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Pulmonary Vascular Disease |

Primary Pulmonary Artery Sarcoma Masquerades as Pulmonary Embolism: 20 Year Observational Analysis FREE TO VIEW

Debabrata Bandyopadhyay, MBBS; Mathew Bunte, MD; Tanmay Panchabhai, MBBS; Navkaran Bajaj, MBBS; Nicholas Smedira, MD; Brian Griffin, MD
Author and Funding Information

Respiratory Institute, Cleveland Clinic, Cleveland, OH


Chest. 2014;146(4_MeetingAbstracts):833A. doi:10.1378/chest.1991876
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Abstract

SESSION TITLE: DVT/PE/Pulmonary Hypertension Posters II

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 29, 2014 at 01:30 PM - 02:30 PM

PURPOSE: Primary pulmonary artery sarcoma (PPAS) is a rare lethal tumor, can mimic pulmonary thromboembolism (PE) in clinical & radiological presentation, leading to inappropriate diagnosis and therapeutic intervention. PPAS is rarely diagnosed pre-operatively, more commonly detected during thrombectomy for PE. We evaluated clinical characteristics of this tumor, utility of imaging modalities in assisting early accurate diagnosis and if early diagnosis improves survival.

METHODS: We reviewed patients with diagnosis of PPAS from Cleveland clinic (CCF) database and PPAS cases published in English literature between 1991 and 2010. We analyzed patient characteristics, therapeutic interventions, methods used for early correct diagnosis and usefulness of imaging techniques to differentiate it from PE. We performed survival analysis; if early diagnosis causes survival benefit.

RESULTS: Total 391 cases were analyzed (10 CCF & 381 literatures). Age at diagnosis was 52±14 years; 55% being male. Leimyosarcoma was most common histology. Symptom duration before diagnosis was 100 (31-210) days. Presenting symptoms were non-specific-shortness of breath (74%), chest pain (31%), cough (23%) and hemoptysis (15%). Fifty-percent cases were misdiagnosed with PE/PAH; 36% had correct diagnosis of PPAS prior to intervention. Thirty-nine percent patients incorrectly received thrombolytic therapy/anticoagulation; 23% underwent thrombectomy. Consequently diagnosis was delayed by 14 (7-90) days. Each day of early diagnosis improved survival (OR 0.96; 95% CI 0.92-0.98). Those diagnosed by CT/MRI had better survival (p<0.001). Chondrosarcoma, young patients with longer duration of symptoms predicted mortality. The characteristic CT scan appearance favoring diagnosis of PPAS over PE were filling defect involving whole of main pulmonary artery and one of its branch, a heterogeneous or delayed enhancement and globular appearance of the lesion. TEE was helpful to note planar invasion of tumor, predicting correct diagnosis in few cases.

CONCLUSIONS: Diagnosis of PPAS is challenging, often misdiagnosed as PE, leading to delay inappropriate therapeutic intervention. Typical clinical features at presentation may be helpful in accurate diagnosis but they are often non-specific. Early diagnosis even by a day, supported by characteristic radiological imaging, lead to improved survival.

CLINICAL IMPLICATIONS: PE is common but PPAS is rare, leading to frequent misdiagnosis delaying proper treatment. Early correct diagnosis will lead to improved survival.

DISCLOSURE: The following authors have nothing to disclose: Debabrata Bandyopadhyay, Mathew Bunte, Tanmay Panchabhai, Navkaran Bajaj, Nicholas Smedira, Brian Griffin

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