Obeso Carillo Gerardo Andrés, García Fontán Eva María, Cañizares Carretero Miguel Ángel, Pérez Pedrosa Alberto
Department of Thoracic Surgery, Vigo University Clinical Hospital, Pizarro No 22, 36204, Vigo, Pontevedra, Spain,
Gen Thorac Cardiovasc Surg. 2013 Nov;61(11):643-7. doi: 10.1007/s11748-012-0200-6. Epub 2013 Jan 11.
Primary pulmonary angiosarcoma is a rare type of malignant vascular tumour characterised by proliferation of tumour cells with endothelial features. Up to date, only sixteen cases have been reported in English Literature. Treatment modalities vary from none to surgery, chemotherapeutic regimens, radiotherapy or immunotherapy, but none of them have been shown to be effective. Unfortunately, these tumours are usually very aggressive and overall mortality is very high. We present two cases of patients with a diagnosis of primary pulmonary angiosarcoma, the largest case series ever described, and a review of the scientific literature.
原发性肺血管肉瘤是一种罕见的恶性血管肿瘤，其特征是具有内皮特征的肿瘤细胞增殖。截至目前，英文文献中仅报道了16例。治疗方式从无治疗到手术、化疗方案、放疗或免疫治疗不等，但均未显示出有效。不幸的是，这些肿瘤通常具有很强的侵袭性，总体死亡率非常高。我们报告了两例诊断为原发性肺血管肉瘤的患者，这是有史以来描述的最大病例系列，并对科学文献进行了综述。
