Stone M M, Beaver B L, Sun C C, Hill J L
Department of Surgery, University of Maryland, Baltimore.
J Pediatr Surg. 1990 Sep;25(9):933-7; discussion 937-8. doi: 10.1016/0022-3468(90)90233-y.
Nephroblastomatosis (NB), a persistence of abnormal embryonal renal tissue beyond 36 weeks' gestation, is often associated with Wilms' tumor. The exact relationship of NB to the development of Wilms' tumor is unclear. Four cases are presented that elucidate the entire morphological spectrum of this disease. Analyses of these cases suggest these conclusions: (1) the NB complex is a spectrum of lesions from benign multifocal nodular renal blastema, resembling residual nephrogenic zones of immature fetal kidney, to Wilms' tumor; (2) infantile NB is a premalignant variant of Wilms' tumor with a favorable outcome usually, when treated early; (3) neonatal nephromegaly requires a complete evaluation and follow-up imaging; persistence mandates biopsy; (4) "second-look" laparotomy is unnecessary with state-of-the-art imaging; and (5) standardization of terminology is essential.
肾母细胞瘤病（NB）是指妊娠36周后异常胚胎性肾组织持续存在的情况，常与肾母细胞瘤相关。NB与肾母细胞瘤发生发展的确切关系尚不清楚。本文报告了4例病例，阐明了该疾病的整个形态学谱。对这些病例的分析得出了以下结论：（1）NB复合体是一系列病变，从良性多灶性结节性肾胚芽，类似于未成熟胎儿肾脏的残余生肾区，到肾母细胞瘤；（2）婴儿期NB是肾母细胞瘤的一种癌前变异型，通常早期治疗预后良好；（3）新生儿肾肿大需要进行全面评估和随访影像学检查；持续存在则需活检；（4）采用先进的影像学检查，无需进行“二次探查”剖腹手术；（5）术语标准化至关重要。
