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J Pediatr Hematol Oncol. 2017 Jan;39(1):50-55.

Outcome of Nephroblastoma Treatment According to the SIOP-2001 Strategy at a Single Institution in Argentina.

Author information

1
Departments of *Hemato-Oncology †Pathology ‡Surgery, Pediatric Hospital "Professor Dr. Juan P. Garrahan," Buenos Aires, Argentina.

Abstract

OBJECTIVE:

Wilms tumor (WT) is a disease with a good prognosis. The aim of this study was to evaluate the outcome of patients with WT, treated according to the SIOP-2001 strategy.

METHODS:

A retrospective analysis of 141 consecutive patients with WT diagnosed at our institution between December 2001 and 2013 was performed.

RESULTS:

A total of 114 patients, median age 38.8 months (3 to 155 mo), were assessable for analysis. Fine-needle aspiration was initially performed in 88 patients (84.6%). Stage distribution was: I: 33%, II: 9.6%, III: 28%, IV: 14%, V: 14.9%. Six patients were stage III because of tumor spillage. The remaining patients received preoperative chemotherapy. Adjuvant chemotherapy was given without randomization, using vincristine-actinomycin for stage II and vincristine-doxorubicin-actinomycin plus radiotherapy for stage III. After a median follow-up of 52 months, 5-year overall survival and event-free survival were 91% and 85%, respectively. Overall survival according to stage was: I: 96%, II: 99%, III: 88%, IV: 78%, V: 90% (P=0.16). There was no significant difference in event-free survival (P=0.7). Seventy-eight (85.7%) were intermediate-risk and 11 (12%) were high-risk patients. Seventeen patients (14.9%) relapsed within 2 to 99 months (median 29.9 mo). Eight patients (7%) died of progressive disease. There were no treatment-related deaths.

CONCLUSIONS:

The SIOP-01 protocol proposes a treatment strategy that is feasible in our institution, achieving good results.

PMID:
27379530
DOI:
10.1097/MPH.0000000000000632
[Indexed for MEDLINE]

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