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Pulmonary Embolism or Pulmonary Artery Intimal Sarcoma: A Diagnostic Challenge FREE TO VIEW

Navitha Ramesh, MD; James Murray, DO
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Unity Health System, Rochester, NY

Chest. 2013;144(4_MeetingAbstracts):631A. doi:10.1378/chest.1703543
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SESSION TITLE: Cancer Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Primary pulmonary intimal sarcomas are rare yet malignant neoplasms. Most cases are misdiagnosed as pulmonary embolism (1), leading to a delay in diagnosis and poor prognosis. We present a case of primary pulmonary sarcoma promptly diagnosed and treated owing to an elevated index of suspicion.

CASE PRESENTATION: 53-year-old female, lifelong non-smoker was seen in the pulmonary clinic for cough and pleuritic chest discomfort for 3 months. Prior evaluation included a non-contrasted CT of chest showing predominantly non-specific peripheral pleural-based opacities. Evaluation was notable for a pleural rub and normal spirometry, lung volumes and diffusing capacity. A CT angiogram identified a large filling defect in left main pulmonary artery and left lower lobe arteries hence the patient was anticoagulated. She returned to the clinic 2 months later, with progressive dyspnea despite ongoing anticoagulation. A repeat CT angiogram demonstrated propagation of the filling defect into the right main pulmonary artery (Figure 1). A PET/CT revealed a hypermetabolic endoluminal abnormality with an SUV of 6.8. Chest MRI revealed a soft tissue mass in the distal left pulmonary artery with non-enhancing thrombus in the more proximal portion of the left pulmonary artery (Figure 2). CT guided biopsy revealed malignant cells, consistent with primary pulmonary artery intimal sarcoma. The patient underwent surgical resection. Two months later, imaging showed a new filling defect in right pulmonary artery, which was hypermetabolic on PET- consistent with recurrent sarcoma, subsequently confirmed by biopsy. Patient is now on chemotherapy and has an excellent functional status a year after her diagnosis.

DISCUSSION: Propagation of the filling defect despite appropriate anticoagulation is unusual in pulmonary embolism. The discordance between clinical symptoms and radiographic burden raised the suspicion of an alternate diagnosis. A short interval follow-up CT angiogram showing progressive filling defect with conglomerate appearance without affecting airways or immediate parenchyma, heightened our suspicion of pulmonary sarcoma.

CONCLUSIONS: Complete resolution of pulmonary thromboembolism is not routinely achieved between 8 days and 11 months after acute PE. Routine re-imaging after cessation of anti coagulant therapy has been suggested in order to achieve a new baseline (2). We believe that short interval follow up CT angiogram should also be recommended when there are any atypical features of history, imaging and the course of recovery after diagnosis.

Reference #1: 1. Delany SG,Doyle TCA, Bunton RW,Hung NA, Joblin LU,Taylor DR. Pulmonary artery sarcoma mimicking pulmonary embolism. Chest 1993;103:1631-1633.

Reference #2: 2. Mathilde Nijkeuter, MD; Marcel M.C Hovens, MD; Bruce L. Davidson, MD, MPH, FCCP; and Menno V. Huisman, MD Resolution of thromboemboli in patients with acute pulmomary embolism: A systemic review. CHEST 2006; 129:192-197

DISCLOSURE: The following authors have nothing to disclose: Navitha Ramesh, James Murray

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