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

Abstract

We report a 48-year-old male patient with spinal epithelioid hemangioendothelioma in T3 and T4 who began with symptoms of paresthesia in the lower limbs and distal weakness of the right lower limb, back pain, increased limitation in walking, urinary incontinence, and constipation. A safe maximum resection was performed, finding residual disease during the PET/CT scan, so it was decided to treat with radiotherapy, and there was a good response to this treatment. A literature review of epithelioid hemangioendothelioma of the thoracic spine was done which showed a mean age of presentation of 41 years and a male-female ratio of 1:0.53. The main symptom was pain, which was present in 100% of the patients, and wide surgery was performed in 56.8% of the patients, intralesional surgery in 31.8%, and biopsy in 11.4%. A total of 46.6% of patients received radiation therapy, and only 6.6% received chemotherapy. The patients had an average follow-up of 38 months. We recommend that extension studies such as PET/CT scans be performed after surgical resection. This can serve as a follow-up with hemangioendothelioma epithelioma patients about metastatic disease or residual disease that will guide us in giving adjuvant treatments, such as radiotherapy or chemotherapy, for better control of the disease.

Keywords: epithelioid hemangioendothelioma; radiation therapy; spine surgery; spine tumor; vascular bone tumor.

Figure 1. Pre-surgical magnetic resonance imaging

(A) In the sagittal sections of T2-weighted MRI, a decrease in the signal of the T3 vertebral body lesion is observed, which is compressing the spinal cord (yellow arrow); (B) In the axial section of T2-weighted MRI, a lesion is observed that destroys the vertebral body (yellow arrow) and occupies the spinal canal, displacing neural tissue to the right (red arrow).

Figure 2. Pre-surgical computed tomography

(A) Axial section shows a lytic lesion affecting the vertebral body of T3 (yellow arrow); (B) Sagittal section shows involvement of articular facets and laminae of T3 and T4 (yellow arrows) and decrease in T3 signal (red arrow).

Figure 3. Histopathology study showing presence of areas of fibrosis with reparative bone resorption, characteristics of epithelioid hemangioendothelioma (white arrow).

Figure 4. Post-surgical PET/CT study

(A, B) PET/CT study before treatment with radiotherapy, in which hypermetabolism is observed with 18F-FDG uptake at the surgical site (white arrow). (C) Control PET/CT study one year after radiotherapy, where there is an absence of 18F-FDG hyper-uptake areas (white arrow). PET/CT: positron emission tomography/ computed tomography; 18F-FDG: 2-deoxy-2-(fluorine-18) fluoro-D-glucose.

Figure 5. CT with post-surgical 3D reconstruction

Lateral (A and B) and anteroposterior (C) views show adequate placement of the fixation system (white arrow).