Primary Pulmonary Artery Sarcoma: Surgical Management and Differential Diagnosis with Pulmonary Embolism and Pulmonary Valve Stenosis

Abstract

Because of similar clinical features, pulmonary artery sarcomas are often confused with other pulmonary vascular obstructive diseases. Computed tomography scanning and gadolinium-enhanced magnetic resonance imaging could be useful methods for differential diagnosis. The prognosis is very poor. The survival time after resection varies from several months to several years depending on the presence of recurrence or metastasis. Early diagnosis and radical surgical resection presents the only opportunity for a potential cure.

Unresolved pulmonary embolism leading to a diagnosis of pulmonary artery sarcoma

“…8 Differentiation by CTPA is not always possible therefore techniques such as 18 F-FDG PET, PET/CT 13 and gadolinium enhanced MRI are often used. 1,4,7,12 By the time of diagnosis, up to 50% have lung metastasis. 1 The prognosis is dismal 2,3,14,15 with a mean survival of 1.5 months 2 which increases to 10e18 months with surgical excision making this the primary treatment.…”

Spindle cell sarcoma of pulmonary artery mimicking thromboembolism with lung metastasis detected in fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography

“…[1] It is thought to arise from multipotential mesenchymal cells of the intima and generally arises from the right, left, and main pulmonary artery. The reported age ranges from 13 to 86 years with the majority of cases occurring in the middle age.…”

“…[1] It is frequently misdiagnosed as pulmonary thromboembolism (PTE), although chest computed tomography (CT) can help differentiate PAS from PTE by showing a low-attenuation filling defect occupying the entire lumen of the proximal or main pulmonary artery, expansion of the involved arteries, or extraluminal tumor extension. [23] Therefore, a new noninvasive technique for the diagnosis of PAS is needed to avoid a delay in diagnosis and in treatment and to prevent unnecessary surgery.…”

Pulmonary Embolism in a Patient with Primary Renal Synovial Sarcoma: The Important Differential Diagnosis of Tumor Embolism and Its Therapeutic Implications

“…A number of case reports address tumor embolisms in sarcomas in general [11, 12], some of which occurred in clear association with surgery. Another rare differential diagnosis for pulmonary embolism is primary pulmonary artery sarcoma [13]. To our knowledge, only 1 case of tumor embolism in synovial sarcoma has been published so far [14] and no cases in primary renal synovial sarcoma, specifically.…”