Pathological features of hepatic epithelioid hemangioendothelioma: An analysis of 5 cases
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摘要:
目的探讨肝上皮样血管内皮细胞瘤(HEHE)的临床病理特点。方法对5例HEHE患者的临床资料、组织病理学、免疫组化结果进行观察。结果 5例HEHE患者中,男3例,女2例。主要临床表现为持续腹部不适、腹痛、恶心、体质量下降、发热等,其中1例为体检时发现。2例为单结节,3例为多结节。肿瘤细胞形态较温和,排列呈条索状、小巢状,细胞圆形、梭形或不规则形,有丰富上皮样的嗜酸性胞质,常见分枝状胞浆突起,胞质内常见空泡形成,肿瘤背景为具有特征性的黏液样透明变性的软骨样基质或玻璃样变性的纤维基质。偶见核分裂和坏死。瘤细胞表达CD31、CD34、FLi-1、FⅧ、血管内皮生长因子等血管源性标志物,增殖活性低。结论 HEHE的临床表现及治疗手段多种多样,肝穿刺活组织检查不仅有助于确诊,也能够对HEHE治疗方案的选择发挥积极作用。
Abstract:Objective To investigate the clinicopathological features of hepatic epithelioid hemangioendothelioma ( HEHE) . Methods The clinical data, histopathological data, and immunohistochemical results of 5 patients with HEHE were observed. Results There were 3 male and 2 female patients. Major clinical manifestations included persistent abdominal discomfort, abdominal pain, nausea, loss of weight, and pyrexia. One patient was found to have this disease during physical examination. Of all patients, 2 had a single nodule and 3 had multiple nodules. The tumor cells were relatively normal in morphology and were arranged in a cord-like shape or a small nested shape; the cells had a round, spindle-like, or irregular shape; the cells were rich in epithelioid eosinophilic cytoplasm, with branched cytoplasmic processes and cavity formation in cytoplasm. The tumor background was characteristic mucoid hyaline degeneration of cartilage-like matrix or glassy degeneration of fibrous matrix. Nuclear division and necrosis were observed occasionally. Vascular markers, such as CD31, CD34, Fli-1, coagulation factor VIII, and vascular endothelial growth factor, were expressed in tumor cells, and tumor cells tended to have a low proliferative activity. Conclusion HEHE has various clinical manifestations and treatment methods. Liver biopsy helps to make a confirmed diagnosis and select a proper treatment regimen before treatment.
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