小儿泌尿生殖系及盆腔横纹肌肉瘤

Rhabdomyosarcoma Involving the Genitourinary Organs,Retroperitoneum Pelvis and Perineal Regions

横纹肌肉瘤是小儿软组织肉瘤中最多见之一,除多位于头颈部外,也好发于泌尿生殖系.自1958年应用手术、放及化疗的综合措施后,该病的预后大为改观,故将我科收治的泌尿生殖系及其相邻近的盆腔及会阴部横纹肌肉瘤作一报道及讨论,以期总结,提高疗效.

This article presents 17 patients with rhabdomyosarcoma of the genitourinary organs,retroperitoneum-pelvis and perineal regions.In 3 patients with bladder or prostatic lesions,surgical treatment were refused by their parents.The remaining 14 patients were treated with combined therapy.Five of 6 patients with bladder lesions stayed free from tumor for 3-10 years(mean 5.2 years)after diagnosis,the other patient died 4 months post operatively.Two patients with paratesticular lesions were free from disease 5 and 3 years respectively following diagnosis.Of the 2 patients with perineal lesions,one died 21 months after diagnosis,probably due to metastasis,while the other with local recurrence was reoperated upon and followed up for 2 years without evidence of neo-recurrence.The worst results were seen in 4 patients with retrope ritoneal pelvic lesion;one died 3 months after gross resection of the tumor,and the other 4 got deteriorated 4 months after diagnosis.Overall, among 9 operated Group-I patients, 8 with either bladder, paratestieular or peri heal lesions were free from disease on follow up of 3-10 years.The biological behaviour of embryonal rhabdomyosarcoma,the sex and age distribution, and the treatment were discussed in the text.