J Korean Soc Radiol.  2019 Sep;80(5):981-986. 10.3348/jksr.2019.80.5.981.

Primary Epithelioid Hemangioendothelioma of the Central Nervous System: A Case Report

• Lee YJ
• Hwang YS
• Seo KJ
• Kim YJ

• KMID: 2459025
• DOI: http://doi.org/10.3348/jksr.2019.80.5.981

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.

MeSH Terms

Brain Neoplasms
Central Nervous System*
Hemangioendothelioma, Epithelioid*
Intracranial Hemorrhages
Magnetic Resonance Imaging
Sarcoma

Figure

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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).

• 

  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.

Reference

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