Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents.

Hettmer, Simone; Andrieux, Geoffroy; Hochrein, Jochen; Kurz, Philipp; Rössler, Jochen; Lassmann, Silke; Werner, Martin; von Bubnoff, Nikolas; Peters, Christoph; Koscielniak, Ewa; Sparber-Sauer, Monika; Niemeyer, Charlotte; Mentzel, Thomas; Busch, Hauke; Boerries, Melanie (2017). Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents. Pediatric blood & cancer, 64(12) Wiley-Liss 10.1002/pbc.26675

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Epithelioid hemangioendothelioma (EHE) is a rare, vascular sarcoma. Visceral forms arise in the liver/ lungs. We review the clinical and molecular phenotype of pediatric visceral EHE based on the case of a 9-year-old male child with EHE of the liver/lungs. His tumor expressed the EHE-specific fusion oncogene WWTR1-CAMTA1. Molecular characterization revealed a low somatic mutation rate and activated interferon signaling, angiogenesis regulation, and blood vessel remodeling. After polychemotherapy and resection of lung tumors, residual disease remained stable on oral lenalidomide. Literature review identified another 24 children with EHE of the liver/lungs. Most presented with multifocal, systemic disease. Only those who underwent complete resection achieved complete remission. Four children experienced rapid progression and died. In six children, disease remained stable for years without therapy. Two patients died from progressive EHE 21 and 24 years after first diagnosis. Natural evolution of pediatric visceral EHE is variable, and long-term prognosis remains unclear.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Haematology/Oncology

UniBE Contributor:

Rössler, Jochen Karl

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1545-5009

Publisher:

Wiley-Liss

Language:

English

Submitter:

Jochen Karl Rössler

Date Deposited:

09 Dec 2020 18:55

Last Modified:

09 Dec 2020 18:55

Publisher DOI:

10.1002/pbc.26675

PubMed ID:

28598585

Uncontrolled Keywords:

children epithelioid hemangioendothelioma liver lung molecular characterization

BORIS DOI:

10.7892/boris.137591

URI:

https://boris.unibe.ch/id/eprint/137591

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