肝上皮样血管内皮瘤临床病理分析和预后探讨  

Clinicopathological features and prognosis of hepatic epithelioid hemangioendothelioma

作　　者：苏杰 黄斌[2] 吴晨霞 姜春婷 崔海宏[3] 王湛博[4] 张宝燕 SU Jie;HUANG Bin;WU Chen-xia;JIANG Chun-ting;CUI Hai-hong;WANG Zhan-bo;ZHANG Bao-yan(Department of Pathology,903 Hospital of PLA,Hangzhou 310013,China;Department of Pathology,Xiaoshan 1st People’s Hospital,Hangzhou 311200,China;Department of Gastroenterology,305 Hospital of PLA,Beijing100017,China;Department of Pathology,PLA General Hospital,Beijing100853,China)

机构地区：[1]联勤保障部队第九○三医院病理科,浙江杭州310013 [2]杭州市萧山区第一人民医院病理科,浙江杭州311200 [3]中国人民解放军第三○五医院消化内科,北京100017 [4]解放军总医院病理科,北京100853

出　　处：《诊断病理学杂志》2021年第7期536-540,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨肝上皮样血管内皮瘤(HEHE)的临床病理特点和其预后意义以及治疗方案。方法回顾性分析4例HEHE病例的临床病理学特点、治疗方案及长期随访结果。结果本组男性3例、女性1例;年龄均<40岁;2例临床表现为右上腹胀痛,2例体检B超发现;3例影像学示肝内多发病灶、1例单发病灶;肺部累及有2例;HBsAg(-)3例、1例小三阳。镜下瘤细胞上皮样或树突状,交织成片,可见胞质空泡,内含红细胞;间质疏松纤维性,可见纤维化或黏液变区;免疫组化染色CD31、CD34和F8因子均弥漫(+)。观察各组织病理学参数与疾病转归,提示瘤细胞显著上皮样、核分级高、核分裂数>10个/50HPF以及有肿瘤性坏死、脉管瘤栓是预后不利因素。治疗上1例接受肝动脉栓塞化疗及伽马刀治疗,随访125个月失访;1例行肝段切除、热化疗、伽马刀及无水乙醇注入等治疗,25个月后死亡;1例未治疗,生存126个月因肺结核死亡;1例给予沙利度胺、干扰素等治疗,随访35个月生存。结论 HEHE罕见,转归差异大可能与肿瘤异质性相关。瘤细胞显著上皮样、核异型大、分裂象易见、有肿瘤性坏死及脉管瘤栓者有转归不良倾向。治疗上无国际通用标准,可行手术切除或肝移植治疗,肝动脉栓塞化疗、伽马刀及沙利度胺、干扰素等治疗也可使患者获益。Objective To investigate the histopathological feature,its prognostic value and treatment modalities of hepatic epithelioid hemangioendothelioma(HEHE).Methods The histopathological parameters,treatments and long term follow-up data were collected of 4 HEHE cases from our hospitals.Results Patients were 3 males and 1 female,aged 21-39 years.2 cases presented with right upper quadrant pain,2 incidental finding.Liver lesions were multiple(3 cases)or solitary(1 case),with lung involved in 2 cases.Laboratory tests showed hepatitis B surface antigen positive in 1 case.Histologically,tumor cells were dendritic and/or epithelioid,in an interdigitating and/or clustered form.Intracytoplasmic vacuoles containing erythrocytes were frequently observed.Stroma was loosely fibrous,with foci of hyalinosis and myxoid degeneration.Immunohistochemically,tumor cells were positive for CD31,CD34 and F8.Histological parameters including mainly distinguished epithelioid tumor cells,high nuclear grade,>10 mitoses/50 HPF,areas of necrosis and tumor thrombosis were suggestive of unfavorable outcome.Treatment modalities and follow-up data were as follows:1 patient treated with transcatheter arterial embolization(TAE)and gamma knife,lost follow-up at 125 months;1 with surgical excision,chemotherapy,gamma knife and alcohol infusion,died 25 months later;1 with no treatment,died of lung tuberculosis at last follow-up at 126 months;1 with cycles of thalidomide and interferon,was alive and well at last follow-up at 35 months.Conclusion HEHE is rare.The prognosis is variable and may be due to tumor heterogeneity.Distinguished epithelioid tumor cells,striking nuclear atypia,>10 mitoses/50 HPF,areas of necrosis and tumor thrombosis tend to be related to poor outcome.There is no consensus treatment.Surgical excision is recommended for resectable lesions,with liver transplantation for unresectable ones.TAE,gamma knife treatment,thalidomide and interferon may also be acceptable.

关 键 词：上皮样血管内皮瘤 肝脏肿瘤 临床病理 预后 

分 类 号：R730.261[医药卫生—肿瘤]