原发性肺恶性血管外皮细胞瘤罕见,至今文献仅报道100例。作者报道1例,女,45岁,因咳嗽和活动时呼吸困难10个月入院。体检;手指呈杵状指,右肺呼吸音减低,全血细胞计数及电解质正常,血沉升高为62。胸片示右肺底一大的肿块伴有胸腔积液。行胸腔穿刺,其细胞培养及抗酸杆菌阴性。增强CT扫描示右下肺叶10cm×6cm×13cm肿块,分叶状,密度不均匀,伴有胸腔积液和少量心包积 Primary pulmonary malignant hemangiopericytoma is rare, so far only 100 cases have been reported in the literature. The author reported 1 case, female, 45 years old, hospitalized for 10 months for breathing difficulties due to cough and activity. Physical examination; fingers were clubbed fingers, the right lung breath sounds reduced, complete blood cell counts and electrolytes normal, ESR increased to 62. The chest radiograph showed a large mass in the right lung with pleural effusion. Thoracic puncture, its cell culture and acid-fast bacilli was negative. An enhanced CT scan showed a 10cm x 6cm x 13cm mass in the right lower lobe, lobulated, with uneven density, pleural effusion, and a small amount of pericardial effusion.