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目的探讨初潮前妇科恶性肿瘤的临床特征、治疗及预后有关问题。方法对北京协和医院1985年11月至2008年7月间收治的初潮前妇科恶性肿瘤患者55例的临床资料进行回顾性分析。结果初潮前妇科恶性肿瘤患者55例,占同期妇科恶性肿瘤的0.9%(55/6330)。55例患者年龄10个月至14岁,其中≤5岁、～10岁、>10岁分别有7例(12.7%),24例(43.6%),24例(43.6%);卵巢肿瘤47例,阴道肿瘤6例,外阴肿瘤1例,盆腔胚胎型横纹肌肉瘤1例。在卵巢肿瘤中,内胚窦瘤和未成熟畸胎瘤各10例,混合性生殖细胞肿瘤和无性细胞瘤各7例,胚胎性癌和绒毛膜上皮癌各2例。在阴道肿瘤中,最常见的组织学类型是内胚窦瘤(3例)。外阴肿瘤为阴蒂Merkel细胞癌。47例卵巢肿瘤和盆腔胚胎型横纹肌肉瘤患者均进行了手术治疗,46例保留了生育功能,46例进行了化疗。阴道肿瘤患者在获取病理学诊断后,有5例进行了化疗。阴蒂肿瘤患者均进行了手术和化疗。在中位随诊52个月的随诊期内,生存率78.2%,复发率29.1%。结论初潮前妇科恶性肿瘤常发生在卵巢和阴道,对此年龄段的女性不应忽视妇科相关检查,治疗过程中应兼顾手术的彻底性和保留生育功能。
Objective To investigate the clinical features, treatment and prognosis of gynecologic malignancies before menarche. Methods The clinical data of 55 cases of gynecologic malignant tumors before the menarche admitted from November 1985 to July 2008 in Peking Union Medical College Hospital were analyzed retrospectively. Results 55 cases of gynecologic malignant tumors before menarche, accounting for 0.9% (55/6330) of gynecologic malignancies in the same period. 55 patients were from 10 months to 14 years old, including 7 cases (12.7%), 24 cases (43.6%), 24 cases (43.6%) ≤5 years old, , 6 cases of vaginal tumors, 1 case of vulvar tumors, 1 case of pelvic embryonal rhabdomyosarcoma. In ovarian tumors, 10 cases of endodermal sinus tumor and immature teratoma, 7 cases of mixed germ cell tumors and dysgerminoma, 2 cases of embryonal carcinoma and choriocarcinoma each. In vaginal tumors, the most common histological type is endodermal sinus tumor (3 cases). Vulvar tumors are clitoral Merkel cell carcinoma. Forty-seven patients with ovarian tumors and pelvic embryonal rhabdomyosarcoma were treated surgically, 46 with reproductive function retained, and 46 with chemotherapy. Vaginal cancer patients in access to pathological diagnosis, 5 patients were chemotherapy. Patients with clitoral tumors have undergone surgery and chemotherapy. During the median follow-up of 52 months, the survival rate was 78.2% and the recurrence rate was 29.1%. Conclusions Gynecological malignancies often occur in the ovary and vagina before menarche. Women in this age group should not neglect the gynecological examination, and should consider the thoroughness of surgery and the function of preserving fertility in the course of treatment.