Journal article
Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children's Oncology Group study ARST0332
Cancer, Vol.130(15), pp.2683-2693
04/03/2024
DOI: 10.1002/cncr.35305
PMCID: PMC11260243
PMID: 38567652
Abstract
Embryonal sarcoma of the liver (ESL) is a rare mesenchymal tumor most common in childhood; the optimal treatment approach is uncertain. The clinical features and outcomes of patients with ESL enrolled in a Children's Oncology Group (COG) clinical trial that evaluated a risk-based strategy for treating soft tissue sarcomas in patients aged <30 years were evaluated.
This subset analysis included patients with ESL enrolled in 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 four cycles of chemotherapy; radiotherapy for residual tumor was optional.
Thirty-nine eligible/evaluable patients with ESL were analyzed. All tumors were >10 cm in diameter; four were metastatic. Tumor resection was performed upfront in 23 and delayed in 16. Positive surgical margins (n = 6) and intraoperative tumor rupture (n = 6) occurred only in upfront resections. Eight patients received radiotherapy. Estimated 5-year event-free and overall survival were 79% (95% confidence interval [CI], 65%-93%) and 95% (95% CI, 87%-100%), respectively. Positive margins increased the local recurrence risk. One of 13 patients with documented hemorrhagic ascites and/or tumor rupture developed extrahepatic intra-abdominal tumor recurrence.
The treatment strategy used in ARST0332 achieved favorable outcomes for patients with ESL despite a substantial proportion having high-risk disease features. Deferring tumor resection until after neoadjuvant chemotherapy may decrease the risk of intraoperative tumor rupture and improve the likelihood of adequate surgical margins.
Details
- Title: Subtitle
- Embryonal sarcoma of the liver in pediatric and young adult patients: A report from Children's Oncology Group study ARST0332
- Creators
- Sheri L Spunt - Stanford UniversityWei Xue - Florida CollegeZhengya Gao - Florida CollegeAndrea Hayes Dixon - Howard UniversityLynn Million - Stanford UniversityStephanie F Polites - Mayo ClinicSanjeev A Vasudevan - Texas Children's HospitalSimon C Kao - University of IowaM Beth McCarville - St. Jude Children's Research HospitalDavid M Parham - University of Southern CaliforniaDonald A Barkauskas - University of Southern CaliforniaZhongjie Cai - University of Southern CaliforniaCarrye Cost - University of Colorado DenverLeo Mascarenhas - Children's Hospital of Los AngelesAaron R Weiss - Maine Medical Center
- Resource Type
- Journal article
- Publication Details
- Cancer, Vol.130(15), pp.2683-2693
- DOI
- 10.1002/cncr.35305
- PMID
- 38567652
- PMCID
- PMC11260243
- NLM abbreviation
- Cancer
- eISSN
- 1097-0142
- Grant note
- U10CA098543 / NIH HHS U10CA29511 / NIH HHS U10180899 / NIH HHS P01CA23099 / NIH HHS U10CA180803 / NIH HHS P30CA21765 / NIH HHS U10CA180886 / NIH HHS U10CA098413 / NIH HHS
- Language
- English
- Electronic publication date
- 04/03/2024
- Academic Unit
- Radiology; Stead Family Department of Pediatrics
- Record Identifier
- 9984582305302771
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