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Revue Maghrebine de Pediatrie [La]. 2007; 17 (5): 241-248
en Francés | IMEMR | ID: emr-180591

RESUMEN

Bilateral Wilms tumor [WT] is uncommon malignancy, the incidence is about 5% of all nephroblastoma. It shows association with some congenital anomalies. there is no scheduled regimen for bilateral WT and their management offers a great challenge. To evaluate the clinical characteristics and the treatment result. 12 patients with bilateral WT diagnosed between 1997 and 2005, were retrospectively reviewed. Data collected: patients characteristics, chemotherapy, modality of surgical resection, renal function and survival. 11 patients [pts] had synchronous tumor and one metachronous lesions. The median age at primary diagnosis was 17 months [range 6m - 72m]. Congenital malformation were present in 4 children. 1 pt had hemi-hypertrophy, genitourinary horse kidney, 3 had hypertension.. All pts received preoperative prolonged chemotherapy [CT] for 25 to175 days [median 45 days] 7 received double agent chemotherapy. [VCR + Actinomycine], 2 triple agents: one [VCR + Actino + Adriblastine], the others [Etoposide, Carboplatine, Cyclophosphamied] for tumor progression. Two pt died before surgery due to infection. Bilateral partial nephrectomy 1 case total unilateral nephrectomy with partial nephrectomy on the other side in 3 cases. 9 pts are alive without disease and normal renal function 87 months after. The 10th in course of treatment after unilateral nephrectomy.Prolonged preoperative chemotherapy permit conservative surgical treatement of favorable histology bilateral Wilm's tumor and may improve the preservation of renal masses and function without impairing patient survival

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