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A rare vascular tumor presenting as persistent pain at the site of a chronic DVT
Joseph J Fulton, Ashish Jain
University of North Carolina Hospitals, Chapel Hill, NC

Table of Contents

  • A rare vascular tumor presenting as persistent pain at the site of a chronic DVT


Background: Vascular neoplasms are unusual and, as such, pose a diagnostic and treatment challenge to the vascular surgeon. Epithelioid hemangioendotheliomas (EHE) are soft tissue tumors that typically arise in the liver, lungs, neck, bones, or spleen, that are composed of epithelioid endothelial cells. Intravascular EHEs are exceptionally rare. To date, and to our knowledge, there have only been about 35 cases of primary vascular EHE reported in the English literature. Intravascular EHE has been documented mainly in peripheral vessels, arising more commonly in large veins than in arteries. Metastases occur in an estimated 30% of patients, most commonly extending to regional lymph nodes or spreading to the lungs. EHE has a variable natural history and tumor histology has been of little help in predicting clinical outcomes.
Case Report: A 30-year old female presented with a two-year history of focal pain in her left, medial, upper thigh. Additionally, one year ago she developed cramping in her left calf when walking, and mild swelling in the left leg. At that time, she was diagnosed with a chronic DVT of her left femoral vein at another hospital and treated with 4 months of anticoagulation. All of her symptoms persisted and she eventually referred herself to our vascular clinic.
At presentation, the patient’s main complaint continued to be her focal thigh pain, which was exacerbated with any contact. She denied any weight loss, shortness of breath, or other systemic symptoms. The patient had no significant past medical or family history, and denied smoking. Physical exam was significant for tenderness to palpation of the medial thigh, slightly diminished pedal pulses, and 1+ edema of the left leg. No masses could be palpated. Duplex ultrasound revealed a focal, chronic occlusion of the femoral vein in the upper thigh, with an adjacent short-segment obstruction of the superficial femoral artery. Her ankle-brachial index was 0.82 on the left and 1.1 on the right. A tumor was suspected, and further diagnostic imaging was required. Magnetic resonance imaging found no evidence of a mass, and offered no additional information.
Given a high suspicion for a tumor, the patient underwent surgical exploration. A 4 cm mass was discovered encasing both the femoral vein and superficial femoral artery. A specimen was sent for frozen pathology, which was worrisome for metastatic adenocarcinoma. The mass was resected and the artery was repaired with a short segment interposition graft. The vein was not reconstructed. Final tissue pathology identified the diagnosis of EHE. Staging imaging studies revealed pulmonary nodules in all lobes, which were biopsy confirmed as metastatic EHE. No adjuvant chemotherapy or radiation was offered. The patient is currently symptom free and is undergoing serial imaging to monitor for disease progression.
Conclusion: Vascular tumors must be considered in young patients with unusual, otherwise unexplained vessel obstruction. Early diagnosis can be critical to patient outcome.
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