gms | German Medical Science

Objectives: Primary pulmonary artery sarcomas (PPAS) present rare mesenchymal tumors which are frequently misdiagnosed as unilateral pulmonary embolism. Mean survival without surgical intervention is only 1.5 months.

Methods: Three patients with PPAS who underwent extended pneumonectomy with reconstruction of the remaining pulmonary artery on cardio-pulmonary bypass between August 2003 and December 2004 are presented.

Results: Patients were male and 30, 47 and 78 years old. All patients have been initially diagnosed by spiral contrast-enhanced CT-scan as having massive unilateral pulmonary embolism, and received heparine followed by oral anticoagulation. In all cases, duplex sonography of the lower extremities revealed no deep vein thromobosis, and chest MRI demonstrated no gadolinium enhancement. In contrast, in all patients PET scan revealed a tumor-typical increase in tracer uptake (F-18 fluorodeoxyglucose) with no signs of either mediastinal, pulmonary or distant metastasis. On postoperative histological examination, angiosarcomas grad II to III were found with no spread to intrapulmonary or hilar lymph nodes and resection margins being free. All patients are followed by clinical examination and chest CT scan. Within a follow-up of 28, 32 and 44 months, no signs of recurrence have been observed.

Conclusions: Patients diagnosed as suffering unilateral pulmonary thromboembolic disease who clinically do not respond to an adequate systemic anticoagulation and in whom no source of thromboembolic disease can be found, shall undergo FDG-PET scan. Otherwise, PPAS may be misdiagnosed as deaths due to "massive unilateral chronic pulmonary embolism". Radical resection with vascular reconstruction is technically feasible, and may offer long term survival.