原发性肝上皮样血管内皮瘤 (HEHE) 是一种极其罕见的血管源性肿瘤，全球发病率<0.1例/10万人。 一名 29 岁女性，有上腹痛病史和无意体重减轻（六个月内体重减轻 3 公斤） ）被转诊进行上消化道内窥镜检查。检查没有肉眼病理发现，但快速尿素酶试验呈阳性。一线治疗采用含克拉霉素三联疗法治疗幽门螺杆菌感染。根除治疗完成后，出现弥漫性腹痛。腹部计算机断层扫描（CT）显示多个肝脏结节。连续进行了三次核心肝活检，但没有得出结论。随后进行了肝结节切除术。标本的组织病理学显示为 2 级肝细胞癌；骨闪烁扫描显示转移呈阴性。多学科小组（MDT）建议患者服用索拉非尼，但该药物的耐受性较差。应肿瘤学家的要求，使用免疫组织化学染色检查组织学，结果显示 CD31 和 CD34 的表达。根据临床、形态学和免疫组织化学检查结果，诊断为肝上皮样血管内皮瘤。根据多学科团队的研究结果，肝移植被认为是唯一的治疗方法。由于这种疾病的罕见性，结合临床、放射学和组织病理学方法以及MDT方法可以帮助达成正确的最终诊断。正如该临床病例所证明的，进行肝活检的免疫组织化学检查以确认 HEHE 诊断至关重要。肝上皮样血管内皮瘤是一种罕见的血管肿瘤，经常被误诊和处理不当。该病例强调了跨学科团队合作和使用的至关重要性使用非侵入性和侵入性技术来实现明确诊断。© EFIM 2024。

Primary hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare tumour of vascular origin with an incidence of <0.1 cases per 100,000 people worldwide.A 29-year-old female with the history of epigastric pain and unintentional weight loss (3 kg over six months) was referred for upper endoscopy. The examination was without visual pathological findings, but a rapid urease test was positive. First-line treatment with clarithromycin-containing triple therapy for Helicobacter pylori infection was given. After completion of eradication therapy, diffuse abdominal pain developed. An abdominal computed tomography (CT) showed multiple liver nodules. Three consecutive core liver biopsies were performed and were inconclusive. A subsequent surgical liver nodule resection was performed. Histopathology of the specimen revealed grade 2 hepatocellular carcinoma; bone scintigraphy was negative for metastasis. A multidisciplinary team (MDT) recommended giving the patient sorafenib, which was poorly tolerated. The histology was reviewed using immunohistochemistry staining at the request of the oncologist, which showed expression of CD31 and CD34. Based on clinical, morphological and immunohistochemistry findings, a diagnosis of hepatic epithelioid hemangioendothelioma was made. Based on the multidisciplinary team's findings, liver transplantation was indicated as the only curative treatment.Because of the rarity of this disease, combining clinical, radiological and histopathological methods as well as an MDT approach can help to reach the correct final diagnosis. As demonstrated in this clinical case, it is crucial to perform immunohistochemistry of a liver biopsy to confirm a HEHE diagnosis.Hepatic epithelioid hemangioendothelioma is a rare vascular tumour that is often misdiagnosed and mismanaged.This case emphasises the critical importance of interdisciplinary teamwork and the use of non-invasive and invasive techniques to achieve a definitive diagnosis.© EFIM 2024.