Kumar A P, Pratt C B, Coburn T P, Johnson W W
J Pediatr Surg. 1978 Jun;13(3):281-5. doi: 10.1016/s0022-3468(78)80401-1.
Nodular renal blastema and nephroblastomatosis were present in 8 of 118 patients (6.8%) with Wilms' tumor. Five of these 8 patients (63%) had bilateral Wilms' tumors. Two had hemihypertrophy. Preoperative renal angiograms were accurate in detecting these metanephric anomalies. The surgical approach consisted of removal of the most diseased kidney and biopsy for diffuse tumors and wedge resections for localized tumors for the remaining kidney. Postoperatively, radiation was administered when tumor extended outside the kidney. Chemotherapy consisted of vincristine and dactinomycin for 18 mo and adriamycin for 6 mo. This method of management resulted in tumor-free survival of these 8 patients for 1--44 mo (median 24 mo). Nodular renal blastema and nephroblastomatosis may possibly develop into Wilms' tumor. All of these three conditions respond to surgery, chemotherapy, and radiation. When a Wilms' tumor is encountered, it is better to explore and possibly biopsy the opposite kidney. There is a place for second-look laparotomy in this spectrum of congenital anomalies.
118例威尔姆斯瘤患者中有8例（6.8%）存在结节性肾胚基和肾母细胞瘤病。这8例患者中有5例（63%）患有双侧威尔姆斯瘤。2例有半身肥大。术前肾血管造影在检测这些后肾异常方面是准确的。手术方法包括切除病变最严重的肾脏，对弥漫性肿瘤进行活检，对剩余肾脏的局限性肿瘤进行楔形切除术。术后，当肿瘤延伸至肾脏外时给予放疗。化疗方案为长春新碱和放线菌素D治疗18个月，阿霉素治疗6个月。这种治疗方法使这8例患者无瘤生存1至44个月（中位时间24个月）。结节性肾胚基和肾母细胞瘤病可能会发展为威尔姆斯瘤。这三种情况均对手术、化疗和放疗有反应。当遇到威尔姆斯瘤时，最好探查对侧肾脏并可能进行活检。在这一系列先天性异常中，二次剖腹探查有其应用价值。
