Sato Yasuyuki, Iizuka Jyunpei, Imai Kenji, Sawada Yugo, Komatsu Tomonori, Yago Rie, Kondo Tsunenori, Ishida Hideki, Tanabe Kazunari
Department of Urology, Tokyo Women's Medical University.
Nihon Hinyokika Gakkai Zasshi. 2010 Jul;101(5):683-8. doi: 10.5980/jpnjurol.101.683.
The patient was a 30-year-old man who had undergone living-donor kidney transplantation for renal failure caused by IgA nephropathy at age 29. On post-transplantation day 83, he visited our department with a chief complaint of asymptomatic hematuria. CT performed on post-transplantation day 95 revealed a tumor (size, 4 cm) in the right native kidney that had not been observed at the time of transplantation. CT performed on post-transplantation day 153 showed that the tumor had enlarged to 6 cm, while retrograde pyelogram performed on post-transplantation day 171 was negative for renal pelvic tumor. On post-transplantation day 193, radical right nephrectomy was performed. The tumor had directly invaded the diaphragm and the lower surface of the liver, and was histopathologically diagnosed as rhabdoid tumor of the kidney. As the pathological tissue was extremely malignant, hepatic posterior segmentectomy, right adrenalectomy, and lymph node dissection were further performed for metastases on post-transplantation day 200. On the 23rd day after radical right nephrectomy (post-transplantation day 216), the patient developed dyspnea. Chest CT showed pleural effusion, hemothorax in right lung and metastases in both lungs. The patient's general status gradually worsened thereafter, and he died on the 53rd day after radical right nephrectomy (post-transplantation day 246). Rhabdoid tumor of the kidney is a rare renal tumor that affects children, and only four adult cases have been reported to date. We report our experience with this rare case.
该患者为一名30岁男性,29岁时因IgA肾病导致肾衰竭接受了活体供肾肾移植。移植后第83天,他因无症状血尿为主诉前来我院就诊。移植后第95天进行的CT检查显示,右侧原肾有一个肿瘤(大小为4 cm),移植时未观察到。移植后第153天进行的CT检查显示肿瘤已增大至6 cm,而移植后第171天进行的逆行肾盂造影显示肾盂肿瘤为阴性。移植后第193天,进行了根治性右肾切除术。肿瘤已直接侵犯膈肌和肝脏下表面,组织病理学诊断为肾横纹肌样瘤。由于病理组织恶性程度极高,于移植后第200天进一步进行了肝后段切除术、右肾上腺切除术及淋巴结清扫术以治疗转移灶。在根治性右肾切除术后第23天(移植后第216天),患者出现呼吸困难。胸部CT显示有胸腔积液、右肺血胸及双肺转移。此后患者的一般状况逐渐恶化,于根治性右肾切除术后第53天(移植后第246天)死亡。肾横纹肌样瘤是一种罕见的儿童肾肿瘤,迄今为止仅报道过4例成人病例。我们报告了我们对这一罕见病例的诊治经验。