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目的分析幼少女生殖系统恶性肿瘤的临床病理特点及诊治方法。方法回顾性收集2001年1月至2016年5月于首都医科大学附属北京妇产医院妇瘤科诊治的幼年及少年女性生殖系统恶性肿瘤患者病例资料。结果本组12例,年龄10个月至14岁,<5岁者占75%,≥5岁者占25%。病理以卵巢或阴道卵黄囊瘤(6例)和阴道横纹肌肉瘤(4例)为主,其余包括性腺母细胞瘤和幼年型颗粒细胞瘤各1例。生殖细胞肿瘤共占58.3%。患儿主要临床症状为阴道出血、排液,发现外阴肿物及盆腔肿物。治疗以肿物局部切除和化疗为主。卵黄囊瘤及幼年型颗粒细胞瘤患儿预后较好。横纹肌肉瘤预后极差,对化疗不敏感。结论幼少女生殖系统恶性肿瘤发生率低,应及早发现,手术切除肿瘤并辅助规范化化疗。
Objective To analyze the clinicopathological features and diagnosis and treatment of reproductive system malignant tumors in young girls. Methods The data of patients with reproductive system malignant tumors of juvenile and juvenile genitals from January 2001 to May 2016 in Beijing Obstetrics and Gynecology Hospital of Capital Medical University were retrospectively collected. Results The group of 12 patients, aged 10 months to 14 years old, <5 years old accounted for 75%, ≥ 5 years accounted for 25%. Pathology of ovarian or vaginal yolk sac tumor (6 cases) and vaginal rhabdomyosarcoma (4 cases), the rest including gonadoblastoma and juvenile granulosa cell tumor in 1 case. Germ cell tumors together accounted for 58.3%. The main clinical symptoms of children with vaginal bleeding, drainage, found vulvar tumor and pelvic mass. Local treatment of tumor removal and chemotherapy based. Yolk sac tumor and juvenile granulosa cell carcinoma of children with good prognosis. Very poor prognosis of rhabdomyosarcoma, chemotherapy is not sensitive. Conclusions The prevalence of malignant tumors in young girls is low and should be detected as soon as possible. The tumor should be surgically removed and the chemotherapy should be normalized.