Section of Histopathology, Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan.
Department of Pathology and Clinical Laboratory, French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan.
Diagn Pathol. 2020 Sep 25;15(1):120. doi: 10.1186/s13000-020-01039-2.
Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature.
All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained.
Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon.
Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.
肝上皮样血管内皮细胞瘤是一种极其罕见的恶性血管肿瘤，通常为多灶性，且在许多情况下为偶然发现。在此，我们描述了在我院实践中所见的肝上皮样血管内皮细胞瘤病例的临床病理特征，并对已发表的文献进行了详细回顾。
本研究纳入了 2006 年 1 月 1 日至 2019 年 12 月 31 日期间在阿克巴汗大学医院病理与检验科诊断为肝上皮样血管内皮细胞瘤的所有病例。我们复习了切片并获取了随访信息。
在研究期间报告了 7 例病例。其中 4 例为女性，3 例为男性。年龄范围为 20 岁至 77 岁，平均年龄为 45 岁。3 例患者表现为右上腹疼痛；1 例患者表现为黄疸，而 3 例患者无症状。所有 7 例患者的影像学检查均发现了病变。5 例患者的肝病变为多灶性。所有病例的临床鉴别诊断均为转移性癌和多灶性肝细胞癌。5 例患者的肝功能检查正常。1 例患者的肿瘤已转移至右肺。对所有 7 例患者进行的肝芯活检的组织学检查显示出经典的上皮样血管内皮细胞瘤特征。肿瘤细胞表达血管标志物（CD34、CD31 和 ERG）阳性，细胞角蛋白、Hep par 1 和 Glypican 3 阴性。7 例患者均未进行手术切除，所有患者均接受化疗。5 例患者获得随访。其中，3 例患者死于疾病，另 1 例患者存活，但肺部、大网膜和结肠均有转移。
本研究报告了 7 例病例的临床病理特征，并对已发表文献进行了详细回顾。我们的病例预后较差，这很可能是由于该国大部分地区缺乏肝脏肿瘤手术的专业知识，因此无法进行手术切除。
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