), glut-1 and claudin-1, is characteristic of malignant perineurioma. Herein, we report an intraneural malignant perineurioma in median nerve of a 13-year-old girl. The clinicopathological features were summarized and the related literatures were reviewed.
authors.affiliation: Department of Pathology, 251 Hospital of PLA Zhangjiakou, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Huang
authors.affiliation: Department of Pathology, 251 Hospital of PLA Zhangjiakou, China.
authors.forename: Hongwei
authors.initials: H
authors.lastname: Li
authors.affiliation: Department of Pathology, 251 Hospital of PLA Zhangjiakou, China.
authors.forename: Zhengwen
authors.initials: Z
authors.lastname: Xiong
authors.affiliation: Department of Pathology, Chongqing Cancer Institute Chongqing, China.
authors.forename: Rui
authors.initials: R
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008475:Median Nerve / Q000473:pathology; D020423:Median Neuropathy / Q000473:pathology*; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D018317:Nerve Sheath Neoplasms / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor
keywords: Malignant perineurioma; median nerve