Departments of Pathology, Carol Davila University of Medicine and Pharmacy, and Fundeni Clinical Institute, Bucharest, Romania;
Rom J Morphol Embryol. 2022 Jan-Mar;63(1):197-202. doi: 10.47162/RJME.63.1.21.
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor and the mediastinal localization is amongst the most infrequent. We present the case of a 37-year-old woman with a history of resected left thyroid tumor that presented to our department for evaluation of a left supraclavicular palpable mass in close contact with local vascular structures, and with heterogeneous contrast enhancement as described by computed tomography (CT) and magnetic resonance imaging (MRI). Considering the history of the patient, the presumptive diagnosis of thyroid tumor recurrence was established, and the patient was referred to surgical department. During procedure, we encountered important bleeding from a ruptured jugular vein branch, which we assumed to be a newly formed tumor blood vessel. After surgery (48 hours postoperatively), the patient developed important local thrombosis that encompassed the left internal jugular vein, left subclavian vein and the left brachiocephalic trunk that partially subsided after anticoagulant therapy. The histological examination revealed the presence of a vascular tumor proliferation of epithelioid endothelial cells that was characteristic of an EHE confirmed later on the immunohistochemical studies as Yes-associated protein 1-transcription factor E3 (YAP1-TFE3) subtype. In addition to the case report, some relevant information from the scarce literature data about mediastinal EHE were reviewed here.
上皮样血管内皮细胞瘤（EHE）是一种罕见的血管肿瘤，纵隔定位是最不常见的。我们报告了一例 37 岁女性的病例，该患者有左甲状腺肿瘤切除史，因左锁骨上可触及的肿块就诊，该肿块与局部血管结构密切接触，且计算机断层扫描（CT）和磁共振成像（MRI）显示不均匀增强。考虑到患者的病史，我们初步诊断为甲状腺肿瘤复发，并将患者转至外科。在手术过程中，我们遇到了颈内静脉分支破裂引起的大量出血，我们认为这是新形成的肿瘤血管。手术后（术后 48 小时），患者出现了重要的局部血栓形成，包括左颈内静脉、左锁骨下静脉和左头臂干，抗凝治疗后部分缓解。组织学检查显示存在上皮样内皮细胞的血管肿瘤增殖，这是 EHE 的特征，随后免疫组织化学研究证实为 Yes 相关蛋白 1-转录因子 E3（YAP1-TFE3）亚型。除了病例报告外，我们还回顾了一些关于纵隔 EHE 的罕见文献数据中的相关信息。
