INTRODUCTION:Pulmonary artery sarcoma (PAS) is a rare and frequently fatal malignancy of the main pulmonary artery. The diagnosis is often difficult because the clinical manifestations are similar to pulmonary thromboembolism. The origin of this tumor is uncertain, and prognosis is poor because of advanced disease at the time of presentation and high recurrence rate. We describe a patient with PAS presenting with hemoptysis.
CASE PRESENTATION:A 41 year old previously healthy male presented with mild hemoptysis. His physical exam was unremarkable expect for a systolic ejection murmur. Biological and hematologic panels were within normal limits. Room air saturation was 99%. A chest radiograph indicated a right hilar mass. Chest computed tomography (CT) revealed a large soft tissue mass in the main pulmonary artery (PA) with extension into the right PA. A right hilar mass appeared to occlude the right lower lobe PA and a separate right upper lobe mass appeared to invade into the right upper lobe bronchus. He was systemically heparinized. During another episode of hemoptysis the patient produced a soft tissue mass that was found to be a high grade spindle cell carcinoma. Carinal and mainstem bronchial brushings were negative. Additional evaluation included a PET-CT scan in which the right-sided hilar and pulmonary masses, as well as the main and right pulmonary artery masses demonstrated significant FDG uptake. There were no other FDG avid regions. Transesophageal echocardiography showed mild pulmonary insufficiency and a large echo-dense mass involving the pulmonary valve with extension into the pulmonary trunk and right PA with normal biventricular size and function. A perfusion study showed virtually no perfusion to the right lung. At operation a pulmonary valve replacement with a 23 mm Medtronic Freestyle stentless porcine bioprosthesis-root valve and aggressive thromboendarterectomy of the main pulmonary artery and proximal right PA was performed concomitantly with an intrapericardial pneumonectomy. The patient’s postoperative course was uncomplicated and adjuvant chemotherapy with concurrent thoracic radiation therapy were delivered. At most recent follow up (10 months) the patient is free of recurrent disease. Final pathology revealed pulmonary artery sarcoma with extension into the right upper lobe bronchus.
DISCUSSIONS:Primary pulmonary artery sarcoma is a rare entity with just over 200 cases reported. Without treatment survival is rare beyond 1.5 months. Complicating the treatment of this malignancy is the difficulty in diagnosis due to its precarious clinical presentation and absence of specific symptoms. Often the diagnosis is made postoperatively following pulmonary thromboembolectomy. The patient reported here produced a tumor specimen with an episode of hemoptysis facilitating the preoperative diagnosis. Additionally, the hilar and parenchymal masses were suggestive a malignancy. Preoperative diagnosis can be difficult, but is feasible and can allow for resection with curative intent.
CONCLUSION:Once the diagnosis of PAS is made, aggressive surgical resection with adjuvant chemo and radiation therapy offer the best chance for long term survival. With this approach survival has been reported to be as great as 62 months. Often surgical palliation may be the only option to control the sequelae of tumor spread such as progression of pulmonary thromboembolism.
DISCLOSURE:Matthew Romano, No Financial Disclosure Information; No Product/Research Disclosure Information