Wu Ying, Huang Jing, Wang Qin, Zhang Mei, Luo Yimin, Wang Xihua, Zhu Xiaoli, Liu Hongbing
Department of Respiratory Medicine, Zhongda Hospital, School of Medicine, Southeast University, Nanjing 210009, People's Republic of China.
Department of Respiratory Medicine, Jinling Hospital, Nanjing University School of Medicine, Nanjing 210002, People's Republic of China.
Onco Targets Ther. 2019 Aug 7;12:6227-6235. doi: 10.2147/OTT.S212416. eCollection 2019.
Pulmonary artery sarcoma (PAS) is a rare and aggressive mesenchymal tumor that often mimics thromboembolic disease. Due to its rare and fatal nature, patients are often underdiagnosed or misdiagnosed. There is still no consensus regarding the diagnosis and treatment of PAS. We present a case of a 63 year old male misdiagnosed with pulmonary thromboembolism who received anticoagulant therapy. FDG positron emission tomography (PET) integrated with computed tomography (PET/CT) and subsequent surgery led to the final diagnosis of PAS. Whole exome sequencing of the tissue identified the genetic alterations profile of PAS: copy number variation (CNV) of and mutations of and . Treated with chemotherapy followed by anlotinib, the patient's survival time was 8 months after firm diagnosis. To our knowledge, anlotinib used as a treatment for PAS has not been reported.
肺动脉肉瘤（PAS）是一种罕见的侵袭性间充质肿瘤，常酷似血栓栓塞性疾病。由于其罕见且致命的特性，患者常常被漏诊或误诊。关于PAS的诊断和治疗目前仍未达成共识。我们报告一例63岁男性患者，其被误诊为肺血栓栓塞症并接受了抗凝治疗。氟代脱氧葡萄糖正电子发射断层扫描（FDG PET）与计算机断层扫描相结合（PET/CT）并随后进行手术，最终确诊为PAS。对该组织进行的全外显子组测序确定了PAS的基因改变图谱： 的拷贝数变异（CNV）以及 和 的突变。患者在确诊后接受了化疗，随后使用安罗替尼治疗，生存时间为8个月。据我们所知，尚未有将安罗替尼用于治疗PAS的报道。
