Suprasellar epithelioid hemangioendothelioma: Case report and review of the literature

Abstract

Background: Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin, which is clinically and histologically intermediate between benign hemangioma and angiosarcoma. It is most commonly found in the liver, lung, and bone, however, 46 intracranial cases have been reported in the literature, of which this is the fifth reported suprasellar tumor.

Case description: A 45-year-old woman developed progressive lethargy, somnolence, and memory decline over the course of 6 months. On computed tomography (CT), she was found to have a large hypothalamic mass and underwent subtotal resection via a bifrontal craniotomy.

Conclusions: While primary intracranial EHE is an uncommon presentation of a rare tumor, the suprasellar region does not seem to be an unusual location when it does occur. Prognosis is generally good, and may be better for primary intracranial disease than that for EHE originating elsewhere. Surgery is the first line of therapy, with variable benefit from adjuvant chemotherapy or radiation when total resection is not possible. Chemotherapeutic approaches in current use are directed at preventing endothelial proliferation.

Keywords: Epithelioid hemangioendothelioma; intracranial; review; suprasellar; vascular tumor.

Figure 1

(a) Sagittal T1 precontrast, (b) sagittal T1 postcontrast, (c) axial fluid-attenuated inversion recovery

Figure 2

(a) Hematoxylin and Eosin (H and E), low power, (b) H and E, medium power, (c) H and E, medium power, (d) H and E, medium power, (e) H and E, low power, (f) CD34, (g) CD31, (h) Erg, (i) SMA, (j) EMA, (k) CD163, (l) CD3, (m) CD20, (n) Ki-67, (o) GFAP