Cancer2013-09-05 2:57 AM

Synchronous bilateral Wilms tumor - Indolfi - 2013 - Cancer - Wiley Online Library

Abstract BACKGROUND: The optimal management of bilateral Wilms tumor (BWT) is challenging, and their survival is lower than for unilateral tumors. This report discusses a large series of BWTs treated in Italy in the last 2 decades. METHODS: This analysis concerns patients with synchronous BWT registered at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers between 1990 and 2011; details on their treatment and outcome are presented and discussed. RESULTS: Ninety BWTs were registered in the AIEOP Wilms tumor database. Preoperative chemotherapy was given for a median 12 weeks before definitive tumor resection was attempted. Forty-eight percent of the patients had preservation of bilateral renal parenchyma. The proportion of bilateral nephron-sparing surgeries was not higher in the 37 patients initially given doxorubicin/vincristine/actinomycin D (32%) than in the 43 children receiving vincristine/actinomycin D alone (58%). The 4-year disease-free survival rate was 66.5% ± 5% and overall survival was 80% ± 5% for the cohort as a whole. The 4-year disease-free survival (overall survival) for 18 children with diffuse anaplasia or postchemotherapy blastemal-type tumors was 51% ± 13% (62% ± 13%), as opposed to 72% ± 3% (88% ± 4%) for 68 children with a favorable histology (log-rank P = .04 [P = .007]). CONCLUSIONS: These results provide further evidence that the optimal duration and choice of drugs for preoperative chemotherapy remain an open question. Outcome remained significantly worse for BWT than for unilateral Wilms tumor. To enable the conservative treatment of as many affected kidneys as possible, only centers with experience in BWT should manage such cases.

KEYWORDS

SHARE & LIKE

COMMENTS

ABOUT THE AUTHOR

Cancer

0 Following 1 Fans 0 Projects 38 Articles

SIMILAR ARTICLES

Abstract BACKGROUND: Similar to the pancreatic intraepithelial neoplasia (PanIN)-pancreatic carcinoma sequence model, intrahepatic cholangiocarcinoma

阅读更多

Abstract BACKGROUND: Breast cancer occurs rarely in men. To the authors' knowledge, no population-based estimates of the incidence of human epiderm

阅读更多

Abstract BACKGROUND: MicroRNAs (miRNAs) play a crucial role in carcinogenesis; however, it largely remains unclear whether miRNAs in gastric juice,

阅读更多

Abstract BACKGROUND: Recently, chromosomal rearrangements involving receptor tyrosine kinases (RTKs) have been described in common epithelial maligna

阅读更多

Abstract BACKGROUND: Patients with metastatic gastric cancer have poor survival. The purpose of this study was to compare outcomes of metastatic gast

阅读更多

Abstract BACKGROUND: Alterations in the regulator of G-protein signaling (RGS) pathway have been implicated in several cancers; therefore, the author

阅读更多

Abstract BACKGROUND: Germline mutations in BRCA1 and BRCA2 (BRCA1/2) are related to an increased lifetime risk of developing breast and ovarian cance

阅读更多

Abstract BACKGROUND: Ipilimumab 3 mg/kg was the first agent to demonstrate improved survival in previously treated patients with metastatic melanoma

阅读更多

Abstract BACKGROUND: The management of relapsed aggressive lymphomas remains problematic. Ixabepilone (BMS-247550, epothilone B analog), a potent inh

阅读更多

Abstract BACKGROUND: Radiotherapy may lead to side effects that undermine patients' quality of life (QOL). Although mind-body practices like qigong a

阅读更多