初潮前妇科恶性肿瘤55例分析

Premenarchal gynecologic malignant tumors:a review of 55 cases.

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。

Objectlve To evaluate the clinical characteristics, treatment and prognosis of premeuarcha/ gynecologic malignant tumors. Methods Patients admitted between November 1985 and July 2008 with premenarchal gynecologic malignant tumors were retrospectively analyzed. Results There were 55 cases of premenarchal gynecologic malignant tumors, which was 0. 9% of the total gynecologic malignant tumors at the corresponding period. Age range was 10 months to 14 years old there were 7 cases （ 12. 7% ） at the age of less than 5 years old, 24 cases （43. 6% ） at the age from 5 to 10 years old and 24 cases （43.6%） at the age more than 10 years old, respectively. There were ovarian（47） , vaginal（6） , vulvar tumors（ 1 ）, and pelvic embryonal rhabdomyosarcoma（ 1 ）. In the ovarian tumors, there were 10 cases of endodermal sinus tumors, 10 cases of immature teratomas, 7 cases of mixed germ cell tumor, 7 cases of disgerminoma, 2 cases of embryonal carcinoma , 2 cases of trophoblastoma. The endodermal sinus tumor（ 3 cases）was the most common type in vaginal tumors. The case of vulvar tumor was Merkel cell carcinoma of clitoris. The 47 cases of ovarian tumors and pelvic embryonal rhabdomyosarcoma all received surgery and 46 cases preserved fertility, 46 cases received chemotherapy. Five cases of vaginal tumors with the pathological diagnosis received chemotherapy. The case of vulvar tumor received both surgery and chemotherapy. Survival rate was 78. 2% and recurrence rate was 29. 1% during the follow-up period （mean 52 months）. Conclusion The common premenarchal gynecologic malignant tumors were ovarian and vaginal tumors, gynecologic examinations should not be neglected. Both operation completeness and fertility preservation should be consideyed.