Hsueh C, Hsueh W, Gonzalez-Crussi F
Department of Pathology, Loyola University Medical School, Maywood, Illinois 60153.
Pediatr Pathol. 1987;7(4):437-46. doi: 10.3109/15513818709161405.
We report a case of bilateral renal dysplasia with "cystic nephroblastomatosis" in a 29-week-old white female infant. The patient presented with bulging flanks, anuria, and respiratory distress. She survived for 1 1/2 days. Both kidneys were markedly enlarged by a diffuse cystic malformation. Microscopically, there was no demarcation between cortex and medulla. Numerous cysts and partially differentiated metanephric blastema cells were seen throughout the cortex and medulla. In addition, dysplastic tissues including cartilage occurred in the medulla. No evidence of Wilm's tumor was found. To our knowledge, this unique form of renal pathology has not been previously reported.
我们报告一例29周龄白人女婴双侧肾发育不良合并“囊性肾母细胞瘤病”的病例。该患者表现为侧腹膨隆、无尿和呼吸窘迫。她存活了1.5天。双侧肾脏因弥漫性囊性畸形而明显增大。显微镜下，皮质和髓质之间无分界。在整个皮质和髓质中可见大量囊肿和部分分化的后肾胚基细胞。此外，髓质中出现了包括软骨在内的发育异常组织。未发现威尔姆斯瘤的证据。据我们所知，这种独特形式的肾脏病理学此前尚未见报道。
