Background: 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.
Case description: 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 in situ 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.
Conclusions: 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.
Keywords: Case report; chondrosarcoma; pulmonary artery sarcoma (PAS); pulmonary embolism (PE).
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