膀胱恶性非上皮性肿瘤的诊断及治疗

Diagnosis and treatment of malignant bladder non-epithelial tumors

目的探讨恶性膀胱非上皮性肿瘤的临床诊断、治疗方法及与预后的关系。方法回顾性分析从1974-2007年17例恶性膀胱非上皮性肿瘤的临床资料及随访结果。结果17例患者中横纹肌肉瘤10例，恶性淋巴瘤2例，恶性嗜铬细胞瘤2例，平滑肌肉瘤2例，癌肉瘤1例。10例膀胱横纹肌肉瘤患者大多行手术、放疗及化疗的综合治疗，其中7例〈5岁，5例随访8年仍存活，2例失访；3例成人膀胱横纹肌肉瘤均于1年内出现全身多发转移死亡。2例膀胱恶性淋巴瘤均采用膀胱部分切除术，术后行放疗或加化疗，随访3年均存活。2例膀胱恶性嗜铬细胞瘤中1例行转移淋巴结切除术加化疗，随访5年存活；另1例因转移的淋巴结未能切除，术后行放疗，2年后死亡。2例膀胱平滑肌肉瘤只行单纯手术治疗，分别随访1、5年均无瘤存活。1例膀胱癌肉瘤术后行化疗，1年内死亡。结论恶性膀胱非上皮肿瘤以膀胱横纹肌肉瘤多见，幼儿多发，成人预后较幼儿差；其他类型肿瘤少见。不同的病理类型其治疗效果和预后不同。

Objective To study the clinical diagnosis, treatment, and prognosis of malignant bladder non-epithelial tumors. Methods The clinical data of 17 cases with malignant bladder non-epithelial tumor, 15 males and 3 females, aged 28 （3 - 73 ） , were analyzed. Results Ten of the 17 cases were diagnosed as with rhabdomyosarcoma, 2 with malignant lymphoma, and 2 with malignant pheochromocytoma, 2 with leiomyosarcoma, and 1 with carcinosarcoma. All patients underwent operation, or were treated with radiotherapy or chemotherapy. Among the 10 cases with rhabdomyosarcoma, 7 were aged under 5, 5 of them survived for more than 8 years, and 2 were lost to follow-up; other 3 adult patients died in one year with metastasis all over the body. Two patients with malignant lymphoma underwent partial cystectomy and adjuvant radiotherapy or chemotherapy, and survived for more than 3 years. Of the 2 cases with malignant pheochromocytoma, one underwent lymphadenectomy and adjuvant chemotherapy and survived for more than 5 years ; and another case died 2 years after operation and chemotherapy. Two patients with leiomyosarcoma underwent simple resection, one survived for more than 5 years ; and another survived 1 year after operation. One case with carcinosarcoma receiving operation followed by chemotherapy died in one year. Conclusion Malignant bladder non-epithelial tumors are rare clinically and most of them occur in children. Most of them are rhabdomyosarcoma. The prognosis of malignant bladder non-epithelial tumors, different in pathological types, is relatively worse in adults.