Pulmonary Vascular Disease |

Pulmonary Artery Sarcoma: Not Every Filling Defect Is a Pulmonary Thromboembolism FREE TO VIEW

Himanshu Bhardwaj, MD; Muhammad Khawar, MD; Ahmed Awab, MD
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University of Oklahoma Health Sciences Center, Oklahoma City, OK

Chest. 2014;146(4_MeetingAbstracts):871A. doi:10.1378/chest.1993932
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SESSION TITLE: Pulmonary Vascular Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary artery sarcoma (PAS) is a rare malignancy arising from the mesenchymal cells of the intima of pulmonary artery.It is often misdiagnosed as pulmonary thromboembolism (PTE), as both diseases initially appear as intraluminal 'filling defects' in the pulmonary artery on contrast-enhanced CT scans. We describe a patient initially misdiagnosed as PTE,who later was found to have PAS.

CASE PRESENTATION: A 76-years-old male prsented with dry non-productive cough, pleuritic chest pain and dyspnea. Initial vital signs were stable and physical examination was unremarkable.Computed tomographic (CT) angiography scan revealed a “filling defect” (Fig 1 A) in the left main pulmonary artery. Unfractionated heparin was started and bridged to Coumadin; patient was discharged home with the diagnosis of PTE. After discharge, patient’s symptoms kept worsening with additional anorexia & weight loss. Due to worsening dyspnea and further weight loss of 20 pounds over the next 6 months, a follow-up CXR and CT-Chest angiogram (Fig 1 B) was performed. This revealed a 16 x 10 x 8 cm mass in the left main pulmonary artery which seemed to have originated from the filling defect seen on the initial CT-scan. An ultrasound-guided biopsy of the mass (Fig 2 A & B) was consistent with the diagnosis of PAS. Due to extensive disease, patient was offered only the palliative chemotherapy, which he declined.

DISCUSSION: PAS, first described by Mandelstamm in 1923, is a rare and often lethal tumor, usually diagnosed during surgery or autopsy. The absence of predisposing factors for PTE and the progressive worsening dyspnea in patients with an intraluminal “filling defect” despite anticoagulation therapy is factors suggestive of PAS. Recently, it has been reported that, positron emission tomographic scan (PET-CT) may help to make a differential diagnosis between PAS and PTE. Surgical resection remains the primary treatment, if detected early in the course of disease.

CONCLUSIONS: Clinicians should maintain high clinical suspicion of PAS in patients with a filling defect in the pulmonary artery without any underlying risk factors for PTE and worsening of symptoms despite anticoagulation therapy.

Reference #1: Ito K, Kubota K, Morooka et al. Diagnostic usefulness of 18F-FDG PET/CT in the differentiation of pulmonary artery sarcoma and pulmonary embolism. Ann Nucl Med 2009; 23:671-676.

Reference #2: Kruger I, Borowski A, Horst et al. Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Surg 1990; 38:91-95

DISCLOSURE: The following authors have nothing to disclose: Himanshu Bhardwaj, Muhammad Khawar, Ahmed Awab

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