Rare Tumors of the Urinary Tract

Abstract

Malignant renal tumors cover 6% of all childhood cancers (Pastore et al. 2006; Brok et al., Eur J Cancer. 68:179–195, 2016). Wilms tumor or nephroblastoma is largely the most common type of cancer in the kidney of children, accounting for more than 90% of primary renal tumors. Other tumors, like clear cell sarcoma of the kidney (CCSK), rhabdoid tumor of the kidney (RTK), renal cell carcinoma (RCC), renal medullary carcinoma, congenital mesoblastic nephroma (CMN), primitive neuroectodermal tumor of the kidney, adenoma of the kidney, and others, are much rarer. Although Wilms tumor will account for the vast majority of malignant pediatric renal masses, the differential diagnosis with other rare or very rare tumors is extensive, encompassing a spectrum of both benign and highly malignant diseases as their treatment and prognosis is quite different (Vujanić et al., Nat Rev Urol. 15:693–701, 2018; Irtan et al., Eur J Cancer. 116:13–20, 2019).KeywordsRenal cell carcinomaRenal tumorTransitional cell carcinomaRhabdoid tumor