Wang Zhan-Bo, Yuan Jing, Chen Wei, Wei Li-Xin
Zhan-Bo Wang, Jing Yuan, Wei Chen, Li-Xin Wei, Department of Pathology, Chinese People's Liberation Army General Hospital, Beijing 100853, China.
World J Gastroenterol. 2014 Apr 7;20(13):3672-9. doi: 10.3748/wjg.v20.i13.3672.
To investigate the expression of ERG, CD34, CD31 (PECAM-1, platelet/endothelial cell adhesion molecule 1) and factor VIII-related antigen (FVIIIRAg) in the diagnosis of hepatic angiosarcoma patients.
Patient samples were collected from January 1986 to December 2012 from the People's Liberation Army General Hospital in Beijing, China. We obtained twenty-four samples of hepatic angiosarcoma (HAS) that were confirmed by two pathologist. The samples were the result of three autopsy cases, eight biopsy cases and 13 patients who underwent surgical tumor removal. The HAS cases accounted for 2.23% (24/1075) of all hepatic vascular tumors at the hospital during the same time period. Patient histories including age, gender, clinical manifestations, medical treatments, laboratory tests, radiological images, histological observations and outcomes for each case were analyzed in detail. All samples were evaluated histologically with hematoxylin and eosin staining. Using immunohistochemistry, the expression and localization of ERG was examined in all HAS specimens and compared to the known endothelial markers CD34, CD31 and FVIIIRAg. The endothelial markers were also evaluated in a panel of non-HAS tumors.
This cohort of 24 HAS cases is, to the best of our knowledge, currently the largest cohort in the world in the publicly available literature. Hepatic angiosarcoma tissue samples were obtained from 14 males and 10 females with a mean age of 50.6 years (range: 7-86 years). The patients presented with the following clinical manifestations: abdominal pain (16/24), back pain (3/24), heart palpitations (1/20), cough (1/24) or no clinical symptoms (3/24). Tumors were predominantly localized in the right hepatic lobe (15/24) or left hepatic lobe (6/24), or a diffuse growth on the right and left hepatic lobes (3/24). Eleven patients underwent surgical resection (45.8%), two patients received a liver transplant (8.3%), eight patients received interventional therapy (33.3%) and three patients received no treatment (lesions discovered at autopsy, 12.5%). Postoperative follow-up of patients revealed that 87.5% (21/24) of patients had died and three cases were not able to be tracked. In all cases, the mean survival time was 12.1 mo. While 100% of the HAS samples were positive for ERG expression, expression of the other markers was more variable. CD31 was expressed in 79.2% (19/24) of samples, CD34 was expressed in 87.5% (21/24) of samples and FVIIIRAg was expressed in 41.7% (10/24) of samples.
ERG is a more sensitive and specific diagnostic marker for hepatic angiosarcoma in comparison to CD31, CD34 and FVIIIRAg.
研究ERG、CD34、CD31（血小板/内皮细胞黏附分子1，PECAM-1）和因子VIII相关抗原（FVIIIRAg）在肝血管肉瘤患者诊断中的表达情况。
1986年1月至2012年12月期间，从中国北京解放军总医院收集患者样本。我们获取了24例经两名病理学家确诊的肝血管肉瘤（HAS）样本。这些样本来自3例尸检病例、8例活检病例以及13例接受手术切除肿瘤的患者。在同一时期，HAS病例占该医院所有肝血管肿瘤的2.23%（24/1075）。详细分析了每个病例的患者病史，包括年龄、性别、临床表现、治疗情况、实验室检查、影像学图像、组织学观察及预后。所有样本均采用苏木精-伊红染色进行组织学评估。运用免疫组织化学方法，检测所有HAS标本中ERG的表达及定位，并与已知的内皮细胞标志物CD34、CD31和FVIIIRAg进行比较。同时，也在一组非HAS肿瘤中评估了这些内皮细胞标志物。
据我们所知，这组24例HAS病例是目前公开文献中世界上最大的队列。肝血管肉瘤组织样本来自14名男性和10名女性，平均年龄为50.6岁（范围：7 - 86岁）。患者表现出以下临床表现：腹痛（16/24）、背痛（3/24）、心悸（1/20）、咳嗽（1/24）或无临床症状（3/24）。肿瘤主要位于右肝叶（15/24）或左肝叶（6/24），或在左右肝叶呈弥漫性生长（3/24）。11例患者接受了手术切除（45.8%），2例患者接受了肝移植（8.3%），8例患者接受了介入治疗（33.3%），3例患者未接受治疗（尸检时发现病变，12.5%）。对患者的术后随访显示，87.5%（21/24）的患者已死亡，3例无法追踪。所有病例的平均生存时间为12.1个月。虽然100%的HAS样本ERG表达呈阳性，但其他标志物的表达则更具变异性。79.2%（19/24）的样本表达CD31，87.5%（21/24）的样本表达CD34，41.7%（10/24）的样本表达FVIIIRAg。
与CD31、CD34和FVIIIRAg相比，ERG是肝血管肉瘤更敏感、更特异的诊断标志物。
