Luzar Boštjan, Falconieri Giovanni
Institute of Pathology, Medical Faculty University of Ljubljana, Korytkova 2, Ljubljana 1000, Slovenia.
Department of Pathology, University of Trieste School of Medicine, Strada di Fiume 449, Trieste 34149, Italy.
Surg Pathol Clin. 2017 Jun;10(2):337-343. doi: 10.1016/j.path.2017.01.008. Epub 2017 Mar 14.
Cutaneous malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas of neuroectodermal origin arising in the dermis and/or subcutis. In contrast with their deep soft tissue and visceral counterparts, cutaneous MPNSTs are rarely associated with neurofibromatosis type 1. Two main subtypes of cutaneous MPNST can be distinguished histologically: conventional (ie, spindle cell) and epithelioid MPNST. The 2 subtypes also differ in predilection for deep versus superficial locations, association with preexistent benign peripheral nerve sheath tumors and S100 immunohistochemistry. Herein, we review current knowledge of cutaneous MPNST and discuss its differential diagnosis.
皮肤恶性外周神经鞘瘤（MPNST）是起源于神经外胚层的罕见肉瘤，发生于真皮和/或皮下组织。与深部软组织和内脏的同类肿瘤不同，皮肤MPNST很少与1型神经纤维瘤病相关。皮肤MPNST在组织学上可分为两种主要亚型：传统型（即梭形细胞型）和上皮样MPNST。这两种亚型在对深部与浅表部位的偏好、与先前存在的良性外周神经鞘瘤的关联以及S100免疫组化方面也存在差异。在此，我们综述了皮肤MPNST的现有知识并讨论其鉴别诊断。
