Olowu Wasiu Adekunle, Salako Abdulkadr Ayo, Adelusola Kayode Adebowale, Sowande Oludayo Adedapo, Adetiloye Victor Adebayo, Adefehinti Olufemi, Osasan Stephen Adebayo
Paediatric Nephrology and Hypertension Unit, Obafemi Awolowo University Teaching Hospitals Complex, PMB 5538, Ile-Ife, Osun State, Nigeria.
Urology Unit, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun State, Nigeria.
Clin Exp Nephrol. 2008 Apr;12(2):144-148. doi: 10.1007/s10157-007-0015-4. Epub 2008 Jan 5.
A case of nephrotic syndrome (NS) and acute renal failure (ARF) associated with embryonal rhabdomyosarcoma (RMS) in a 10-year-old boy is reported. Ultrasound revealed irregular, echogenic, circumferential urinary bladder base mass, bilateral hydroureter and hydronephrosis. Histopathology of percutaneous renal and urethrocystoscopic biopsy specimens, respectively, revealed focal segmental glomerulosclerosis (FSGS) and embryonal RMS. Tumour remission was induced with pulse doses of intravenous vincristine, cyclophosphamide, methotrexate and actinomycin D over a 15-month period. He has been followed-up for 28 months and has maintained a drug-free tumour and proteinuria remission for 1 year. While some malignancies have been reported in association with NS, its occurrence in association with RMS is quite exceptional. We conclude that RMS may be associated with FSGS and NS. Effective treatment of the RMS was associated with sustained remission of the nephrotic proteinuria.
报告了一名10岁男孩患肾母细胞瘤（RMS）合并肾病综合征（NS）和急性肾衰竭（ARF）的病例。超声检查显示膀胱底部有不规则、回声增强的环形肿块，双侧输尿管积水和肾积水。经皮肾活检和尿道膀胱镜活检标本的组织病理学检查分别显示局灶节段性肾小球硬化（FSGS）和胚胎性RMS。在15个月的时间里，通过静脉注射大剂量长春新碱、环磷酰胺、甲氨蝶呤和放线菌素D诱导肿瘤缓解。他已接受随访28个月，无瘤且蛋白尿缓解已维持1年。虽然已有一些恶性肿瘤与NS相关的报道，但其与RMS相关的情况相当罕见。我们得出结论，RMS可能与FSGS和NS有关。RMS的有效治疗与肾病性蛋白尿的持续缓解相关。
