姚玲女　孔繁斗

[摘要] 目的 探讨原发性外阴阴道恶性黑色素瘤的临床特点、治疗及预后。 方法 回顾性分析大连医科大学附属第一医院自1995年6月～2010年2月期间收治的16例外阴阴道恶黑的临床资料。 结果 本组16例病例中，外阴恶黑12例（75.00%），阴道恶黑4例（25.00%）。手术率81.00%，手术方式以外阴广泛切除术和局部扩大切除术为主，8例患者应用了辅助治疗方法（免疫治疗、放疗、化疗）。随访时间1～98个月，失访2例，随访率87.50%，随访期内因复发或转移死亡9例，死亡率56.70%。外阴恶黑患者平均生存期37.10个月，3年生存率（除外失访和生存不足3年患者）为55.50%，仅3例患者现无瘤生存；阴道恶黑患者中1例生存期为17个月，2例现带瘤平均生存34.00个月，1例失访。 结论 原发性外阴阴道恶黑恶性程度高，治疗应以手术为主，辅以免疫、放化疗，早期诊断、合理分期、早期治疗、定期随诊是改善预后的关键因素。

[关键词] 恶性黑色素瘤；外阴；阴道；治疗；预后

[中图分类号] R737.3 [文献标识码] A [文章编号] 2095-0616（2014）10-10-05

[Abstract] Objective To investigate the clinical characteristics， treatment and the prognostic factors in patients with the primary malignant melanoma of vulva and vagina. Methods The clinical data of 16 patients with primary malignant melanoma of vulva and vagina， admitted to the first affiliated hospital of Dalian Medical University from 1995.6 to 2010.2 were analyzed retrospectively. Results Of the 16 cases， there were 12 cases of primary malignant melanoma of vulva（75%）， 4 cases of primary malignant melanoma of vagina（25%）.The rate of surgery was 81%， radical vulvectomy and extended resection were the main forms of the operations， and 8 cases was received the secondary treatment methods （immunotherapy， radiotherapy， chemotherapy）. The flow-up time was from 1 to 98 months， 2 cases were out of flow-up， and the flow-up rate was 87.5%.During the flow-up time， 9 patients died of recrudescence or metastasis， and the death rate was 56.7%.The mean survival period of the patients with the primary malignant melanoma of the vulva was 37.1 months， and the survival rate in 3 years was 55.5%（not including the patients who were out of flow-up and whose flow-up time were less than three years）， and only 3 cases were alive without tumor. Among patients with the primary malignant melanoma of the vagina， the survival period of 1 patient was 17 months， 2 patients had been alive with tumor for 26-42monthes， the mean period was 34 months； and the other 1 patient was out of flow-up. Conclusion Malignant melanoma of the female genital tract is an high malignancy tumor， the therapy should be based on operation， supplemented by immunotherapy， radiotherapy， chemotherapy. Early diagnose， equitable staging， early therapy and flow-up in time are very important for improving prognosis.

[Key words] Malignant melanoma； Vulva； Vagina； Therapy； Prognosis

女性生殖器恶性黑色素瘤临床非常罕见，仅占所有恶黑色素瘤（以下简称恶黑）的1%～5%，多见于外阴、阴道，也可见于宫颈、子宫体、子宫内膜及卵巢，恶性程度高，易转移，预后差。为进一步了解女性生殖器恶黑的临床特征、治疗及预后，并为大样本的临床分析提供病例，笔者回顾性分析了16例外阴阴道恶黑病例资料，报道如下。endprint

1 资料与方法

1.1 一般资料

选择大连医科大学附属第一医院1995年6月～2010年2月期间收治的16例外阴阴道恶黑患者为研究对象，年龄33～86岁，中位年龄55岁，其中12例外阴恶黑（中位年龄为56岁），4例阴道恶黑（中位年龄为53岁）。……