Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK.
Department of Internal Medicine, University of Oklahoma Health Sciences Center, Oklahoma City, OK.
World Neurosurg. 2024 Aug;188:99-110. doi: 10.1016/j.wneu.2024.05.006. Epub 2024 May 9.
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor predominantly arising in soft tissue. We report a rare case of thoracic spinal EHE with pulmonary metastasis.
Case report and systematic review of spinal EHE.
A 36-year-old man presented with bilateral lower extremity weakness, progressive paresthesia, and urinary incontinence. He underwent open surgical resection of the tumor and decompression of the spinal cord, with subsequent improvement in neurologic function. Systematic review identified 84 cases of spinal EHE, 73 of which were primary, and 14 of which developed extra-spinal metastases.
EHE is an exceedingly rare tumor that may present with a wide swath of clinical symptoms. At present, no guidelines or formal treatment recommendations have been established. Surgical debulking has demonstrated efficacy as a front-line treatment, particularly in the setting of compressive neurologic dysfunction; data regarding adjuvant chemoradiation are less consistently reported, mandating further study.
上皮样血管内皮细胞瘤（EHE）是一种罕见的血管肿瘤，主要发生在软组织中。我们报告了一例罕见的胸段脊柱 EHE 合并肺转移病例。
病例报告并对脊柱 EHE 进行系统回顾。
一名 36 岁男性因双下肢无力、进行性感觉异常和尿失禁就诊。他接受了肿瘤的开放性手术切除和脊髓减压，随后神经功能得到改善。系统回顾共确定了 84 例脊柱 EHE 病例，其中 73 例为原发性，14 例发生了脊柱外转移。
EHE 是一种非常罕见的肿瘤，可能表现出广泛的临床症状。目前，尚无指南或正式的治疗建议。手术切除已被证明是一种有效的一线治疗方法，特别是在存在压迫性神经功能障碍的情况下；关于辅助放化疗的数据报道较少，需要进一步研究。
