Nita L Seibel1,2, Yueh-Yun Chi3, Elizabeth J Perlman4, Jing Tian3, Junfeng Sun5, James R Anderson6, Michael L Ritchey7, Patrick R Thomas8, James Miser9, John A Kalapurakal10, Paul E Grundy11, Daniel M Green12. 1. Cancer Therapy Evaluation Program, National Cancer Institute, Bethesda, Maryland. 2. George Washington University School of Medicine and Health Science, Washington, District of Columbia. 3. Department of Biostatistics, University of Florida, Gainesville, Florida. 4. Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois. 5. Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, Maryland. 6. Merck Research Laboratories-Oncology, North Wales, Pennsylvania. 7. Phoenix Children's Hospital, Phoenix, Arizona. 8. Temple University Hospital, Philadelphia, Pennsylvania. 9. City of Hope National Medical Center, Duarte, California. 10. Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois. 11. Department of Pediatrics and Oncology, University of Alberta, Edmonton, Alberta, Canada. 12. Department of Epidemiology and Cancer Control, St Jude Children's Research Hospital, Memphis, Tennessee.
Abstract
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.
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.
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Authors: Prakriti Roy; Sophie E van Peer; Martin M de Witte; Godelieve A M Tytgat; Henrike E Karim-Kos; Martine van Grotel; Cees P van de Ven; Annelies M C Mavinkurve-Groothuis; Johannes H M Merks; Roland P Kuiper; Janna A Hol; Geert O R Janssens; Ronald R de Krijger; Marjolijn C J Jongmans; Jarno Drost; Alida F W van der Steeg; Annemieke S Littooij; Marc H W A Wijnen; Harm van Tinteren; Marry M van den Heuvel-Eibrink Journal: PLoS One Date: 2022-01-13 Impact factor: 3.240