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

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. Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children’s Oncology Group studies AREN0321 and AREN03B2. Cancer. 2024 Jul 1;130(13):2361-2371. doi: 10.1002/cncr.35266. Epub 2024 Feb 23. PubMed PMID: 38396300; PubMed Central PMCID: PMC11162327.

On NWTS-5, treatment for clear cell sarcoma of the kidney (CCSK) included vincristine/doxorubicin/cyclophosphamide/etoposide (Regimen I) and radiation therapy (RT), yielding 5-year event-free survival (EFS) of 100%, 88%, 73%, and 29% for stage I, II, III, and IV, respectively. COG study AREN0321 risk-adapted therapy: RT was omitted for stage I if lymph nodes were sampled and carboplatin was added for stage IV (Regimen UH-1). Patients with stage II/III received Regimen I with RT. Four-year EFS was analyzed for patients enrolled on AREN0321 and those on AREN03B2 who received AREN0321 stage-appropriate chemotherapy.