Jirasirinuphan Phantira, Chang Amber L, Deepak Ananya, Chang Ching Kuo
University of California, Davis, CA, USA.
Kaiser-Permanente, Oakland, CA, USA.
AME Case Rep. 2023 Jan 16;7:1. doi: 10.21037/acr-22-57. eCollection 2023.
Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of the blood vessels. We report a patient with vascular EHE with delayed pulmonary metastasis, of which there are no previously known case reports.
A 40-year-old female presents with a painful right groin mass and swelling in the lower extremity. A 3.8 cm soft tissue mass was identified in the femoral sheath with the abutment of the femoral artery on a computerized tomography (CT) scan. Surgical resection of the femoral vein was performed, and the final pathology confirmed a diagnosis of EHE. A second resection of the femoral artery with bypass grafting was performed with clear surgical margins. The patient did well post-operatively with annual surveillance of the right groin as well as chest X-rays. However, the patient developed a metastatic Epithelioid hemangioendothelioma (EHE) to the right lower lobe, 12 years after the initial EHE treatment. She underwent a pulmonary resection with clear margins. The patient remains disease-free after one year. EHE is a rare soft tissue sarcoma with unpredictable clinical behavior. While most commonly presenting in the lung and liver they can also originate from any vascular system. Delayed pulmonary metastasis from vascular EHE has not been reported.
Our case shows that indolent metastasis can occur in EHE, despite a prolonged disease-free interval. This case highlights the need for long-term surveillance with serial imaging of not only the primary site but pulmonary imaging beyond 5 years may be beneficial.
上皮样血管内皮瘤（EHE）是一种罕见的血管肉瘤。我们报告一例血管型EHE患者发生延迟性肺转移，此前尚无已知病例报告。
一名40岁女性，出现右侧腹股沟疼痛性肿块及下肢肿胀。计算机断层扫描（CT）显示股鞘内有一个3.8 cm的软组织肿块，股动脉受压。对股静脉进行了手术切除，最终病理确诊为EHE。再次对股动脉进行切除并进行旁路移植，手术切缘清晰。患者术后情况良好，每年对右侧腹股沟及胸部X线进行监测。然而，在最初治疗EHE 12年后，患者右肺下叶出现转移性上皮样血管内皮瘤（EHE）。她接受了切缘清晰的肺切除术。一年后患者仍无疾病复发。EHE是一种罕见的软组织肉瘤，临床行为不可预测。虽然最常出现在肺和肝脏，但也可起源于任何血管系统。血管型EHE的延迟性肺转移尚未见报道。
我们的病例表明，尽管无病间期延长，EHE仍可发生惰性转移。该病例强调不仅需要对原发部位进行长期的连续影像学监测，而且5年以上的肺部影像学检查可能也有益处。
