Intradural Extramedullary Epithelioid Hemangioendothelioma of the Thoracic Spinal Cord: A Case Report

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, and develops infrequently in the central nervous system. To our knowledge, this is the first case of EHE of the spinal cord. An 85-year-old man presented with about 6-month progressive myelopathy. Magnetic resonance imaging (MRI) demonstrated an oval-shaped intradural extramedullary mass at T10 level with extensive intramedullary edema. A reddish tumor was removed via a total laminectomy of T9−T10. Histologically, the tumor cells with nuclear atypia and active mitoses were immunopositive for vascular tumor markers, and formed a lobular architecture associated with capillary-sized vessels lined with edematous endothelial cells. Although very rare, EHE should be considered in the differential diagnosis of a spinal intradural extramedullary tumor.

Spinal Cord Syndrome Due to Extramedullary Epithelioid Hemangioendothelioma of the Thoracic Spine: A Case Report and Literature Review

“…Such involvement can simulate a clinical picture of spinal cord syndrome. However, it is important to consider differential diagnoses of conditions that affect the spine including myeloma, sarcomas, metastatic lesions, schwannoma, meningioma, and other vascular tumors [ 1 ].…”

“…Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, accounting for less than 1% of all vascular cancers [ 1 ]. It has an incidence rate of 0.038/100,000 per year and a prevalence of <1/1,000,000.…”

A case of extramedullary subdural spindle cell hemangioendothelioma of the thoracic spinal cord treated curatively with radiation therapy

“…From this, we conjecture that epidural lesions are more likely to occur in the upper segment of the thoracic spine for SCH, while intramedullary lesions are more likely to occur in the middle segment, and extramedullary subdural lesions are more likely to occur in the lower segment. Moreover, some histological types of intradural extramedullary lesions, such as epithelioid hemangioendothelioma and capillary hemangioma, are also prone to occur at the lower spinal cord level [ 10 , 11 ].…”