What is epithelioid hemangioendothelioma of the T8 and T9 vertebrae?

Medical Advisory BoardAll articles are reviewed for accuracy by our Medical Advisory Board
Educational purpose only • Exercise caution as content is pending human review
Article Review Status
Submitted
Under Review
Approved

Last updated: February 23, 2026 • View editorial policy

Personalize

Help us tailor your experience

Which best describes you? Your choice helps us use language that's most understandable for you.

Epithelioid Hemangioendothelioma of the T8 and T9 Vertebrae

Epithelioid hemangioendothelioma (EHE) of the T8 and T9 vertebrae is a rare, low-to-intermediate grade malignant vascular tumor arising from endothelial cells that carries an unpredictable prognosis and requires aggressive surgical resection when feasible, as complete excision offers the best chance for survival. 1, 2

Tumor Characteristics and Biology

EHE is a rare vascular neoplasm with biological behavior intermediate between benign hemangioma and high-grade angiosarcoma. 2, 3 The tumor is composed of epithelioid or histiocytoid endothelial cells with rounded nuclei and prominent cytoplasmic vacuolization, representing primitive lumen formation. 3 These cells grow in small nests or cords and only focally line well-formed vascular channels, which frequently leads to misdiagnosis as metastatic carcinoma. 3

Diagnostic Features

The definitive diagnosis requires immunohistochemical confirmation showing tumor cells positive for vascular markers including vimentin, CD31, CD34, factor VIII-related antigen, ERG, and FLI1. 2, 4 The epithelioid appearance and solid growth pattern can mimic carcinoma, but EHE typically lacks the pleomorphism and mitotic activity seen in true carcinomas. 3

Clinical Presentation in Spinal Disease

Focal back pain is the most common presenting symptom in spinal EHE. 2 The disease can progress rapidly to cause:

  • Pathologic vertebral fractures with acute collapse 1, 4
  • Spinal cord compression leading to neurological deficits including paraplegia 1, 2
  • Unsteady gait from progressive cord compression 2

Multifocal disease occurs in approximately 40% of cases, making comprehensive staging essential. 2

Radiological and Pathological Considerations

Radiological features are nonspecific, typically appearing as osteolytic lesions with decreased vertebral body height and enhancing masses on MRI. 2 A critical pitfall is that percutaneous needle biopsies are frequently non-diagnostic and may delay appropriate treatment. 2, 4 Open biopsy or resection specimens are often required for definitive diagnosis.

Treatment Approach

En bloc surgical resection should be attempted whenever anatomically feasible, as complete excision is associated with the best outcomes. 1, 2, 4 However, only 15% of patients are suitable candidates for total resection. 1

Surgical Strategy

For vertebral EHE with cord compression:

  • Preoperative embolization to reduce intraoperative blood loss 2
  • Vertebrectomy with complete tumor excision when possible 4
  • Spinal canal decompression for neurological preservation 2, 4
  • Posterior instrumentation and stabilization (typically 2-3 levels above and below) 2

Adjuvant Therapy

Radiotherapy should be administered following incomplete resection or when en bloc resection is not achievable. 2, 5 Radiotherapy offers local pain control with good tolerance and improved quality of life, though the optimal radiation dose remains undefined. 5

The role of chemotherapy is not well-established, though it has been used in multimodal treatment protocols for aggressive or metastatic disease. 1, 2

Prognosis and Natural History

The natural history of spinal EHE is highly unpredictable and primarily determined by tumor location and resectability. 1, 2 The lesions are potentially aggressive with capacity for:

  • Local recurrence after incomplete resection 3
  • Distant metastases (reported in approximately 15% of cases) 3
  • Rapid progression leading to death within weeks in aggressive presentations 1

Patients achieving wide surgical margins without adjuvant therapy can remain disease-free for years, as demonstrated in cases with 6-year follow-up showing no recurrence. 4

Critical Management Pitfalls

The most dangerous error is misdiagnosing EHE as metastatic carcinoma based on epithelioid morphology alone, which leads to inappropriate palliative rather than curative surgical approaches. 3 Additionally, relying on percutaneous biopsy results can cause treatment delays when samples are non-diagnostic. 2

Expert histopathological review is mandatory to exclude angiosarcoma, which carries universal recurrence and early mortality after any intervention. 6

References

6
Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Related Questions

What is the appropriate lisinopril dosing for adults with hypertension, heart failure, post‑myocardial infarction, and diabetic nephropathy, and how should the dose be adjusted for impaired renal function, elderly patients, and pregnancy?
How should a ventricular septal defect be managed based on defect size, symptoms, and pulmonary pressures?
What is the recommended first‑line and subsequent treatment for acute sciatica in otherwise healthy adults and young adults?
Should I start levothyroxine for an asymptomatic patient with a TSH of 6.9 mIU/L, normal free T4 and T3, who is not pregnant and has no cardiac disease?
What oral elemental iron dose should be given to a non‑pregnant healthy adult with a transferrin saturation of 15%?
Is there a rapid detox protocol for alprazolam (Xanax)?

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

Have a follow-up question?

Our Medical A.I. is used by practicing medical doctors at top research institutions around the world. Ask any follow up question and get world-class guideline-backed answers instantly.

Ask Question