少女生殖道恶性肿瘤的治疗

目的 探讨少女生殖道恶性肿瘤的临床特点、治疗方法和疗效。方法 回顾性分析27例少女生殖道恶性肿瘤的临床资料。结果 患儿的年龄为2－12岁，平均6．5岁。卵巢恶性肿瘤23例，其中卵巢恶性生殖细胞肿瘤21例，占77．8％；性腺外恶性肿瘤4例，包括阴道内胚窦瘤2例，阴道和盆腔横纹肌肉瘤各1例。卵巢恶性肿瘤的临床表现主要为腹痛和腹部包块，分别为77．8％和74．1％；而阴道恶性肿瘤的临床表现，主要为阴道流血和阴道肿块。91．3％（21／23）的患儿仅行患侧附件切除术，保留了子宫和对侧卵巢。少女生殖道恶性肿瘤对化学治疗（化疗)十分敏感，术后根据高危因素不同，分别给予顺铂、长春新碱、博莱霉素（PVB）方案，顺铂、鬼臼素、博莱霉素（PEB）方案或长春新碱、更生霉素、环磷酰胺（VAC）方案化疗。在平均为47．4个月的随诊期内，生存率达71．4％。结论 卵巢恶性生殖细胞肿瘤对化疗十分敏感，应作为主要的治疗手段。手术的原则应保留生育功能。

Treatment of childhood genital malignancies

Objective To present the clinical profile and the result of treatment in childhood genital malignancies. Methods Twenty seven childhood patients (<12 years) with genital malignancies diagnosed and treated in Peking Union Medical College hospital were analyzed retrospectively focusing on the clinical characteristics and outcome of the treatment. Results Patients ranged in age from 2 to 12 years with mean 6 5 years at diagnosis. Of the 27 patients, 23 had ovarian malignancies including 21 (77 8%) germ cell tumor. Four of the patients had extragonadal tumors with 2 endodermal sinus tumor of the vagina, and 2 embryonic rhabdomysarcoma. In ovarian malignancies, abdominal mass and abdominal pain were main clinical features accounting for 77 8% and 74 1% respectively. In vaginal malignancies, vaginal bleeding and polypoid tumor of the vagina were important clinical manifestations. 91 3%(21/23) of the patients underwent only unilateral salpingo oophorectomy with preservation of contralateral ovary and the uterus. Childhood genital malignancies were very sensitive to the chemotherapy. Postoperative vincristine, actinomycine, cyclophosphamide (VAC) or cisplatine, vincristine, blyomycine (PVB) chemotherapy was individualized according to the tumor site, International Federation of Gynecology and obstetrics stage and histopathology. Survival rate in our series was 71 4% during the follow up time (mean 47 4 months). Conclusions Malignant germ cell tumor of the ovary is the most common tumor in the childhood genital malignancies. Principle of primary surgery is preservation of fertility and chemotherapy is also considered as a very important treatment modality.