Liang Pan, Zhu Bing-Bing, Ren Xiu-Chun, Gao Jian-Bo
Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Front Oncol. 2022 May 30;12:713947. doi: 10.3389/fonc.2022.713947. eCollection 2022.
Primary spinal epithelioid hemangioendothelioma (SEHE) is an extremely rare angiocentric vascular neoplasm with inherent metastatic potential, which pursues a clinical course intermediate between hemangioma and angiosarcoma. The present study sought to present computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical features of primary SEHE and review the literature.
Clinical data of four patients with primary SEHE confirmed by pathology from January 2011 to May 2020 were analyzed retrospectively. Two cases underwent CT scan, while 3 cases underwent MRI scan. Image observation included the tumor location, bone changes, morphology, density/signal characteristics, and enhanced features.
The four patients were all women who ranged in age from 25 to 74 years. Four cases of the lesions were in the vertebral body; among them, two cases involved the accessory of the vertebra. Soap bubble appearance was detected in 2 cases, honeycomb osteolytic appearance in 2 cases, a sclerotic margin in 3 cases, and accompanying vertebral compression fracture in 1 case. CT showed a slightly inhomogeneous low density with punctate high-density foci. MRI showed an inhomogeneous low signal on T-weighted image (TWI) and a high signal on TWI. Contrast-enhanced CT/MRI demonstrated moderate to intensive inhomogeneous enhancement of the lesions. The discs were normal. In one case, lesions presented a dural tail sign.
Primary SEHE is so rare in the clinic as an aggressive vascular tumor. The relatively typical clinical features and radiographic findings can help in preoperative diagnosis.
原发性脊柱上皮样血管内皮瘤（SEHE）是一种极为罕见的具有固有转移潜能的血管中心性血管肿瘤，其临床病程介于血管瘤和血管肉瘤之间。本研究旨在呈现原发性SEHE的计算机断层扫描（CT）和磁共振成像（MRI）表现及临床特征，并复习相关文献。
回顾性分析2011年1月至2020年5月间经病理确诊的4例原发性SEHE患者的临床资料。2例行CT扫描，3例行MRI扫描。图像观察包括肿瘤位置、骨质改变、形态、密度/信号特征及强化特点。
4例患者均为女性，年龄25至74岁。4例病变均位于椎体，其中2例累及椎附件。2例表现为皂泡样外观，2例为蜂窝状溶骨性外观，3例有硬化边缘，1例伴有椎体压缩骨折。CT显示密度略不均匀，有散在点状高密度灶。MRI在T加权像（TWI）上呈不均匀低信号，在TWI上呈高信号。增强CT/MRI显示病变呈中度至明显不均匀强化。椎间盘正常。1例病变出现硬膜尾征。
原发性SEHE在临床上作为一种侵袭性血管肿瘤极为罕见。相对典型的临床特征和影像学表现有助于术前诊断。
