Epithelioid Hemangioendothelioma of Spleen and Bone: A Case Report
Melvin Denolf, Annelies Rappaport, Sigrun Delvaux
TL;DR
This case report describes a rare vascular tumor found in the spleen and bone, highlighting its unique imaging features and diagnosis.
Contribution
The report presents a rare case of EHE with multifocal involvement in the spleen and bone, emphasizing diagnostic imaging and CAMTA1 staining.
Findings
Multifocal bone lesions showed a target appearance on imaging.
Splenic lesions demonstrated delayed enhancement with persistent rim enhancement.
Bone biopsy with CAMTA1 staining confirmed the diagnosis of EHE.
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor that can originate in various parenchymatous organs, soft tissue, and bone. Extrahepatic involvement is exceedingly rare. In this case, multifocal disease in the spleen and bone was present. Bone lesions showed a target appearance. Splenic lesions showed delayed enhancement of solid components with persistent rim enhancement. A bone biopsy with CAMTA1 staining confirmed the diagnosis. Teaching point: The presence of multifocal bone and splenic lesions can raise suspicion of EHE when other multifocal diseases are excluded.
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Taxonomy
TopicsVascular Tumors and Angiosarcomas · Occupational and environmental lung diseases · Eosinophilic Disorders and Syndromes