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Clinical characterization and long-term outcomes in pediatric epithelioid hemangioendothelioma.
- Source :
-
Pediatric blood & cancer [Pediatr Blood Cancer] 2020 Feb; Vol. 67 (2), pp. e28045. Date of Electronic Publication: 2019 Nov 14. - Publication Year :
- 2020
-
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.<br /> (© 2019 Wiley Periodicals, Inc.)
- Subjects :
- Adolescent
Adult
Child
Child, Preschool
Combined Modality Therapy
Disease Progression
Female
Follow-Up Studies
Hemangioendothelioma, Epithelioid therapy
Humans
Male
Prognosis
Retrospective Studies
Survival Rate
Young Adult
Hemangioendothelioma, Epithelioid mortality
Hemangioendothelioma, Epithelioid pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1545-5017
- Volume :
- 67
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Pediatric blood & cancer
- Publication Type :
- Academic Journal
- Accession number :
- 31724797
- Full Text :
- https://doi.org/10.1002/pbc.28045