Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children’s Oncology Group study ARST0332

Spunt SL, Xue W, Gao Z, Hayes Dixon A, Million L, Polites SF, Vasudevan SA, Kao SC, McCarville MB, Parham DM, Barkauskas DA, Cai Z, Cost C, Mascarenhas L, Weiss AR. Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children’s Oncology Group study ARST0332. Cancer. 2024 Aug 1;130(15):2683-2693. doi: 10.1002/cncr.35305. Epub 2024 Apr 3. PubMed PMID: 38567652; PubMed Central PMCID: PMC11260243.

Embryonal sarcoma of the liver (ESL) is a rare mesenchymal tumor most common in childhood; the optimal treatment approach is uncertain. We analyzed the clinical features and outcomes of patients with ESL enrolled on a Children’s Oncology Group (COG) clinical trial that evaluated a risk-based strategy for treating soft tissue sarcomas in patients <30 years of age. This subset analysis included patients with ESL enrolled on COG study ARST0332. Central review of records, pathology, and imaging confirmed the diagnosis, presenting features, and surgery extent and complications. All patients received dose-intensive ifosfamide/doxorubicin chemotherapy, with cycle timing dependent on surgery and radiotherapy. Tumor resection occurred before study entry or after 4 cycles of chemotherapy; radiotherapy for residual tumor was optional.