肺动脉肉瘤临床及影像学表现

目的:分析肺动脉肉瘤(pulmonary artery sarcoma,PAS)患者的临床及CT影像学表现,以提高对本病的认识。方法:回顾性分析自2001年11月至2012年11月,北京安贞医院收治并经病理证实的PAS病例共9例,对其临床特点、实验室检查及影像学表现进行讨论。结果:9例患者确诊年龄32~67岁,中位数年龄为43岁。临床表现主要为胸闷、心悸、胸痛及晕厥等,2例表现为低氧血症。7例患者术前诊断为中央型肺动脉栓塞并行溶栓治疗无效。7例D-二聚体正常,6例乳酸脱氢酶(LDH)升高。9例患者均行CT肺动脉成像,8例表现为肺动脉腔内充盈缺损,为分叶状、向外隆起、边缘清晰;1例表现为沿主肺动脉及右肺动脉管壁浸润性生长;单纯累及右肺动脉1例,累及左、右肺动脉及分支部2例,累及主肺动脉及左右肺动脉主干6例,其中2例导致右肺动脉闭塞,9例均显示不同程度强化,3例同时发现肺动脉外转移灶。结论:PAS临床表现及实验室检查缺乏特异性,CT肺血管成像对本病的诊断具有重要意义。当临床发现有类似肺动脉血栓栓塞症状,D-二聚体正常,溶栓治疗无效时,应警惕本病可能。特别是CTPA发现病灶边缘饱满、隆起,甚至有分叶,增强扫描明显不均匀强化者,高度提示本病。

The clinical and imaging manifestation of pulmonary artery sarcoma

Objective：Our objective was to analyze the clinical characteristics and imaging characteris- tics of pulmonary artery sarcoma（ PAS）, in order to improve its diagnosis accuracy. Methods：The clinical data and imaging characteristics of 9 patients with PAS, who were treated at Beijing Anzhen Hospital from November 2001 to November 2012, were retrospectively analyzed. The diagnoses of PAS were all confirmed by surgical biopsy. Results：The median age was 43 year old （32 -67years）. The clinical symptoms were unspecific, including chest distress, chest pain or syncope, and the D-Direct were normal in 7 patients. Seven patients were misdiagnosed as pulmonary embolism, and were treated with failed thrombolytic therapy. Nine patients underwent pulmonary CT angiography, from which, plumpness, even lobulated intraluminal filling defects were detected in eight patients, and infiltrative growth along the vessel wall was detected in one patient. The main pulmonary artery and bilateral pulmonary arteries were involved in 6 patients, and the right pulmonary arteries were occluded by the lesions in 2 of them. Only right pulmonary artery was involved in one patient, and bilateral pulmonary arteries were both involved in two patients. The lesions can be enhanced in all 9 patients and metastases were detected in three patients. Conclusion： PAS is a rare entity that can present in a variety of ways. To a patient prediagnosed as pulmonary embolism but with a normal D-Dimer and having ineffective anticoagulation therapy, PAS should be suspected. The pulmonary CT angiography finding is very important for the diagnosis of PAS. Suspicion should be heightened if the occupying lesion in pulmonary CT angiography is plumpness, evenlobulated, and obvious non-homogeneous enhanced.