Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2

Daniel J. Benedetti; Lindsay A. Renfro; Ian Tfirn; Najat C. Daw; John A. Kalapurakal; Peter F. Ehrlich; Geetika Khanna; Elizabeth Perlman; Anne Warwick; Kenneth W. Gow; Arnold C. Paulino; Nita L. Seibel; Paul Grundy; Conrad V. Fernandez; James I. Geller; Elizabeth A. Mullen; Jeffrey S. Dome

doi:10.1002/cncr.35266

Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2

Daniel J. Benedetti^*, Lindsay A. Renfro, Ian Tfirn, Najat C. Daw, John A. Kalapurakal, Peter F. Ehrlich, Geetika Khanna, Elizabeth Perlman, Anne Warwick, Kenneth W. Gow, Arnold C. Paulino, Nita L. Seibel, Paul Grundy, Conrad V. Fernandez, James I. Geller, Elizabeth A. Mullen, Jeffrey S. Dome

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

5 Scopus citations

Abstract

Background: On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5-year event-free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children’s Oncology Group study AREN0321 of risk-adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH-1). Patients who had stage II/III disease received regimen I with RT. Methods: Four-year EFS was analyzed for patients enrolled on AREN0321 and on those enrolled on AREN03B2 who received AREN0321 stage-appropriate chemotherapy. Results: Eighty-two patients with CCSK enrolled on AREN0321, 50 enrolled on AREN03B2 only. The 4-year EFS rate was 82.7% (95% confidence interval [CI], 74.8%–91.4%) for AREN0321 and 89.6% (95% CI, 81.3%–98.7%) for AREN03B2 only (p =.28). When combining studies, the 4-year EFS rates for patients who had stage I (n = 10), II (n = 47), III (n = 65), and IV (n = 10) disease were 90% (95% CI, 73.2%–100.0%), 93.4% (95% CI, 86.4%–100.0%), 82.8% (95% CI, 74.1%–92.6%), and 58.3% (95% CI, 34%–100.0%), respectively. There were no local recurrences among seven patients with stage I disease who were treated without RT. One stage I recurrence occurred in the brain, which was the most common site of relapse overall. Among patients with local stage III tumors, neither initial procedure type, margin status, nor lymph node involvement were prognostic. Conclusions: Patients with stage I CCSK had excellent outcomes without local recurrences when treated without RT. Patients with stage IV disease appeared to benefit from a carboplatin-containing regimen, although their outcomes remained unsatisfactory. Further research is needed to improve outcomes for patients with advanced-stage disease (ClinicalTrials.gov identifiers NCT00335556 and NCT00898365).

Original language	English (US)
Pages (from-to)	2361-2371
Number of pages	11
Journal	cancer
Volume	130
Issue number	13
DOIs	https://doi.org/10.1002/cncr.35266
State	Published - Jul 1 2024

Funding

We thank the parents and children who enrolled in these studies and the many research coordinators, pediatric oncologists, pathologists, surgeons, radiation oncologists, radiologists, and other health professionals who cared for the children entered in the studies. We also thank the entirety of the AREN0321 and AREN03B2 study committees, including pathology, surgery, radiology, and oncology reviewers, as well as research and protocol coordinators who worked on the protocols over the years. Presented at the 55th Congress of the International Society of Pediatric Oncology (SIOP) in Ottawa, Canada, October 13, 2023. Supported by Grants 2U10CA180899-06, U10CA180886, U10CA180899, U10CA098543, U10CA098413, and U24CA114766 to support the Children's Oncology Group and Grant R50CA275931 to Jeffrey S. Dome from the National Cancer Institute, National Institutes of Health, The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Supported by St Baldrick's Foundation. Supported by the Solder True Life Foundation (Elizabeth A. Mullen). We thank the parents and children who enrolled in these studies and the many research coordinators, pediatric oncologists, pathologists, surgeons, radiation oncologists, radiologists, and other health professionals who cared for the children entered in the studies. We also thank the entirety of the AREN0321 and AREN03B2 study committees, including pathology, surgery, radiology, and oncology reviewers, as well as research and protocol coordinators who worked on the protocols over the years. Presented at the 55th Congress of the International Society of Pediatric Oncology (SIOP) in Ottawa, Canada, October 13, 2023. Supported by Grants 2U10CA180899\u201006, U10CA180886, U10CA180899, U10CA098543, U10CA098413, and U24CA114766 to support the Children's Oncology Group and Grant R50CA275931 to Jeffrey S. Dome from the National Cancer Institute, National Institutes of Health, The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Supported by St Baldrick's Foundation. Supported by the Solder True Life Foundation (Elizabeth A. Mullen).

Keywords

brain metastases
carboplatin
clear cell sarcoma of the kidney (CCSK)
radiotherapy

ASJC Scopus subject areas

Oncology
Cancer Research

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1002/cncr.35266

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Benedetti, D. J., Renfro, L. A., Tfirn, I., Daw, N. C., Kalapurakal, J. A., Ehrlich, P. F., Khanna, G., Perlman, E., Warwick, A., Gow, K. W., Paulino, A. C., Seibel, N. L., Grundy, P., Fernandez, C. V., Geller, J. I., Mullen, E. A., & Dome, J. S. (2024). Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2. cancer, 130(13), 2361-2371. https://doi.org/10.1002/cncr.35266

@article{5cbf00bfb43e4c98b81151636d5899e0,

title = "Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2",

abstract = "Background: On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5-year event-free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children{\textquoteright}s Oncology Group study AREN0321 of risk-adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH-1). Patients who had stage II/III disease received regimen I with RT. Methods: Four-year EFS was analyzed for patients enrolled on AREN0321 and on those enrolled on AREN03B2 who received AREN0321 stage-appropriate chemotherapy. Results: Eighty-two patients with CCSK enrolled on AREN0321, 50 enrolled on AREN03B2 only. The 4-year EFS rate was 82.7% (95% confidence interval [CI], 74.8%–91.4%) for AREN0321 and 89.6% (95% CI, 81.3%–98.7%) for AREN03B2 only (p =.28). When combining studies, the 4-year EFS rates for patients who had stage I (n = 10), II (n = 47), III (n = 65), and IV (n = 10) disease were 90% (95% CI, 73.2%–100.0%), 93.4% (95% CI, 86.4%–100.0%), 82.8% (95% CI, 74.1%–92.6%), and 58.3% (95% CI, 34%–100.0%), respectively. There were no local recurrences among seven patients with stage I disease who were treated without RT. One stage I recurrence occurred in the brain, which was the most common site of relapse overall. Among patients with local stage III tumors, neither initial procedure type, margin status, nor lymph node involvement were prognostic. Conclusions: Patients with stage I CCSK had excellent outcomes without local recurrences when treated without RT. Patients with stage IV disease appeared to benefit from a carboplatin-containing regimen, although their outcomes remained unsatisfactory. Further research is needed to improve outcomes for patients with advanced-stage disease (ClinicalTrials.gov identifiers NCT00335556 and NCT00898365).",

keywords = "brain metastases, carboplatin, clear cell sarcoma of the kidney (CCSK), radiotherapy",

author = "Benedetti, {Daniel J.} and Renfro, {Lindsay A.} and Ian Tfirn and Daw, {Najat C.} and Kalapurakal, {John A.} and Ehrlich, {Peter F.} and Geetika Khanna and Elizabeth Perlman and Anne Warwick and Gow, {Kenneth W.} and Paulino, {Arnold C.} and Seibel, {Nita L.} and Paul Grundy and Fernandez, {Conrad V.} and Geller, {James I.} and Mullen, {Elizabeth A.} and Dome, {Jeffrey S.}",

note = "Publisher Copyright: {\textcopyright} 2024 The Authors. Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.",

year = "2024",

month = jul,

day = "1",

doi = "10.1002/cncr.35266",

language = "English (US)",

volume = "130",

pages = "2361--2371",

journal = "cancer",

issn = "0008-543X",

publisher = "John Wiley and Sons Inc.",

number = "13",

}

Benedetti, DJ, Renfro, LA, Tfirn, I, Daw, NC, Kalapurakal, JA, Ehrlich, PF, Khanna, G, Perlman, E, Warwick, A, Gow, KW, Paulino, AC, Seibel, NL, Grundy, P, Fernandez, CV, Geller, JI, Mullen, EA & Dome, JS 2024, 'Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2', cancer, vol. 130, no. 13, pp. 2361-2371. https://doi.org/10.1002/cncr.35266

TY - JOUR

T1 - Treatment and outcomes of clear cell sarcoma of the kidney

T2 - A report from the Children's Oncology Group studies AREN0321 and AREN03B2

AU - Benedetti, Daniel J.

AU - Renfro, Lindsay A.

AU - Tfirn, Ian

AU - Daw, Najat C.

AU - Kalapurakal, John A.

AU - Ehrlich, Peter F.

AU - Khanna, Geetika

AU - Perlman, Elizabeth

AU - Warwick, Anne

AU - Gow, Kenneth W.

AU - Paulino, Arnold C.

AU - Seibel, Nita L.

AU - Grundy, Paul

AU - Fernandez, Conrad V.

AU - Geller, James I.

AU - Mullen, Elizabeth A.

AU - Dome, Jeffrey S.

PY - 2024/7/1

Y1 - 2024/7/1

N2 - Background: On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5-year event-free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children’s Oncology Group study AREN0321 of risk-adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH-1). Patients who had stage II/III disease received regimen I with RT. Methods: Four-year EFS was analyzed for patients enrolled on AREN0321 and on those enrolled on AREN03B2 who received AREN0321 stage-appropriate chemotherapy. Results: Eighty-two patients with CCSK enrolled on AREN0321, 50 enrolled on AREN03B2 only. The 4-year EFS rate was 82.7% (95% confidence interval [CI], 74.8%–91.4%) for AREN0321 and 89.6% (95% CI, 81.3%–98.7%) for AREN03B2 only (p =.28). When combining studies, the 4-year EFS rates for patients who had stage I (n = 10), II (n = 47), III (n = 65), and IV (n = 10) disease were 90% (95% CI, 73.2%–100.0%), 93.4% (95% CI, 86.4%–100.0%), 82.8% (95% CI, 74.1%–92.6%), and 58.3% (95% CI, 34%–100.0%), respectively. There were no local recurrences among seven patients with stage I disease who were treated without RT. One stage I recurrence occurred in the brain, which was the most common site of relapse overall. Among patients with local stage III tumors, neither initial procedure type, margin status, nor lymph node involvement were prognostic. Conclusions: Patients with stage I CCSK had excellent outcomes without local recurrences when treated without RT. Patients with stage IV disease appeared to benefit from a carboplatin-containing regimen, although their outcomes remained unsatisfactory. Further research is needed to improve outcomes for patients with advanced-stage disease (ClinicalTrials.gov identifiers NCT00335556 and NCT00898365).

AB - Background: On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5-year event-free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children’s Oncology Group study AREN0321 of risk-adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH-1). Patients who had stage II/III disease received regimen I with RT. Methods: Four-year EFS was analyzed for patients enrolled on AREN0321 and on those enrolled on AREN03B2 who received AREN0321 stage-appropriate chemotherapy. Results: Eighty-two patients with CCSK enrolled on AREN0321, 50 enrolled on AREN03B2 only. The 4-year EFS rate was 82.7% (95% confidence interval [CI], 74.8%–91.4%) for AREN0321 and 89.6% (95% CI, 81.3%–98.7%) for AREN03B2 only (p =.28). When combining studies, the 4-year EFS rates for patients who had stage I (n = 10), II (n = 47), III (n = 65), and IV (n = 10) disease were 90% (95% CI, 73.2%–100.0%), 93.4% (95% CI, 86.4%–100.0%), 82.8% (95% CI, 74.1%–92.6%), and 58.3% (95% CI, 34%–100.0%), respectively. There were no local recurrences among seven patients with stage I disease who were treated without RT. One stage I recurrence occurred in the brain, which was the most common site of relapse overall. Among patients with local stage III tumors, neither initial procedure type, margin status, nor lymph node involvement were prognostic. Conclusions: Patients with stage I CCSK had excellent outcomes without local recurrences when treated without RT. Patients with stage IV disease appeared to benefit from a carboplatin-containing regimen, although their outcomes remained unsatisfactory. Further research is needed to improve outcomes for patients with advanced-stage disease (ClinicalTrials.gov identifiers NCT00335556 and NCT00898365).

KW - brain metastases

KW - carboplatin

KW - clear cell sarcoma of the kidney (CCSK)

KW - radiotherapy

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Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2

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