摘要：

目的： 探讨头颈部色素性神经鞘瘤（melanotic schwannoma, MS）的临床表现、鉴别诊断及预后，以提高对该疾病的认识及治疗水平。方法： 徐州市中心医院口腔科2019年5月收治了1例头颈部MS患者，总结其临床表现、影像学资料及病理资料，并分析国内外相关文献。结果： 患者因发现右颈部肿物10 d入院就诊。颈部CTA示：病灶周围血管及软组织受压、推移，神经源性肿瘤可能。外科手术切除。免疫组织化学结果：HMB45(+)、 KI67(10%, +)、CD34(+)、S100(+)。病理诊断：（右颈部）色素性神经鞘瘤。结论： 色素性神经鞘瘤来自神经嵴具有双向分化潜能的细胞，鉴于其潜在恶性，患者术后须长期随访。

关键词: 色素性神经鞘瘤, 免疫组织化学, 超微结构, 鉴别诊断, 预后

Abstract:

Objective: Through reporting the clinical features, the differential diagnosis and prognosis of a case of melanotic schwannoma (MS) of the head and neck to improve understanding and treatment of this diseases. Methods: A case of head and neck MS in the Department of Stomatology, Xuzhou Municipal Central Hospital, was admitted in May 2019. The clinical manifestations, imaging findings, and pathological data were summarized, and relevant domestic and foreign literatures were analyzed. Results: The patient complained of a mass in the right neck for the past ten days. The CTA of the neck showed that the blood vessels and soft tissues around the lesion were compressed and pushed forward, and the lesion may be neurogenic origin. It was removed by surgery. Immunohistochemistry examination: HMB45(+), KI67(10%, +), CD34 (+), S100(+). Pathological diagnosis: This lesion (right neck) was consistent with MS. Conclusion: MS is derived from neural crest cells with bidirectional differentiation potential. Considering its potential malignancy, the patient needs long-term follow-up after surgery.

Key words: melanotic schwannoma, immunohistochemistry, ultrastructure, differential diagnosis, prognosis