一名71岁男性因低烧、腹痛被转诊至我院。他已接受甲氨蝶呤（MTX）治疗类风湿性关节炎6年多，但入院后因肝功能迅速恶化而停止服用MTX。计算机断层扫描（CT）显示多个肝脏病变并伴有晚期强化，高度提示它们是胆管癌。除了 PIVKA-II 水平略有升高（即 45 mAU/mL（范围 0-40 mAU/mL））外，肿瘤标志物水平正常。我们对最大的病变进行了活检，并进行了内镜下胆道引流，以分别对肝脏病变进行明确诊断并治疗黄疸。病理学研究显示圆形、多边形和纺锤形上皮非典型细胞以肉瘤样方式生长。非典型细胞CD31、CD34、波形蛋白和TFE3呈阳性，其中一些细胞内有空泡，从而诊断为肝脏上皮样血管内皮瘤（EHE）。内镜胆道引流术后4周，患者康复。 CT显示胆道引流后3个月EHE病变明显消退，12个月后完全消退。胆道引流后23个月，患者在主动脉旁淋巴结进一步发展为霍奇金淋巴瘤，目前正在接受恶性淋巴瘤的化疗。然而，在确认 EHE 病变完全消退后至少 16 个月内，我们尚未在肝脏或远处器官中检测到任何 EHE 病变。肿瘤学家应注意 EHE 的自发消退，并研究 MTX 停用与 EHE 消退之间的相关性。© 2023 作者。由巴塞尔 S. Karger AG 出版。

A 71-year-old man with slight fever and dull abdominal pain was referred to our hospital. He had been receiving methotrexate (MTX) to treat his rheumatoid arthritis for more than 6 years but stopped taking MTX after admission due to the rapid aggravation of his liver function. Computed tomography (CT) showed multiple liver lesions with late enhancement, highly suggesting them to be cholangiocarcinomas. Tumor marker levels were normal except for a slightly elevated PIVKA-II level, i.e., 45 mAU/mL (range 0-40 mAU/mL). We did a biopsy to the largest lesion and endoscopic biliary drainage to make a definitive diagnosis of the hepatic lesions and treat jaundice, respectively. Pathological study showed round, polygonal, and spindle-shaped epithelial atypical cells growing in a sarcomatoid fashion. Atypical cells were positive for CD31, CD34, vimentin, and TFE3, and some of them had intracellular vacuoles, leading to the diagnosis of epithelioid hemangioendothelioma (EHE) of the liver. The patient got well 4 weeks after the endoscopic biliary drainage. CTs showed marked regression of the EHE lesions 3 months after biliary drainage and complete regression in 12 months. The patient further developed Hodgkin lymphoma in the para-aortic lymph nodes 23 months after the biliary drainage and is now under chemotherapy for the malignant lymphoma. We, however, have not detected any EHE lesions in the liver or distant organs for at least 16 months after the confirmation of complete regression of the EHE lesions. Oncologists should note the spontaneous regression of the EHE and investigate the correlation between MTX cessation and EHE regression.© 2023 The Author(s). Published by S. Karger AG, Basel.