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Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study‐5 (NWTS‐5)
Author(s) -
Seibel Nita L.,
Chi YuehYun,
Perlman Elizabeth J.,
Tian Jing,
Sun Junfeng,
Anderson James R.,
Ritchey Michael L.,
Thomas Patrick R.,
Miser James,
Kalapurakal John A.,
Grundy Paul E.,
Green Daniel M.
Publication year - 2019
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.27450
Subject(s) - medicine , etoposide , cyclophosphamide , wilms' tumor , vincristine , surgery , regimen , chemotherapy
Purpose To improve the event‐free survival (EFS) and overall survival (OS) for patients with clear cell sarcoma of the kidney (CCSK) by incorporating cyclophosphamide and etoposide into treatment on National Wilms Tumor Study (NWTS)‐5. Patients and methods Patients less than 16 years of age with a centrally confirmed pathological diagnosis of CCSK were eligible for treatment on this prospective single‐arm study conducted between August 1995 and June 2002. Staging consisted of CT scans of chest, abdomen, pelvis, bone scan, skeletal survey, and CT or MRI of the head. Treatment consisted of vincristine/doxorubicin/cyclophosphamide alternating with cyclophosphamide/etoposide for 24 weeks and radiation to sites of disease. Results One hundred eight eligible patients were enrolled on study (69% males, 63% Caucasian), with a median age of 22 months. Stage distribution was as follows: stage I, 12; II, 44; III, 45; IV, 7. Median follow‐up was 9.7 years. Five‐year EFS and OS were 79% (95% CI: 71%–88%) and 90% (95% CI: 84%–96%). Five‐year EFS for stage I–IV was 100%, 88%, 73%, and 29%, respectively. Twenty of the 23 disease‐related events occurred within three years of initial treatment. The most common site of recurrence was brain (12/23). Conclusion The outcome for patients with CCSK treated on NWTS‐5 was similar to NWTS‐4 and accomplished over a shorter treatment duration. Stage was highly predictive of outcome. Brain metastases occurred more frequently than on NWTS‐4. Regimen I showed more benefit for patients with stage I and II disease as compared with higher stages of disease where new therapies are needed.