A MISSED PULMONARY ARTERY SARCOMA REQUIRING RADICAL EXCISION AND PNEUMONECTOMY: SURGICAL TECHNIQUE

Abstract

<h3>BACKGROUND</h3> A 64-year-old previously well male patient was referred with a 3-month history of progressive shortness of breath (New York Heart Association class III) and non-exertional chest pain radiating to the back. TA transthoracic echocardiogram (TTE) demonstrated mild flow acceleration across the pulmonary valve, without insufficiency and estimated pulmonary pressures of 55 - 60 mmHg. Repeat CT scan identified an approximately 6 cm mass completely filling the right pulmonary artery (RPA) to the hilum and extending down the main pulmonary artery (MPA), potentially involving the pulmonary valve. The left pulmonary artery (LPA) appeared mostly spared. Multidisciplinary oncological team evaluation suggested concerns for pulmonary artery sarcoma prompting consideration for urgent radical excision with homograft reconstruction and right pneumonectomy. <h3>METHODS AND RESULTS</h3> Upon discovery of bilateral PAS in our patient, we decided intraoperatively to continue with a complete radical resection and pneumonectomy, keeping with the goal of complete curative excision. This required extensive planning for surgery including ensuring homograft availability for reconstruction of the pulmonary root and its branches. Cardioplegic arrest was utilized to allow aortic transection to provide optimal exposure posterior to the aorta to determine tumor extension. Given only partial extension into the LPA, we prioritized good margins distally towards the left lung, and used an additional pulmonary allograft for reconstruction. Complete obstruction of the RPA and tumor extension into the right lung necessitated a right pneumonectomy. Pathology confirmed intimal sarcoma with negative margins, and confirmed tumor spread into the right lung parenchyma. At 6 months follow-up, this patient is doing well with no signs of tumour recurrence. Repeat CT demonstrates a patent and functional reconstructed MPA and LPA. <h3>CONCLUSION</h3> Though this technique requires meticulous planning and surgical expertise, it offers a reasonable alternative to conventional management with greater survival benefit. The hope is this approach can build on past literature and lead to longer survival post-operation in patients diagnosed with this rare but deadly disease.