成人肾恶性横纹肌样瘤6例分析

Adult malignant rhabdoid tumors of kidney (Report of 6 cases)

目的:探讨成人恶性肾横纹肌样瘤的临床表现、病理特点和诊治方法。方法:对6例成人恶性肾横纹肌样瘤进行临床分析及文献复习,从临床表现、病理特点和诊治方法等方面进行总结。临床表现为腰痛2例,无痛全程肉眼血尿2例,查体发现2例;B超、CT及IVU等影像学检查均提示肾脏占位性病变,直径2.8～20.4cm。CT发现肿瘤内有坏死液化及不均匀强化影像。4例经腰行根治性肾切除术,1例经腹行左肾根治性切除术,1例经胸腹联合行左肾根治性切除术。结果:6例术后病理报告均为肾恶性横纹肌样瘤,其中1例并发透明细胞癌。术后均给予生物免疫治疗。1例术后2个月发现肺转移,1年后死于肿瘤肺转移;1例术后出现肺部感染,1年后死于心肺功能衰竭;3例术后半年无瘤生存,现在随访中;1例失访。结论:成人肾恶性横纹肌样瘤罕见,临床表现无特征性,CT表现为肿瘤内部坏死液化,轻度强化而且强化不均匀,最终确诊依赖于病理组织学、免疫组织化学和电镜检查结果。治疗方法包括开放手术或腹腔镜手术,并且辅助术后化疗或生物免疫治疗。

Objective：To evaluate the clinical presentations, pathologic characteristics, and the diagnosis and treatment of adult malignant rhabdoid tumor of kidney. Methods： Six cases of adult malignant rhabdoid tumor of kidney were analyzed, and the relevant literature was reviewed. Three cases were men, the others women, age range from 21 to 67 years old. Two cases complained of lumbago; two complained of gross painless hematuria; the rest tumors discovered by physical examination. Sonography, computed tomography and IVU revealed solid, cystic masses in kidneys that ranged in size from 2. 8 to 20. 4 cm. Results： Radical nephrectomies were performed on 6 patients. Postoperative pathology confirmed malignant rhabdoid tumor of kidney in all cases. 2 cases died one year after operation, 1 case lost follow up and 3 still survive now. Conclusions：Malignant rhabdoid tumor of kidney has aggressive behavior and poor prognosis and is extremely rare in adults. It does not have characteristic clinical manifestation and usually identified by immunohistochemical, pathologic and electron microscopic techniques. Treatment contains open operation or laparoscopic operation, respond well to aggressive chemotherapy regimens and immunotherapy, survival tends to be short.