以腹痛为主罕见腹腔内多发上皮样血管内皮细胞瘤1例并文献复习

A Rare Case of Epithelioid Hemangioendothelioma in Abdominal Cavity with Abdominal Pain and Literature Review

目的:探讨上皮样血管内皮细胞瘤(epithelioid hemangioendothelioma,EHE)在腹腔内发病的临床表现及病理特点,以提高该病的诊疗水平。方法:分析笔者所在医院收治的1例腹腔内多发上皮样血管内皮细胞瘤患者的诊疗情况结合文献复习,阐释该病的临床表现、诊断、鉴别诊断、治疗等。结果:行"剖腹探查+腹腔结节活检术",取肠道上结节2块,肝脏结节1块送检。送检肠道组织(1、2)免疫组化示:Vimentin(+)、CD34(+)、CD31(+)、F8(+)、Fli-1(+)、Caldesmon(+);送检肝脏组织活检示:送检为少许肝脏组织,部分组织变性。经免疫组化,明确诊断肠道送检组织为上皮样血管内皮细胞瘤。结论:上皮样血管内皮细胞瘤在腹腔内广泛发病的病例极其罕见,且以"腹痛"为主要症状又极易误诊,当影像学及各项辅助检查排除其他病因所致腹痛后应警惕该病发生的可能性。

Objective:To investigatthe clinical and pathological features of epithelioid hemangioendothelioma(EHE) in abdominal cavity in order to improve its diagnosis and treatment level.Method:Analyzed a case of diagnosis and treatment in multiple intraperitoneal epithelium-like vascular endothelial cell tumor in our hospital.The clinical manifestation,diagnosis,differential diagnosis and treatment of the disease were explained with the help of literature review.Result:"Exploratory laparotomy+peritoneal nodule biopsy" was performed.2 lumps of upper intestine and 1 lumpy of liver were taken for examination.Dispatch of intestinal tissue(1,2) immunohistochemistry:Vimentin(+),CD34(+),CD31(+),F8(+),Fli-1(+),Caldesmon(+).Liver biopsy showed that a little liver tissue,partial tissue degeneration.Immunohistochemical method showed epithelioid hemangioendothelioma.Conclusion:Epidermal vascular endothelial cell tumor is very rare in the abdominal cavity,and the first symptom of abdominal pain is often misdiagnosed.When imaging and other auxiliary examinations exclude other causes of abdominal pain,we should be alert to the possibility of the disease.