Joseph J M, Suter O C, Nenadov-Beck M, Gudinchet F, Frey P, Meagher-Villemure K
Department of Pediatric Surgery, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
J Pediatr Surg. 2003 Apr;38(4):E13. doi: 10.1053/jpsu.2003.50151.
Bilateral fetal rhabdomyomatous nephroblastoma is a rare variant of Wilms' Tumor. The authors report the evolution over 48 months of a 10-month-old baby with bilateral nephroblastoma for which a left nephrectomy was initially performed. A right kidney tumor was enucleated preserving the kidney. The transformation of the primary tumor into a completely differentiated cystic nephroblastoma or nephromalike tumor and the appearance of a metachronous lesion was seen. This report emphasizes the role of nephron-sparing surgery in bilateral Wilms' Tumor when a benign transformation occurs under chemotherapy.
双侧胎儿横纹肌瘤样肾母细胞瘤是肾母细胞瘤的一种罕见变异型。作者报告了一名10个月大双侧肾母细胞瘤婴儿在48个月内的病情演变，该婴儿最初接受了左肾切除术，右肾肿瘤行剜除术以保留肾脏。观察到原发肿瘤转变为完全分化的囊性肾母细胞瘤或肾样肿瘤，并出现了异时性病变。本报告强调了在化疗期间发生良性转变时，保留肾单位手术在双侧肾母细胞瘤治疗中的作用。
