Arch Pathol Lab Med. 2018 Feb;142(2):263-267. doi: 10.5858/arpa.2016-0171-RS.
Epithelioid hemangioendothelioma is a rare vascular tumor, composed of epithelioid and histiocytoid vascular endothelial cells in myxoid or fibrotic stroma, which can arise in multiple locations throughout the body. In the liver, this neoplasm usually presents on imaging as an incidental finding of multifocal, heterogeneously enhancing nodules in both lobes or presents clinically with nonspecific abdominal symptoms. Histologically, the tumor has been mistaken for metastatic carcinoma, angiosarcoma, hepatocellular carcinoma, and cholangiocarcinoma. The neoplasm usually stains positive for vascular markers, such as factor VIII-related antigen, CD31, and CD34, and negative for cytokeratins. The translocation t(1;3)(p36.3;q25), resulting in the CAMTA1- WWTR1 fusion product, is the most commonly identified genetic abnormality with this tumor. Although hepatic epithelioid hemangioendothelioma can have a varied clinical course, it is generally considered less aggressive than angiosarcoma. There is no consensus treatment protocol and techniques including liver transplantation, liver resection, chemotherapy and/or radiation therapy, and surveillance have all been used with varying outcomes.
上皮样血管内皮细胞瘤是一种罕见的血管肿瘤，由黏液样或纤维性基质中的上皮样和组织细胞样血管内皮细胞组成，可发生在全身多个部位。在肝脏中，这种肿瘤通常在影像学上表现为多灶性、异质性增强结节，位于两叶，或者临床上表现为非特异性腹部症状。组织学上，该肿瘤易误诊为转移性癌、血管肉瘤、肝细胞癌和胆管细胞癌。该肿瘤通常对血管标志物呈阳性染色，如因子 VIII 相关抗原、CD31 和 CD34，而对细胞角蛋白呈阴性。该肿瘤最常见的遗传异常是 t(1;3)(p36.3;q25)，导致 CAMTA1-WWTR1 融合产物。虽然肝上皮样血管内皮细胞瘤的临床过程可能不同，但它通常被认为比血管肉瘤侵袭性小。目前尚无共识的治疗方案，包括肝移植、肝切除术、化疗和/或放疗以及监测在内的各种技术都已被用于治疗，结果各不相同。
