Dural composite hemangioendothelioma: The first intracranial case

Abstract

Background: Composite hemangioendothelioma (CHE) is a rare, locally aggressive neoplasm of intermediate malignant potential. It is composed of a mixture of vascular tumors with a predilection for the dermis and subcutis of the extremities.

Case description: In this report, we describe a 41-year-old man who presented with a 2-month history of headache, dizziness, and intermittent seizures. Magnetic resonance imaging showed a hemorrhagic, multilobulated, and dural-based mass with extension into the calvarium. The mass measured 10.3 × 4.8 × 4 cm along the interhemispheric fissure and encased the superior sagittal sinus. Excision was performed, and histopathologic examination revealed a heterogeneous mixture of vascular components consisting of epithelioid hemangioendothelioma, retiform hemangioendothelioma, and hemangioma. This is the first report of a primary intracranial CHE.

Conclusion: The spectrum of mesenchymal neoplasms within the cranium expands to encompass CHE.

Keywords: Dura; Epithelioid hemangioendothelioma; Falx cerebri; Retiform hemangioendothelioma; Vascular neoplasm.

Figure 1:

Baseline noncontrast computed tomography and magnetic resonance imaging (MRI) images of the brain. (a) Coronal view shows an interhemispheric mass with left-sided mass effect. (b) Axial view using bone window reveals lytic involvement of calvarium. (c) Axial T2-weighted MRI shows superior sagittal sinus involvement. (d) Coronal T2-weighted MRI reveals tumor extension through the calvarium. (e) Sagittal T1-weighted MRI post-gadolinium shows tumor enhancement.

Figure 2:

Histologic features of the intracranial composite hemangioendothelioma. (a) Hematoxylin and eosin (H&E)-stained section shows an area of epithelioid hemangioendothelioma, with cells containing intracytoplasmic vacuoles embedded in a hyaline stromal matrix. (b) H&E-stained section shows an area of retiform hemangioendothelioma, with abundant arborizing vascular channels. (c) H&E-stained section shows an area of hemangioma, with dilated vascular channels lined by oval nuclei without atypia. (d) An anti-CD31 antibody reveals abundant endothelial cells in this neoplasm. (e) An anti-ERG antibody confirms the abundant endothelial cells in this neoplasm. (f) The Ki-67 proliferation index is low, with up to 3.6% of cells labeled with this antibody.