摘要：

恶性外周神经鞘瘤（malignant peripheral nerve sheath tumor，MPNST）是来源于外周神经干或神经鞘分化的具有侵袭性的组织恶性肿瘤，临床上较少见。本文报告1例我科近期收治的口腔颌面颈部MPNST病例。查阅近年来国内外相关文献，讨论该疾病的生长方式、好发部位、临床表现、组织病理学特点、影像学表现，以及在治疗方面手术切除的彻底性及预后。MPNST作为具有较高恶性程度的肿瘤，其手术方式的设计、切除范围大小及术后是否进一步行放化疗等决定了该病的复发率。

关键词: 恶性外周神经鞘瘤, 异质性外周神经肿瘤, 口腔颌面颈部

Abstract:

Malignant peripheral nerve sheath tumor (MPNST) is a invasive malignant tumor originating from the differentiation of peripheral nerve trunks or nerve sheaths, which is extremely rare in clinical practice. This paper reports a case of MPNST of oral, maxillofacial and neck that recently admitted to our department. Combined with relevant literature reports at home and abroad in recent years, the growth pattern, prone site, clinical manifestations, histopathological features, imaging findings, as well as the thorougency of surgical resection in treatment and prognosis were discussed. As a kind of tumor with a high degree of malignancy, the recurrence rate of MPNST is determined by the design of surgical method, the size of resection, and whether to further radiotherapy and chemotherapy after surgery.

Key words: malignant peripheral nerve sheath tumor, heterogeneous peripheral nerve tumor, oral and maxillofacial and neck regions

中图分类号: 

• R782

• R739.8