Obstructive Lung Diseases: Fellow Case Report Poster - Obstructive Lung Disease |

Pulmonary Embolism: The Great Masquerader FREE TO VIEW

Alberto Revelo, MD; Parimalkumar Chaudhari, MD; George Maguire, MD; Lisa Paul, MD
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New York Medical College, Valhalla, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):902A. doi:10.1016/j.chest.2016.08.1002
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SESSION TITLE: Fellow Case Report Poster - Obstructive Lung Disease

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: We present a rare case of pulmonary artery sarcoma (PAS) with classic acute pulmonary embolism (PE) presentation.

CASE PRESENTATION: A 29 year old man smoker presented with dyspnea, chest pain and hypoxemia. He denied long travels, drug use and medical history was relevant for Asthma. Acute PE involving main and left pulmonary arteries was diagnosed (Fig. 1). An Echocardiogram, DVT and hypercoagulable studies were normal. He was discharged on Rivaroxaban. A month later, he's back with recurrent symptoms; a CTPA showed new multiple bilateral pulmonary nodules and cavitations. He denied constitutional symptoms. Examination showed a well appearing man; SaO2 of 97% on room air; chest, heart and skin exam were normal. Laboratory tests showed eosinophilia of 10% and ESR at 94 MM/HR. Connective tissue disease and ANCA antibodies work up were negative. Unfractioned heparin was started. Repeated CTPA showed increased clot burden, pulmonary nodules and ground glass opacities(Fig. 2).Trans-bronchial biopsies via electromagnetic navigational bronchoscopy resulted in benign lung tissue. A right heart catheterization showed mildly elevated PA pressures, normal PCWP and CO. Trans-pulmonary pressure gradient was19 and PVR 3.82 WU. Cardiothoracic surgery service proceeded with pulmonary thrombo endarterectomy and lung biopsy. A mass involving the main and left pulmonary arteries was found with chronic thrombo embolic disease. Pathology revealed high grade/advanced intimal pulmonary artery sarcoma positive for Vimentin and FLI-1; negative for S-100, CD 31, CD 34, SMA, AE1/AE3 and Actin. Systemic chemotherapy was advised.

DISCUSSION: This patient presented with acute symptom onset, elevated ESR and pulmonary nodules. Peripheral emboli to the distal pulmonary circulation may account for periodic exacerbations like in this patient. Altogether with increased clot burden despite anticoagulation was suspicious for malignancy. Metastasis are common and surgery remains the mainstay of treatment; prognosis is poor.

CONCLUSIONS: PAS are exceedingly rare and diagnosis is often delayed and mistaken for chronic PE. A suspicion should be raised when PE is diagnosed without risk factors or evidence of DVT, a markedly elevated ESR and recurrent symptoms are seen along with increased clot burden after adequate anticoagulation.

Reference #1: Surgical Treatment for pulmonary artery sarcoma.European Journal of Cardio-thoracic Surgery (2008) Hong Kwan Kim et al

Reference #2: Pulmonary Artery Sarcoma. Clinical Features.CHEST1996;110:1480-88James E Parish, MD FCCP et al

DISCLOSURE: The following authors have nothing to disclose: Alberto Revelo, Parimalkumar Chaudhari, George Maguire, Lisa Paul

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