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

Yeon Ju Lee; Yun Sup Hwang; Kyung Jin Seo; Young Joo Kim

doi:10.3348/jksr.2019.80.5.981

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

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

Case Report

J Korean Soc Radiol 2019;80(5):981-986.

Published online: 20 January 2019

DOI: https://doi.org/10.3348/jksr.2019.80.5.981

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

Yeon Ju Lee, MD¹, Yun Sup Hwang, MD¹, Kyung Jin Seo, MD², Young Joo Kim, MD^1,^∗

¹Department of Radiology, Uijeongbu St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Uijeongbu, Korea

²Department of Pathology, Uijeongbu St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Uijeongbu, Korea

^∗Corresponding author Young Joo Kim, MD Department of Radiology, Uijeongbu St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 271 Cheonbo-ro, Uijeongbu 11765, Korea. Tel 82-31-820-3599 Fax 82-31-846-3080 E-mail violet2@catholic.ac.kr

Received 21 November 2018 Revised 10 January 2019 Accepted 18 February 2019

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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).

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.

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