Hybrid Peripheral Nerve Sheath Tumors, Including a Malignant Variant in Type 1 Neurofibromatosis

作者：Kacerovska Denisa; Michal Michal; Kuroda Naoto; Tanaka Azusa; Sima Radek; Denisjuk Natalja; Kreuzberg Boris; Ricarova Renata; Kazakov Dmitry V^*

来源：American Journal of Dermatopathology, 2013, 35(6): 641-649.

DOI：10.1097/DAD.0b013e31827e2917

• neurofibromatosis type 1
• plexiform neurofibroma
• hybrid plexiform schwannoma-neurofibroma
• hybrid plexiform neurofibroma-perineurioma
• incipient

The authors report a small case series of hybrid nerve sheath tumors occurring in the setting of type 1 neurofibromatosis. Four lesions were benign and consisted of plexiform neurofibromas with considerable areas of perineuriomatous differentiation in patients with type 1 neurofibromatosis. In these lesions, biphasic (Schwannian and perineuriomatous) differentiation was apparent on immunohistochemistry, with the perineuriomatous areas staining for epithelial membrane antigen, glut-1, and claudin-1 and being negative for S-100 protein. Three patients were members of a single family, with a history of various malignant neoplasms. Included in the series is 1 hybrid lesion in which neurofibromatous and perineuriomatous areas were clearly visible on hematoxylin- and eosin-stained slides. The lesion was unique in that it manifested malignant change in the S-100 protein-positive component, which was classified as malignant peripheral nerve sheath tumor. The malignant component showed areas with an epithelioid cell morphology.

• 出版日期2013-8