title: Pediatric renal cell carcinoma.
abstract: Renal cell carcinoma (RCC) comprises about 5% of pediatric renal neoplasms. It has been recognized as a second malignancy in multiple reports. It is generally symptomatic at diagnosis, and most children with RCC present with more locally advanced disease than do adults. Contemporary investigation of pediatric RCC has demonstrated that a large percentage of these tumors bear cytogenetic translocations involving the MiT family of transcription factors. Surgical therapy for these children resembles operative intervention for adult RCC, though debate continues about the precise role of lymph node dissection. There are no adequately powered studies to support conclusions about adjuvant or neoadjuvant chemotherapy for children with RCC. This may be ameliorated by a multi-institutional protocol which is enrolling patients.
authors.affiliation: James Buchanan Brady Urological Institute, The Johns Hopkins Hospital, 600 N. Wolfe St., Baltimore, MD 21287, USA. jsausville@smail.umaryland.edu
authors.forename: Justin E
authors.initials: JE
authors.lastname: Sausville
authors.forename: David J
authors.initials: DJ
authors.lastname: Hernandez
authors.forename: Pedram
authors.initials: P
authors.lastname: Argani
authors.forename: John P
authors.initials: JP
authors.lastname: Gearhart
mesh_terms: D002292:Carcinoma, Renal Cell / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D002648:Child; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D016609:Neoplasms, Second Primary / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D014178:Translocation, Genetic