摘要目的 分析椎管内原发性黑色素细胞瘤的临床特点,探讨其诊断及治疗方法.方法 回顾性分析2011年6月至2014年12月于北京天坛医院神经外科接受手术治疗的6例椎管内原发性黑色素细胞瘤患者的临床资料,对其临床表现、诊断、治疗、预后进行分析.结果 患者中男性5例,女性1例;年龄23～50岁,平均年龄39.8岁.6例患者中,4例主诉颈肩腰背痛、2例主诉四肢麻木无力.术前均进行MRI检查,仅1例诊断为原发性黑色素细胞瘤,其余均为术后病理证实.肿瘤全切除2例,近全切除2例,2例为多发或播散病例,仅行部分切除.术后随访时间9～52个月,平均25.7个月.1例患者术后9个月复发,16个月死亡;1例术后4年复发;其余4例均为初发且术后均未复发.2例复发患者均为术后未行放疗者,4例未复发患者中2例术后行放疗,所有患者均未化疗.结论 椎管内原发黑色素细胞瘤恶性程度较低,需依靠术后病理进行诊断.全切肿瘤术后不易复发、转移.治疗首选手术,提倡早期全切除,预后较好,部分切除患者采取适宜的放疗可延缓肿瘤复发.

abstractsObjective To investigate the clinical features,diagnostic and therapeutic strategy of primary intraspinal melanocytoma.Method The clinical data of primary intraspinal melanocytoma patients who underwent surgical operations from June 2011 to December 2014 in Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University were retrospectively reviewed.Results Six cases of intraspinal melanocytoma,identified with pathology were collected,including 5 male and 1 female patients with the average age of 39.8 years (range 23-50 years).Limbs anesthesia and weakness were present in 2 cases.Neck and back pain were present in 4 cases.All patients performed MRI examination before operation,and only 1 case was diagnosed as primary melanocytoma,and the others were confirmed by postoperative pathology.All cases were confirmed by the pathological examination.Total resection was achieved in 2 cases,subtotal resection was achieved in 2 cases,2 disseminated cases only achieved partial resection.The average period of postoperative follow-up process was 25.7 months (range 9-52 months).One case recurred in 9 months after surgery and died in 16 months,one case recurred in 1 year after surgery,and the other 4 cases had no recurrence.The recurrenced 2 patients had been not treated with radiotherapy,2 patients of the 4 that not recurrenced had received radiotherapy.All patients didn't receive chemotherapy.The other patients were in good condition after surgery.Conclusions The intraspinal primary melanocytoma is less likely to recurrence and metastasis if total resection is achieved.Diagnosis relies on pathology.Surgery is the first choice and early total resection is strongly recommend.Appropriate radiotherapy for partial resection cases can delay tumor recurrence.