Kanno Hiroki, Sato Toshihiro, Midorikawa Ryuta, Kojima Satoki, Fukutomi Shogo, Goto Yuichi, Nomura Yoriko, Yoshitomi Munehiro, Kawahara Ryuichi, Sakai Hisamune, Hisaka Toru, Akagi Yoshito, Okuda Koji
Department of Surgery, Kurume University School of Medicine, Kurume, Japan.
Case Rep Gastroenterol. 2021 Mar 11;15(1):344-351. doi: 10.1159/000513803. eCollection 2021 Jan-Apr.
Hepatic epithelioid hemangioendothelioma (EHE) is a rare malignant tumor with unknown pathogenesis. Herein, we report a case of a hepatic EHE presenting synchronously with a hepatocellular carcinoma (HCC). To the best of our knowledge, this is the second case report of synchronous hepatic EHE and HCC. An 84-year-old man presented with back pain. During examination, a tumor in liver segment 3 was coincidentally detected. Tumor marker (carbohydrate antigen 19-9, alpha-fetoprotein, and protein induced by vitamin K absence or antagonist-II) levels were elevated. Contrast-enhanced computed tomography revealed perinodular enhancement in the arterial and portal phases. Another tumor was detected in liver segment 2, which was homogeneously enhanced in the arterial phase, followed by washout in the portal and late phases. Based on these imaging findings, we diagnosed the tumor in segment 3 as a solitary cholangiocellular carcinoma and the tumor in segment 2 as a solitary HCC. Lateral sectionectomy of the liver was performed. Microscopically, spindle-shaped and epithelioid cells were present in the tumor in segment 3. On immunohistochemistry, the tumor cells were positive for CD31 and CD34, focally positive for D2-40, and negative for AE1/AE3. Therefore, the tumor in segment 3 was ultimately diagnosed as an EHE and the tumor in segment 2 as a well-differentiated HCC. Preoperative diagnosis of EHE is difficult owing to the lack of specific findings. Intratumoral calcification, halo sign, and lollipop sign are occasionally found in EHE and are useful imaging findings for diagnosis. Clinical behavior is unpredictable, ranging from indolent growth to rapid progression. Clinical or pathological predictors of the course of EHE are urgently required.
肝上皮样血管内皮瘤（EHE）是一种发病机制不明的罕见恶性肿瘤。在此，我们报告一例肝EHE与肝细胞癌（HCC）同时出现的病例。据我们所知，这是第二例肝EHE与HCC同时出现的病例报告。一名84岁男性因背痛就诊。检查期间，偶然发现肝3段有一个肿瘤。肿瘤标志物（糖类抗原19-9、甲胎蛋白和维生素K缺乏或拮抗剂-II诱导蛋白）水平升高。对比增强计算机断层扫描显示动脉期和门脉期结节周围强化。在肝2段发现另一个肿瘤，动脉期均匀强化，随后在门脉期和延迟期廓清。基于这些影像学表现，我们将3段的肿瘤诊断为孤立性胆管细胞癌，2段的肿瘤诊断为孤立性HCC。进行了肝外侧段切除术。显微镜下，3段肿瘤中可见梭形和上皮样细胞。免疫组织化学检查显示，肿瘤细胞CD31和CD34阳性，D2-40局灶性阳性，AE1/AE3阴性。因此，3段的肿瘤最终诊断为EHE，2段的肿瘤诊断为高分化HCC。由于缺乏特异性表现，EHE的术前诊断困难。EHE偶尔会出现瘤内钙化、晕征和棒棒糖征，这些都是有助于诊断的影像学表现。其临床行为不可预测，从生长缓慢到快速进展不等。迫切需要EHE病程的临床或病理预测指标。
