Li Hang, Shah Deepa, Shah Abhishek, Qiu Xiang, Cao Dianbo
Department of Radiology, The First Hospital of Jilin University, Changchun 130021, Jilin Province, PR China.
Department of Internal Medicine, China-Japan Union Hospital of Jilin University, Changchun 130021, Jilin Province, PR China.
Int J Surg Case Rep. 2014;5(1):19-21. doi: 10.1016/j.ijscr.2013.09.019. Epub 2013 Nov 26.
Malignant epithelioid hemangioendothelioma (EHE) is an uncommon and grave vascular tumor. EHE is frequently angiocentric and is associated with a medium sized vessel, especially a vein. No definite etiological associations have been ascribed to this tumor so far, except an association with oral contraceptives in EHE of liver.
A 47 year old man presented with the complaint of intermittent black stool over the past two weeks. Occasionally, he experienced pain in left lower abdomen. On Computed Tomography (CT), it showed hypervascular lesion in the ileum with persistent enhancement. An exploratory laparotomy was performed with short segmental resection and functional end-to-end anastomosis. It was diagnosed finally with the histopathological and immunohistochemical analysis as a malignant EHE.
EHE is an uncommon endothelial tumor that most frequently arises in soft tissue, liver, lung and skeleton. It behaves biologically in between benign epithelioid hemangioma and the more aggressive epithelioid angiosarcoma. Although a standard systemic treatment for malignant EHE has not been fully established, complete surgical excision is strongly recommended if feasible.
EHE has a variable presentation and CT is helpful in identifying ileal EHE timely in the early stage, even when there is no obvious mass formation, however the diagnosis can be confirmed only after histopathological and immunohistochemical studies.
恶性上皮样血管内皮瘤(EHE)是一种罕见且严重的血管肿瘤。EHE常以血管为中心,与中等大小血管相关,尤其是静脉。到目前为止,除了肝EHE与口服避孕药有关外,尚未明确该肿瘤的病因关联。
一名47岁男性,主诉过去两周间歇性黑便。偶尔,他会感到左下腹疼痛。计算机断层扫描(CT)显示回肠有高血运病变且持续强化。进行了剖腹探查术,行短节段切除及功能性端端吻合。最终经组织病理学和免疫组织化学分析诊断为恶性EHE。
EHE是一种罕见的内皮肿瘤,最常发生于软组织、肝脏、肺和骨骼。其生物学行为介于良性上皮样血管瘤和侵袭性更强的上皮样血管肉瘤之间。尽管恶性EHE的标准全身治疗方法尚未完全确立,但如果可行,强烈建议进行完整的手术切除。
EHE表现多样,CT有助于在早期及时识别回肠EHE,即使没有明显肿块形成,但只有经过组织病理学和免疫组织化学研究才能确诊。