关键词: 中枢神经系统, 恶性黑色素瘤, 临床分型, 诊断, 治疗

Abstract: Objective  To summarize the clinical, imaging, therapeutic and prognostic features of primary central nervous system (CNS) malignant melanoma(MM). Methods  The clinical data of 17 patients with primary CNS MM admitted to the  Department of Neurosurgery, Sanbo Brain Hospital, Capital Medical University, during 2011-2021 were divided into four clinical types for retrospective analysis. Results  The male to female ratio was 11∶6, and the mean age was (39.9±16.6) years. Among them, 6 cases were type A, 5 type B, 4 type C, and 2 type D. There were 11 cases of cranial hypertension, 9 cases of local nerve dysfunction and 3 cases of epilepsy. High T1 signal was observed in 12 cases on magnetic resonance imaging（MRI） scan. There were 13 cases had seeding metastasis, 11 of which were linear. The fourteen cases underwent surgical resection, 3 cases underwent biopsy, and 6 cases underwent anti-tumor therapy after surgery. Overall, the median overall survival (mOS) was 7 months, and the survival of patients with combined treatment was significantly longer than that with surgery alone ［(20.6±7.9) months  vs  (3.3±0.9) months, P=0.001］. There were significant differences in symptoms, imaging, and treatment among the 4  clinical types. Conclusion  The incidence of primary CNS malignant melanoma is low, and most of them have obvious imaging features, which can be divided into four clinical types. The disease requires surgery combined with radiotherapy and other comprehensive treatment, yet now the prognosis is stile poor.

Key words: central nervous system, malignant melanoma, clinical types, diagnosis, treatment