Nerve.  2024 Oct;10(2):172-178. 10.21129/nerve.2024.00633.

Recurrent Large Spinal Hemangioendothelioma: A Rare Case Report

• Chung S
• Jung NH
• Choi SJ
• Kim C

• KMID: 2560399
• DOI: http://doi.org/10.21129/nerve.2024.00633

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare benign vascular tumor, affecting fewer than one in a million individuals. Spinal EHE is exceptionally rare. This case report presents a case of recurrent spinal EHE, focusing on its clinical manifestation and providing a brief overview of its genetic characteristics. Seven years prior, a patient underwent subtotal resection of a lumbar and retroperitoneal EHE. Due to severe intraoperative bleeding, part of the lumbar spine mass was left unresected and subsequently treated with a moderate dose of radiation therapy. More recently, the patient presented with bilateral lower extremity weakness and urinary incontinence. Imaging studies revealed metastases in the thoracic and sacral bones, along with regrowth in the anterior portion of the previously treated spinal EHE. The patient underwent a series of operations to address both the regrowth and metastases, followed by intensity-modulated radiation therapy to target the remaining tumor tissue while minimizing damage to surrounding structures. Although EHE is generally considered benign, this indolent tumor has a tendency for persistent regrowth and metastasis. This case highlights the importance of radical resection and the potential role of higher radiation doses in preventing recurrence. Molecular testing may aid in achieving an accurate diagnosis and guiding effective treatment strategies.

Keyword

Hemangioendothelioma; Radiotherapy, intensity-modulated; Recurrence; Spine