Introduction
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour that
originates from vascular endothelial or pre-endothelial cells. Its
demographic characteristics are unclear and there is no racial and sex
predilection,8The overall incidence of EHE is 0.230
per 1000000 person-years.8 EHE in general affects
patients in the age range 16-74 years but EHE of the bone is more
prevalent in the third and fourth decade of
life.2,3,5EHE can develop in any part of the body but
is reported to more frequently in the liver (34%), lungs (21%), bone
(19%) and other sites (26%). 1.,2 It was first
described and named in 1982 by Weiz and Enzinger and is classified in
the 2020 WHO classification of bone tumours as a low-to-intermediate
grade neoplasm.2.,3 Malignant vascular bone tumours
are rare and constitute less than 1% of primary bone
tumours.3 Bone involvement usually involves the lower
extremity (62%), upper extremities (14%) and vertebra
(10%)3. As such primary EHE of the spine is very
uncommon, with the thoracic spine most likely to be involved followed by
cervical, lumbar, and sacral levels in order of decreasing
frequency3.
Here we report on a 26-year-old female with thoracic T2 and T3 lesions
in keeping with primary EHE of the spine, her clinical presentation,
work up including laboratory, immunohistopathological and imaging
studies. We also conclude on her management and current condition. There
are currently no prior clinical case reports on primary EHE of the spine
in South Africa or Africa as a whole.