Sharma Sheetal, Shah Jigna S, Bali Harleen
Department of OMR, AIIMS, New Delhi, India.
Department of OMR, GDCH, Ahmedabad, Gujarat, India.
J Oral Maxillofac Pathol. 2020 Feb;24(Suppl 1):S86-S90. doi: 10.4103/jomfp.JOMFP_9_20. Epub 2020 Feb 28.
Malignant peripheral nerve sheath tumor (MPNST) is also termed as spindle cell malignancy of the peripheral nerve Schwann cell. It is a rare and highly aggressive, soft-tissue sarcoma of ectomesenchymal origin that accounts for 10% of all sarcomas and only 10%-12% of all lesions occur in the head-and-neck region, thus making it a rare entity. It arises or from the preexisting benign neurofibroma. The diagnosis of MPNST is one of the most elusive among the soft-tissue tumors because of its greater variability in overall presentation both clinically and histologically. This difficulty can be overcome by the use of immunohistochemistry. This article presents a rare case of MPNST of the oral cavity in a 40-year-old female patient with a brief review of the current literature.
恶性外周神经鞘膜瘤（MPNST）也被称为外周神经施万细胞的梭形细胞恶性肿瘤。它是一种罕见且具有高度侵袭性的、起源于外间充质的软组织肉瘤，占所有肉瘤的10%，且仅有10%-12%的病变发生在头颈部区域，因此它是一种罕见的疾病。它起源于先前存在的良性神经纤维瘤。MPNST的诊断是软组织肿瘤中最难捉摸的诊断之一，因为其在临床和组织学上的整体表现具有较大的变异性。使用免疫组织化学可以克服这一困难。本文介绍了一名40岁女性患者口腔MPNST的罕见病例，并对当前文献进行简要综述。
