背景:原发性胃上皮样血管肉瘤是一种高度侵袭性的内皮细胞恶性肿瘤,可能会带来巨大的诊断挑战。
■在这里,我们描述了一个56岁男子出现黑便和上腹部隐痛2周的病例。
方法:原发性胃上皮样血管肉瘤:通过内皮标志物如分化簇31(CD31)的免疫组织化学分析提供明确的诊断,ether-a-go-go相关基因(ERG),和弗氏白血病整合(FLI-1)。
方法:胃镜下胃底出血活检后,结果提示恶性肿瘤,行胃癌根治术。
结果:不幸的是,约1个月后发生区域淋巴结肿大和远处转移.患者没有机会接受化疗或其他治疗,死于多器官功能障碍综合征。
结论:原发性胃上皮样血管肉瘤是罕见的肿瘤,淋巴结和周围器官转移率高。上皮样血管肉瘤中强烈的细胞角蛋白表达代表了病理学家的诊断陷阱。他们的临床行为是不可预测的,仅手术切除的结果令人失望。因此,通常认为预后较差,诊断后患者很少能存活超过1年。
BACKGROUND: Primary gastric epithelioid angiosarcoma is a highly aggressive endothelial cell malignancy and may pose a great diagnostic challenge.
UNASSIGNED: Here we describe the
case of a 56-year-old man presented with melena and epigastric dull pain for 2 weeks.
METHODS: Primary gastric epithelioid angiosarcomas: the definitive diagnosis was provided by immunohistochemical analysis with endothelial markers such as cluster of differentiation 31 (CD31), ether-a-go-go-related gene (ERG), and Freund leukemia integration (FLI-1).
METHODS: After gastroscopic biopsy was performed at the bleeding fundus and the results suggested malignant tumor, radical gastrectomy was performed.
RESULTS: Unfortunately, regional lymph node enlargement and distant metastases occurred about 1 month later. The patient did not have the opportunity to undergo chemotherapy or other treatment and died from multiple organ dysfunction syndrome.
CONCLUSIONS: Primary gastric epithelioid angiosarcomas are rare tumors with a high rate of lymph nodes and peripheral organs metastasis. The strong cytokeratin expression in epithelioid angiosarcomas represents a diagnostic pitfall for pathologists. Their clinical behaviors are unpredictable and results with surgical excision alone have been disappointing. Thus, the prognosis is generally considered poor and patients seldom can survive over 1 year after diagnosis.