Multifocal osseous epithelioid hemangioendothelioma involving extremities and spine with visceral metastasis

Epithelioid hemangioendothelioma (EH) is a rare locally aggressive intermediate grade malignant vasoformative tumor. Bone is a rarer primary site accounting for less than 1% of all malignant bone tumors. Multicentricity and/or multifocality are a relatively frequent occurrence, most commonly cluster...

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Bibliographic Details
Main Authors: Maitrayee Roy, Rajni Yadav, Tarun Kumar
Format: Article
Language:English
Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2018-01-01
Series:Indian Journal of Medical and Paediatric Oncology
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Online Access:http://www.ijmpo.org/article.asp?issn=0971-5851;year=2018;volume=39;issue=1;spage=103;epage=105;aulast=Roy
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Summary:Epithelioid hemangioendothelioma (EH) is a rare locally aggressive intermediate grade malignant vasoformative tumor. Bone is a rarer primary site accounting for less than 1% of all malignant bone tumors. Multicentricity and/or multifocality are a relatively frequent occurrence, most commonly clustered in the bones of lower extremities. The axial skeleton is less commonly affected, and concomitant visceral involvement is rarer. We report a unique case of a 20-year-old male who presented with multiple lytic lesions in the skull vault, vertebral bones, bilateral proximal femur, and both radii and humerii with metastasis to the lungs and liver. A final diagnosis of EH was established on biopsy from the right iliac bone. He initially received multiagent chemotherapy and is currently on antiangiogenic therapy. On follow-up positron emission tomography–computerized tomography done 6 months later, marked reduction in fluoro-D-glucose avidity of the hepatic and most skeletal lesions was noted.
ISSN:0971-5851