Key result
Surgical resection of a rare intradural extramedullary epithelioid hemangioendothelioma in the thoracic spinal cord improved motor disturbance, though the patient died of aspiration pneumonia 19 months later.
Why the study?
Epithelioid hemangioendothelioma is a rare vascular tumor that infrequently develops in the central nervous system, with no prior reported cases in the spinal cord.
Case Report (n=1)
This report describes the first known case of primary epithelioid hemangioendothelioma of the spinal cord, indicating it should be considered in the differential diagnosis of spinal intradural extramedullary tumors.
First spinal cord EHE broadens myelopathy differential; leaves open optimal CNS management strategies.
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.
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Ikezawa et al. (2021) conducted a case report in Intradural Extramedullary Epithelioid Hemangioendothelioma of the Thoracic Spinal Cord (n=1). Surgical resection (total laminectomy of T9-T10) was evaluated. Surgical resection of a rare intradural extramedullary epithelioid hemangioendothelioma in the thoracic spinal cord improved motor disturbance, though the patient died of aspiration pneumonia 19 months later.
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