背景:血管肉瘤是一种高度恶性的软组织肉瘤,主要发生在皮肤和皮下组织中,来源于血管内皮细胞。小肠血管肉瘤很少见,预后较差。
方法:我们报告了一例原发性多灶性回肠血管肉瘤,并分析了以前报道的病例,以提高我们对小肠血管肉瘤的认识。小肠血管肉瘤多见于老年和男性患者。消化道出血,贫血,腹痛,弱点,和体重减轻是常见的症状。CD31,CD34,VIII因子相关抗原,ETS相关基因,朋友白血病整合1和vonWillebrand因子是诊断小肠血管肉瘤的有价值的免疫组织化学标志物。小肠血管肉瘤最常见于空肠,其次是回肠和十二指肠.辐射和毒物暴露是血管肉瘤的危险因素。经过明确的诊断,平均和中位生存时间分别为8个月和3个月,分别。Kaplan-Meier生存分析显示,年龄,入渗深度,化疗,肿瘤病变侵犯的小肠段数量是小肠血管肉瘤的预后因素。多因素Cox回归分析显示,化疗和手术均能明显改善患者预后。
结论:对于不明原因的黑便和腹痛,应考虑血管肉瘤,尤其是老年男性和有辐射暴露史的患者。及时治疗,包括手术和辅助化疗,对延长患者生存期至关重要。
BACKGROUND: Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues. Small-intestinal angiosarcomas are rare, and the prognosis is poor.
METHODS: We reported a
case of primary multifocal ileal angiosarcoma and analyze previously reported cases to improve our understanding of small intestinal angiosarcoma. Small intestinal angiosarcoma is more common in elderly and male patients. Gastrointestinal bleeding, anemia, abdominal pain, weakness, and weight loss were the common symptoms. CD31, CD34, factor VIII-related antigen, ETS-related gene, friend leukemia integration 1, and von Willebrand factor are valuable immunohistochemical markers for the diagnosis of small-intestinal angiosarcoma. Small-intestinal angiosarcoma most commonly occurs in the jejunum, followed by the ileum and duodenum. Radiation and toxicant exposure are risk factors for angiosarcoma. After a definite diagnosis, the mean and median survival time was 8 mo and 3 mo, respectively. Kaplan-Meier survival analysis showed that age, infiltration depth, chemotherapy, and the number of small intestinal segments invaded by tumor lesions were prognostic factors for small intestinal angiosarcoma. Multivariate Cox regression analysis showed that chemotherapy and surgery significantly improved patient prognosis.
CONCLUSIONS: Angiosarcoma should be considered for unexplained melena and abdominal pain, especially in older men and patients with a history of radiation exposure. Prompt treatment, including surgery and adjuvant chemotherapy, is essential to prolonging patient survival.