Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5)

Nita L. Seibel*, Yueh Yun Chi, Elizabeth J Perlman, Jing Tian, Junfeng Sun, James R. Anderson, Michael L. Ritchey, Patrick R. Thomas, James Miser, John A Kalapurakal, Paul E. Grundy, Daniel M. Green

*Corresponding author for this work

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Article numbere27450
JournalPediatric Blood and Cancer
Volume66
Issue number1
DOIs
StatePublished - Jan 1 2019

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Clear Cell Sarcoma
Etoposide
Cyclophosphamide
Kidney
Disease-Free Survival
Therapeutics
Survival
Brain
Vincristine
Pelvis
Abdomen
Doxorubicin
Wilms tumor and radial bilateral aplasia
Thorax
Head
Radiation
Neoplasm Metastasis
Bone and Bones
Recurrence

Keywords

  • clear cell sarcoma kidney
  • pediatric kidney cancer
  • treatment

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

Cite this

Seibel, Nita L. ; Chi, Yueh Yun ; 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. / Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5). In: Pediatric Blood and Cancer. 2019 ; Vol. 66, No. 1.
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title = "Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5)",
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.",
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author = "Seibel, {Nita L.} and Chi, {Yueh Yun} and Perlman, {Elizabeth J} and Jing Tian and Junfeng Sun and Anderson, {James R.} and Ritchey, {Michael L.} and Thomas, {Patrick R.} and James Miser and Kalapurakal, {John A} and Grundy, {Paul E.} and Green, {Daniel M.}",
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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). / Seibel, Nita L.; Chi, Yueh Yun; 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.

In: Pediatric Blood and Cancer, Vol. 66, No. 1, e27450, 01.01.2019.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the kidney treated on the National Wilms Tumor Study-5 (NWTS-5)

AU - Seibel, Nita L.

AU - Chi, Yueh Yun

AU - Perlman, Elizabeth J

AU - Tian, Jing

AU - Sun, Junfeng

AU - Anderson, James R.

AU - Ritchey, Michael L.

AU - Thomas, Patrick R.

AU - Miser, James

AU - Kalapurakal, John A

AU - Grundy, Paul E.

AU - Green, Daniel M.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - clear cell sarcoma kidney

KW - pediatric kidney cancer

KW - treatment

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U2 - 10.1002/pbc.27450

DO - 10.1002/pbc.27450

M3 - Article

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