Zhang Yucong, Xu Gang
Department of Radiation Oncology, Shenzhen People's Hospital (The Second Clinical Medical College, Jinan University; The First Affiliated Hospital, Southern University of Science and Technology), Shenzhen, China.
Transl Cancer Res. 2023 Apr 28;12(4):1060-1066. doi: 10.21037/tcr-23-518.
Primary pulmonary artery sarcoma (PAS) is a rare tumor that presents like pulmonary embolism (PE), primary chondrosarcoma in the pulmonary artery is even rarer and few studies have been reported. PAS are commonly misinterpreted as in a clinical setting, many patients initially receive anticoagulant and thrombolysis therapy, but failed to respond. Management of this condition is difficult and prognosis is poor. We report a case of primary pulmonary artery chondrosarcoma that was initially misdiagnosed as PE and Inappropriate interventional therapy was performed, but with poor response. Finally, patient received surgical treatment, postoperative pathology confirmed primary pulmonary artery chondrosarcoma.
A 67-year-old woman who had presented with cough, chest pain and shortness of breath for more than 3 months. Computed tomography pulmonary angiography (CTPA) showed filling defects were seen in the right and left pulmonary arteries, spreading to the outer lumen. The patient was initially diagnosed with PE and underwent transcatheter aspiration for pulmonary artery thrombus, transcatheter thrombolysis, and inferior vena cava filter placement at a local hospital, but with poor response. She was then referred for pulmonary artery tumor resection, endarterectomy and pulmonary arterioplasty. Histopathological examinations confirmed a diagnosis of primary PAS (chondrosarcoma). The patient developed recurrence of pulmonary artery tumors in 10 months after surgery and received six cycles of adjuvant chemotherapy. The lesions progressed slowly after chemotherapy. The patient subsequently developed lung metastasis in 22 months and died of heart failure and respiratory failure 2 years after surgery.
PAS is an extremely rare and the clinical symptoms and radiological features often mimics PE, therefore When doctors make differential diagnosis of pulmonary artery mass lesions, especially when the anticoagulation and thrombolytic effects are very poor. They need to be alert to the possibility of PAS so that early diagnosis and early treatment can prolong the survival of patients.
原发性肺动脉肉瘤（PAS）是一种罕见的肿瘤，临床表现类似肺栓塞（PE），肺动脉原发性软骨肉瘤更为罕见，相关研究报道较少。在临床环境中，PAS常被误诊，许多患者最初接受抗凝和溶栓治疗，但效果不佳。这种疾病的治疗困难，预后较差。我们报告一例原发性肺动脉软骨肉瘤病例，最初被误诊为PE并进行了不恰当的介入治疗，但反应不佳。最后，患者接受了手术治疗，术后病理证实为原发性肺动脉软骨肉瘤。
一名67岁女性，咳嗽、胸痛和气短3个多月。计算机断层扫描肺动脉造影（CTPA）显示左右肺动脉有充盈缺损，延伸至管腔外。患者最初被诊断为PE，在当地医院接受了经导管肺动脉血栓抽吸、经导管溶栓和下腔静脉滤器置入，但效果不佳。随后她被转诊进行肺动脉肿瘤切除、动脉内膜切除术和肺动脉成形术。组织病理学检查确诊为原发性PAS（软骨肉瘤）。患者术后10个月出现肺动脉肿瘤复发，接受了6个周期的辅助化疗。化疗后病变进展缓慢。患者随后在22个月时出现肺转移，术后2年死于心力衰竭和呼吸衰竭。
PAS极为罕见，其临床症状和影像学特征常类似PE，因此医生在对肺动脉肿块病变进行鉴别诊断时，尤其是抗凝和溶栓效果很差时，需要警惕PAS的可能性，以便早期诊断和早期治疗能够延长患者的生存期。
