New York Methodist Hospital, Brooklyn, NY
Copyright 2016, American College of Chest Physicians. All Rights Reserved.
SESSION TITLE: Fellow Case Report Slide: Venous Thromboembolic Disease
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Tuesday, October 25, 2016 at 04:30 PM - 05:30 PM
INTRODUCTION: Primary pulmonary artery sarcoma (PAS) is a rare malignancy commonly misdiagnosed as chronic pulmonary thromboemboli. Median time from symptom onset to definitive diagnosis and treatment is 3-12 months. Surgery can be curative, but only if diagnosed early. We present a case of PAS diagnosed in a patient with worsening dyspnea on exertion and weight loss.
CASE PRESENTATION: A 72 year old man with a 20 pack year smoking history presented with dyspnea on exertion for 10 weeks, cough and weight loss. His physical exam was significant for tachycardia and decreased breath sounds on the right. A chest radiograph showed a prominent right hilum. Chest computed tomography (CT) with contrast found a large filling defect involving the entire right main pulmonary artery. A cardiac MRI showed an intraluminal mass with a superimposed thrombus consistent with a PAS. A PET scan revealed a hypermetabolic mass in the region measuring 2.7 x 8.8cm. Pulmonary function testing was normal, and a right pneumonectomy with sleeve resection of the right pulmonary artery was performed. Pathology revealed intimal sarcoma involving the right pulmonary artery, right upper and lower lobe artery with focal extension into the lung parenchyma.
DISCUSSION: PAS was first described in 1923 on autopsy and is characterized by undifferentiated spindle cells arising from the pulmonary trunk with proliferating along the intima with marked cellular pleomorphism and a high mitotic index. The disease is difficult to diagnose and is associated with a high mortality rate. If able to, a pneumonectomy with neoadjuvant chemotherapy is the treatment of choice, as the median survival without surgery is 1.5 months. It has been suggested that if pulmonary hypertension is present on diagnosis, then the disease is likely bilateral. In that case, a pulmonary endarterectomy can be performed with palliative intent.
CONCLUSIONS: PAS is a rare and aggressive malignancy where diagnosis is often delayed and PA sarcoma is considered after there is no improvement after anticoagulation therapy. A high index of suspicion is required for the prompt diagnosis and management as surgery is the only curative therapy.
Reference #1: A middle-aged man with cough, chest pain, and pulmonary artery filling defect. Shigemitsu H, Orjioke N, Shriki JE, Varras J, Koss MN. Chest. 2014;145(2):407-410.
Reference #2: Genoni M, Biraima AM, Bode B, Shan AC, Wilkler MB, Turina MI. Combined resection and adjuvant therapy improves prognosis of sarcomas of the pulmonary trunk. J Cardiovasc Surg (Torino). 2001;42(6):829-83
DISCLOSURE: The following authors have nothing to disclose: Liam Gross, Stephen Milan, Ruth Minkin
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