Kamocki Zbigniew, Wilamowski Robert, Reszeć Joanna, Zaręba Konrad, Kędra Boguslaw
Department of Medical Pathomorphology, Medical University of Bialystok, Poland.
Contemp Oncol (Pozn). 2012;16(6):590-2. doi: 10.5114/wo.2012.32496. Epub 2013 Jan 4.
Hemangioendotheliomas are poorly differentiated neoplasms built from strands of endothelial cells and have a very narrow lumen filled with blood cells. Hemangioendotheliomas are commonly detected during the initial six months of life, concerning mainly soft tissues and skin. It is the most common bening liver cancer in children. In adults, hemangioendotheliomas are very rare findings, being mostly described in the liver, lungs, brain and bones. Hemangioendotheliomas of the large intestine are extremely rare in adults. Cancer developing there is of high degree of malignancy. The authors present the case of 68 year old patient in whom preoperative benign tumor with ulcers were diagnosed. Intraoperative macroscopic picture suggested malignant process. The patient underwent anterior rectal resection with regional lymphadenectomy. The postoperative histopathological evaluation with immunohistochemical studies angiosarcoma was diagnosed. A four year survival free of cancer was obtained, and the patient died due to myocardial infarct. Literature describes the short periods of survival of patients with vascular sarcoma of the colon. The most common reason for decease are metastases with massive haemorrhages. Bleeding tendency results from growing Kasabach-Merritt syndrome. It is characterized by profound thrombocytopenia and coagulopathy. The presented patient is a unique example of angiosarcoma with long period of survival.
血管内皮瘤是由内皮细胞条索构成的低分化肿瘤，有一个非常狭窄的充满血细胞的管腔。血管内皮瘤通常在出生后的最初六个月内被发现，主要累及软组织和皮肤。它是儿童最常见的良性肝癌。在成人中，血管内皮瘤非常罕见，大多在肝脏、肺、脑和骨骼中被描述。大肠血管内皮瘤在成人中极为罕见。发生在那里的癌症恶性程度很高。作者报告了一例68岁患者的病例，该患者术前被诊断为伴有溃疡的良性肿瘤。术中宏观表现提示为恶性病变。患者接受了直肠前切除术及区域淋巴结清扫术。术后组织病理学评估及免疫组化研究诊断为血管肉瘤。患者获得了四年无癌生存期，最终因心肌梗死死亡。文献描述了结肠血管肉瘤患者的生存期较短。最常见的死亡原因是伴有大量出血的转移。出血倾向是由不断发展的卡萨巴赫-梅里特综合征引起的。其特征是严重的血小板减少和凝血病。该病例是血管肉瘤长期存活的一个独特例子。
