Khoury Joseph D
Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
Clin Lab Med. 2005 Jun;25(2):341-61, vi-vii. doi: 10.1016/j.cll.2005.01.010.
Nephroblastoma, or Wilms tumor, is a malignant embryonal neoplasm that is derived from nephrogenic blastemal cells, with variable recapitulation of renal embryogenesis. The pathogenesis of nephroblastoma is complex and has been linked to alterations of several genomic loci, including WT1, WT2, FWT1, and FWT2. Generally, nephroblastoma is composed of variable proportions of blastema, epithelium, and stroma, each of which may exhibit a wide spectrum of morphologic variations. Distinguishing nephroblastoma with favorable histology from tumors that exhibit anaplasia is an integral component of histologic assessment because of its prognostic and therapeutic implications. Nephrogenic rests and a special variant of nephroblastoma, cystic partially differentiated nephroblastoma, also are discussed.
肾母细胞瘤，又称威尔姆斯瘤，是一种恶性胚胎性肿瘤，起源于肾母细胞，可不同程度地重现肾胚胎发育过程。肾母细胞瘤的发病机制复杂，与多个基因组位点的改变有关，包括WT1、WT2、FWT1和FWT2。一般来说，肾母细胞瘤由不同比例的胚基、上皮和间质组成，每种成分都可能表现出广泛的形态学变异。由于其预后和治疗意义，将组织学良好的肾母细胞瘤与间变肿瘤区分开来是组织学评估的一个重要组成部分。还讨论了肾源性残留和肾母细胞瘤的一种特殊变体，即囊性部分分化型肾母细胞瘤。
