PMID- 30255545
OWN - NLM
STAT- MEDLINE
DCOM- 20191021
LR  - 20200101
IS  - 1545-5017 (Electronic)
IS  - 1545-5009 (Print)
IS  - 1545-5009 (Linking)
VI  - 66
IP  - 1
DP  - 2019 Jan
TI  - Impact of cyclophosphamide and etoposide on outcome of clear cell sarcoma of the 
      kidney treated on the National Wilms Tumor Study-5 (NWTS-5).
PG  - e27450
LID - 10.1002/pbc.27450 [doi]
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.
CI  - (c) 2018 Wiley Periodicals, Inc.
FAU - Seibel, Nita L
AU  - Seibel NL
AUID- ORCID: 0000-0001-6259-7955
AD  - Cancer Therapy Evaluation Program, National Cancer Institute, Bethesda, Maryland.
AD  - George Washington University School of Medicine and Health Science, Washington, 
      District of Columbia.
FAU - Chi, Yueh-Yun
AU  - Chi YY
AD  - Department of Biostatistics, University of Florida, Gainesville, Florida.
FAU - Perlman, Elizabeth J
AU  - Perlman EJ
AD  - Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois.
FAU - Tian, Jing
AU  - Tian J
AD  - Department of Biostatistics, University of Florida, Gainesville, Florida.
FAU - Sun, Junfeng
AU  - Sun J
AD  - Critical Care Medicine Department, Clinical Center, National Institutes of 
      Health, Bethesda, Maryland.
FAU - Anderson, James R
AU  - Anderson JR
AD  - Merck Research Laboratories-Oncology, North Wales, Pennsylvania.
FAU - Ritchey, Michael L
AU  - Ritchey ML
AD  - Phoenix Children's Hospital, Phoenix, Arizona.
FAU - Thomas, Patrick R
AU  - Thomas PR
AD  - Temple University Hospital, Philadelphia, Pennsylvania.
FAU - Miser, James
AU  - Miser J
AD  - City of Hope National Medical Center, Duarte, California.
FAU - Kalapurakal, John A
AU  - Kalapurakal JA
AD  - Lurie Comprehensive Cancer Center of Northwestern University, Chicago, Illinois.
FAU - Grundy, Paul E
AU  - Grundy PE
AD  - Department of Pediatrics and Oncology, University of Alberta, Edmonton, Alberta, 
      Canada.
FAU - Green, Daniel M
AU  - Green DM
AD  - Department of Epidemiology and Cancer Control, St Jude Children's Research 
      Hospital, Memphis, Tennessee.
LA  - eng
GR  - U10CA098543/NH/NIH HHS/United States
GR  - CA-42326/NH/NIH HHS/United States
GR  - U10 CA098543/CA/NCI NIH HHS/United States
GR  - U10CA180899./NH/NIH HHS/United States
GR  - U10CA180886/NH/NIH HHS/United States
GR  - U10 CA180899/CA/NCI NIH HHS/United States
GR  - U10 CA180886/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
DEP - 20180925
PL  - United States
TA  - Pediatr Blood Cancer
JT  - Pediatric blood & cancer
JID - 101186624
RN  - 6PLQ3CP4P3 (Etoposide)
RN  - 8N3DW7272P (Cyclophosphamide)
SB  - IM
MH  - Adolescent
MH  - Antineoplastic Combined Chemotherapy Protocols/*therapeutic use
MH  - Child
MH  - Child, Preschool
MH  - Cyclophosphamide/administration & dosage
MH  - Etoposide/administration & dosage
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Infant
MH  - Kidney Neoplasms/drug therapy/*mortality/pathology
MH  - Male
MH  - Prognosis
MH  - Prospective Studies
MH  - Sarcoma, Clear Cell/drug therapy/*mortality/pathology
MH  - Survival Rate
PMC - PMC6249042
MID - NIHMS986830
OTO - NOTNLM
OT  - clear cell sarcoma kidney
OT  - pediatric kidney cancer
OT  - treatment
COIS- Conflict of Interest: Dr. Anderson reports other from Merck, outside the 
      submitted work; none of the other authors reports a conflict.
EDAT- 2018/09/27 06:00
MHDA- 2019/10/23 06:00
PMCR- 2020/01/01
CRDT- 2018/09/27 06:00
PHST- 2018/07/10 00:00 [received]
PHST- 2018/08/10 00:00 [revised]
PHST- 2018/08/11 00:00 [accepted]
PHST- 2018/09/27 06:00 [pubmed]
PHST- 2019/10/23 06:00 [medline]
PHST- 2018/09/27 06:00 [entrez]
PHST- 2020/01/01 00:00 [pmc-release]
AID - 10.1002/pbc.27450 [doi]
PST - ppublish
SO  - Pediatr Blood Cancer. 2019 Jan;66(1):e27450. doi: 10.1002/pbc.27450. Epub 2018 
      Sep 25.