Journal List > J Korean Soc Radiol > v.80(5) > 1134538

Lee, Hwang, Seo, and Kim: Primary Epithelioid Hemangioendothelioma of the Central Nervous System: A Case Report

Abstract

Primary epithelioid hemangioendothelioma (EHE) of the central nervous system is an extremely rare sarcoma of vascular origin. Imaging findings have been reported for few cases. Herein, we present a case of intracranial EHE manifesting as spontaneous intracranial hemorrhage. The tumor presented as a well-demarcated hemorrhagic lesion. It had a peripheral location, and showed signs of two-layered target-like mild enhancement in the early phase and gradual fill-in delayed enhancement on MRI.

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Fig. 1.
A 72-year-old man with primary epithelioid hemangioendothelioma. A. Unenhanced axial CT scan shows a well-defined, hyperattenuating lesion in the right frontal lobe abutting the pial surface of the brain. Laterally, a band-like heterogeneous hypoattenuating area can be seen with vasogenic edema surrounding the lesion. B. The mass exhibits heterogeneous signal intensity, mainly hypointensity, on the axial T2-weighted image (upper left panel) with peripheral edema, heterogeneously isointense with the gray matter on T1-weighted images (upper central panel), and strongly hypointense “blooming” on susceptibility-weighted images (upper right panel). Axial T1-weighted image immediately after contrast injection (lower left panel) demonstrates two-layered target-like, mild peripheral enhancements. Five-minutes delayed postcontrast axial T1-weighted image shows a greater increase of contrast enhancement area and intensity that is pronounced at the periphery (lower right panel).
jksr-80-981f1.tif
Fig. 1.
A 72-year-old man with primary epithelioid hemangioendothelioma. C. Photomicrograph of hematoxylin and eosin-stained slide (left upper panel, × 12) reveals a relatively well demarcated and hemorrhagic tumor mass. The tumor was composed of epithelioid (black arrows) or spin-dle shaped cells (white arrows) in nests or cords (asterisks) within a myxoid stoma. Some tumor cells show small intracytoplasmic lumens (“blister cells”) that contain red cells (arrowheads) (right upper panel, × 400). Immunohistochemistry reveals positive factor VIII-related antigen staining (left lower panel, × 400) indicating an endothelial origin. Vimentin stain (right lower panel, × 400) reveals positive staining of part of the tumor, indicating a mesenchymal component.
jksr-80-981f2.tif
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