Non-Wilms Pediatric Renal Tumors

Abstract

AbstractThe vast majority of childhood renal tumors are Wilms tumors (nephroblastoma). All other pediatric kidney tumors comprise less than 15 % of childhood renal tumors and are therefore considered rare malignancies of childhood. The most frequently observed non-Wilms renal tumors include clear cell sarcoma of the kidney (CCSK), renal cell carcinoma (RCC), malignant rhabdoid tumor of the kidney (MRTK), and congenital mesoblastic nephroma (CMN). These tumor types are markedly heterogeneous in their clinical characteristics. CMN occurs mainly in early infancy, MRTK occurs in infants and young toddlers, CCSK occurs in toddlers and young school-age children, and RCC is most common in adolescents. The outcome of CMN is excellent, with overall survival rates over 95 % without adjuvant chemotherapy. By contrast, patients with MRTK have overall survival rates of only 20–25 %, even with intensive multimodality treatment regimens. The outcomes of CCSK and RCC are intermediate between CMN and MRTK. The non-Wilms renal tumors also have markedly different biological characteristics, each bearing distinct genetic mutations and translocations. This chapter provides an overview of the major non-Wilms renal tumors of childhood, including the ultra-rare entities of renal medullary carcinoma, metanephric tumors, and renal sarcomas.KeywordsRenal TumorClear Cell Renal Cell CarcinomaRhabdoid TumorFumarate HydrataseClear Cell SarcomaThese keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.