Abstract
There is a paucity of information about the clinical characteristics and long-term outcomes of pediatric epithelioid hemangioendothelioma (EHE), a rare vascular neoplasm commonly presenting in adulthood. In our case series of 24 patients with EHE aged 2-26 years, the majority presented with multi-organ disease. Progression was seen in 63% of patients with a mean time to progression of 18.4 months (range: 0-72). Three patients treated with sirolimus achieved stable disease or partial response for >2.5 years. Longitudinal prospective pediatric studies are needed to develop standardized approaches to surgical and medical management.
Original language | English (US) |
---|---|
Article number | e28045 |
Journal | Pediatric Blood and Cancer |
Volume | 67 |
Issue number | 2 |
DOIs | |
State | Published - Feb 1 2020 |
Externally published | Yes |
Keywords
- epithelioid
- hemangioendothelioma
- pediatric
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Hematology
- Oncology