6例女性生殖道无色素性黑色素瘤临床分析

Primary Malignant Amelanotic Melanoma in the Female Genital Tract: a Report of 6 Cases

  • 摘要: 目的: 探讨原发性女性生殖道无色素性黑色素瘤(Amelanotic melanoma in female genital tract,AMFGT)临床病理特点、诊断、治疗及预后。 方法: 对中国医学科学院肿瘤医院1991年1月至2007年12月间收治的6例AMFGT病例资料进行回顾性分析。 结果: 6例AMFGT中原发于外阴、阴道、宫颈各2例。患者发病时年龄在28~67岁之间,中位年龄52岁;4例为绝经后女性。按照国际妇产科联盟(FIGO)分期为Ⅰa~Ⅱa期。4例患者术前曾分别被误诊为绒毛膜癌、肉瘤、腺癌和淋巴瘤,其中2名患者经免疫组织化学染色检测S-100蛋白及抗黑色素瘤特异性抗体(HMB-45),结果阳性而更正诊断为本病。所有病例术后病理检查S-100蛋白及波状蛋白(Vimentin)均为阳性。5例检测HMB-45中3例阳性。本组治疗后随诊8~30个月(中位15.5个月),6例患者中4例分别在初次诊断后的6、6、12、19个月复发。其中3例于初次诊断后的13、18、19个月死于本病,l例带瘤生存。另2例治疗后分别生存8个月和30个月,无复发迹象。 结论: AMFGT由于缺少色素沉着易被误诊,采用正确的方法活检并结合S-100、Vimentin及HMB-45等免疫组织化学指标检测是确诊本病的关键。治疗上以手术切除作为主要治疗手段。术后的辅助治疗包括化疗、放疗和生物治疗,总体预后差。避免对本病的误诊是治疗的前提。

     

    Abstract: Objective: To analyze the clinical characteristics, pathological diagnosis, treatment and prognosis of amelanotic melanoma in the female genital tract (AMFGT). Methods: The medical records of 6 patients with AMFGT seen in our hospital be-tween 1991 and 2006 were reviewed. Results: Of these 6 patients, four were initially misdiagnosed with chorioepithelioma, sarcoma, adenocarcinoma and lymphoma, respectively.Two of these 4 patients were determined to have AMFGT preopera-tively after immunohistochemical detection for S-100 protein and HMB-45.Specimens removed from all 6 cases were an-alyzed after immunohistochemical staining and Hematoxylin and Eosin staining.Expression of S-100 and Vimentin were observed in all patients.HMB-45 was positive in 3 patients.Two patients suffered relapse at 6 months, one suffered re-lapse at 12 months, and one suffered relapse at 19 months after the primary treatment.One patient died 13 months after the initial diagnosis, one died 18 months after the initial diagnosis, and one died 19 months after the initial diagnosis. Conclusion: The fact that AMFGT lacks pigmentation makes diagnosis very difficult.S-100 protein, HMB-45 and Vi-mentin are important immunohistochemical markers that should be used in the evaluation of AMFGT.

     

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