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Epithelioid hemangioendothelioma in children: The European Pediatric Soft Tissue Sarcoma Study Group experience
Daniel Orbach1, Max M Van Noesel2,3, Bernadette Brennan4
1SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, France.
Insights
Epithelioid hemangioendothelioma in children is rare. This study found that local therapy, primarily surgery, offers a favorable prognosis with high survival rates for localized disease.
Area of Science:
- Pediatric Oncology
- Vascular Tumors
- Epithelioid Hemangioendothelioma
Background:
- Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that can occur in children.
- Limited data exists on the treatment and outcomes of pediatric EHE.
- The European pediatric Soft tissue sarcoma Study Group has been prospectively collecting data on rare pediatric tumors.
Purpose of the Study:
- To analyze the outcomes of children diagnosed with epithelioid hemangioendothelioma.
- To evaluate the effectiveness of different treatment modalities in pediatric EHE.
- To report survival data for localized and metastatic pediatric EHE.
Main Methods:
- Retrospective analysis of pediatric patients with epithelioid hemangioendothelioma.
- Data collected from two prospective studies: NRSTS-05 and MTS-2008.
- Patients received local therapy (surgery) or systemic therapy; no radiotherapy was administered.
Main Results:
- Eleven pediatric patients were analyzed (10 localized, 1 metastatic).
- Median follow-up was 50 months; nine patients survived off therapy.
- Five-year progression-free survival was 77.1% and overall survival was 74.1%.
Conclusions:
- Pediatric epithelioid hemangioendothelioma treated with local therapy, predominantly surgery, shows a favorable prognosis.
- High survival rates are achievable, even with localized disease.
- Further research into optimal systemic therapy for metastatic cases is warranted.
Abstract:
The European pediatric Soft tissue sarcoma Study Group analyzed all children with epithelioid hemangioendothelioma prospectively registered in the NRSTS-05 (EUDRACT 2005-001139-31) and in MTS-2008 (NCT00379457) studies: 10 patients with localized and one with metastatic disease. Median age was 14.3 years (range, 9.0-18.8). Local therapy was initial primary surgery in seven cases, and five patients received systemic therapy. No patients received radiotherapy. After a median follow-up of 50 months (range, 6-176) for living patients, nine patients remain alive off therapy and two died. Five-year progression free and overall survivals are, respectively, 77.1% (95% confidence interval [CI]: 34.5-93.9) and 74.1% (95% CI: 28.1-93.0).
