中枢神经系统恶性黑色素瘤的诊断与治疗

DIAGNOSIS AND TREATMENT OF MALIGNANT MELANOMA IN CENTRAL NERVOUS SYSTEM

目的探讨中枢神经系统恶性黑色素瘤(malignant melanoma,MM)的临床特点,提高对此病的诊治水平。方法回顾性分析1996年9月-2007年4月收治的中枢神经系统MM患者7例。男6例,女1例;年龄18～74岁。5例位于幕上,1例位于脊髓,1例全脑转移。均行CT或MRI检查。病灶位于右额4例,右颞、左颞、左顶和C5～7各1例。6例行病灶切除术,1例行伽玛刀治疗。病理诊断2例为转移性中枢神经系统MM,5例为原发中枢神经系统MM。结果术后1例原发MM失访。6例获随访,随访时间2周～24个月,中位随访时间8个月。1例转移MM于术后2个月死亡,1例转移MM行伽玛刀治疗后2周死亡;1例原发MM术后2年死亡。3例原发MM分别于术后6、10、24个月健在,生活能自理。结论中枢神经系统MM临床症状和体征缺乏特异性,MRI诊断有一定帮助,但主要依靠病理诊断。建议采用手术为主的综合治疗方案。

Objective To investigate the clinical features ot malignant melanoma （MM） in the central nervous system （CNS） and to improve the diagnosis and treatment of this disease. Methods Seven MM-in-CNS patients＇ records between September 1996 and April 2007 were analyzed retrospectively, including 6 males and 1 female aged 18-74 years. The 5 cases were located in the supra-tentorial area, 1 in the spinal cord and 1 in the whole brain. CT or MRI scan was applied. The lesion was in the right frontal area in 4 cases, in the right temporal are in 1 case, in the left temporal area in 1 case, in the left apex area in 1 case and in the cervical spinal cord of C5～7 in 1 case. Six patients underwent neurosurgical operation and 1 patient received the Gamma Knife therapy. The pathological examination revealed that 2 cases were metastatic MM and 5 were primary. Results One patient with primary MM received no follow-up, and the rest 6 patients were followed up for 2 weeks to 2 years with the time of median 8 months. One patient with metastatic MM died 2 months after operation, 1 patient to with metastatic MM died 2 weeks after Gamma-Knife treatment, 1 patient with metastatic MM with primary MM died 2 years after operation, and 3 patients with primary MM were still alive and self-independent 6, 10 and 24 months after operation, respecti vely. Conclusion Since MM-in-CNS is short of specificity in clinical symptoms and signs, its diagnosis mainly relies on the pathological examination and is assisted by MRI. The combined therapy giving priority to operation is recommended.