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. 2020 Feb;67(2):e28045.
doi: 10.1002/pbc.28045. Epub 2019 Nov 14.

Clinical characterization and long-term outcomes in pediatric epithelioid hemangioendothelioma

Eily Cournoyer  1   2 , Alyaa Al-Ibraheemi  3 , Elissa Engel  4 , Gulraiz Chaudry  5 , Stacie Stapleton  6 , Denise M Adams  2
Affiliations

Clinical characterization and long-term outcomes in pediatric epithelioid hemangioendothelioma

Eily Cournoyer et al. Pediatr Blood Cancer. 2020 Feb.

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.

Keywords: epithelioid; hemangioendothelioma; pediatric.

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References

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