Surgery in the treatment of nephroblastomatosis - a series of 19 patients

Autor: Christophe Bergeron, Hubert Ducou Lepointe, Gudrun Schleiermacher, Catherine Patte, Awatef Charieg, Judith Landman-Parker, M. Larroquet, Georges Audry, Liliane Boccon-Gibod
Rok vydání: 2007
Předmět:
Zdroj: Journal of Pediatric Urology. 3:S40
ISSN: 1477-5131
DOI: 10.1016/j.jpurol.2007.01.060
Popis: Purpose Nephroblastomatosis (NBis) is associated with a high risk of developing a Wilms tumor (WT). An homogeneous therapeutic approach was adopted combining chemotherapy (CT), and surgery only in case of WT, certain or suspected, on imaging features and possibly histology by transcutaneous needle biopsy, at diagnosis or during follow-up.This retrospective study evaluates indications and techniques of surgery in the evolution of NBis. Material and methods From 1979-oct.2006, 19 children were cared for in our surgical department,(median age :11 monthes; range 8m-5y). Nbis was bilateral in 17 cases. CT was given in all cases:Vincristine+Actinomycin, as maintenance treatment in case of isolated NBis, and in association with Doxorubicine for 4 to 8 weeks in case of WT. 19 patients were operated on, 10 on both kidneys and 9 on one. Operative procedure consisted in nephron sparing surgey (NSS) for 24 kidneys (83%) and a total nephrectomy in 5 .Surgical indication was decided either “on diagnosis” of a certain or suspected WT (24 kidneys), or later during follow-up for a mass suspect of WT(5 :1 mass stable during CT and 4 masses progressive or appearing after CT). Results No kidney was lost by ischemia. After histological review of the 29 specimens, 25 (86%) corresponded to WT in 17 children, and 4 to residual mass of NBis (3 operated “on diagnosis” and 1 for a progressive mass after CT). For the 25 WT, 5 were low risk tumor, 14 intermediate and 6 high. With a median follow-up of 5 years (4m-15y), recurrence occurred in 6 children (31%), 4 with high risk tumour and 2 with intermediate, all following NSS. Four patients died, among whom 3 who began their treatment before 1983. Mild renal insufficiency (1) and/or HT(2) were found in 2 patients. Conclusions Occurence of WT in NBis is very frequent in our surgical experience. In case of a high risk WT developing in NBis, local recurrences are frequent after NSS. Progressive or new masses after CT are strongly suspected of being a WT unlike masses that are stable during CT.
Databáze: OpenAIRE
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