小儿生殖道横纹肌肉瘤3例并文献复习

Analysis of 3 cases of childhood genital tract rhabdomyosarcoma and literature review

目的探讨小儿生殖道横纹肌肉瘤的临床特征、治疗方法及预后情况。方法对我院1985年11月至2008年7月间收治的小儿生殖道横纹肌肉瘤(RMS)患者进行回顾性分析,并复习相关文献。结果小儿生殖道RMS患者3例,占同期小儿妇科恶性肿瘤的5.4%(3/56)。年龄1～5岁。阴道RMS 2例,盆腔RMS 1例;按国际横纹肌肉瘤协会(IRS)分期均为Ⅲ期。初期均进行保守性手术,并联合化疗,均达到完全缓解。2例阴道RMS分别随诊30、13个月无复发。1例盆腔RMS初期治疗停化疗1年复发,予复发病灶切除,并行联合化疗,完全缓解6个月,6个月后出现肺转移,放弃治疗后死亡。结论生殖道是小儿RMS的常见部位,阴道RMS最为常见,强调综合治疗,保守性手术是可行的。预后取决于肿瘤的组织学亚型、大小、病变范围、初始部位、治疗后残留病灶情况及年龄。

Objective： To investigate the clinical characteristics, treatment and prognosis of childhood genital tract rhabdomyosarcoma （RMS）. Methods： The patients admitted from November 1985 to July 2008 with childhood RMS were analyzed retrospectively. The literatures associated with childhood RMS was reviewed. Results： Three cases of childhood RMS accounted for 5.4% （3/56） of the childhood gynecologic malignancy at the corresponding time period. The ages were from 1 to 5 years old. There were 2 cases of vaginal RMS and 1 case of pelvic RMS. All the 3 cases were in stage Ⅲ based on IRS staging, and all received conservative surgery and combined chemotherapy, and all obtained a complete remission. The two vaginal RMS had no recurrence during the follow-up period （30 and 13 months, respectively）. The pelvic RMS relapsed 1 year later, and the patient was treated with a second tumor excision and chemotherapy. After 6 months of complete remission the tumor relapsed again with lung metastasis. Finally, the patient died of the tumor. Conclusions. The genital tract is the common site of childhood RMS, and the vaginal RMS is the most common. The combined therapy is emphasized, and conservative surgery is possible. The prognosis depends on histological subtype, size, extent of disease at presentation, site of origin, residual disease after treatment and age of the patient.