PT  - JOURNAL ARTICLE
AU  - Igor Motus
AU  - Vladimir Parshin
AU  - Yrii Belov
AU  - Alexander Chernyavsky
AU  - Anton Neretin
TI  - Pulmonary artery sarcoma. Complex problems with a rare tumor
AID  - 10.1183/13993003.congress-2015.PA4300
DP  - 2015 Sep 01
TA  - European Respiratory Journal
PG  - PA4300
VI  - 46
IP  - suppl 59
4099  - http://erj.ersjournals.com/content/46/suppl_59/PA4300.short
4100  - http://erj.ersjournals.com/content/46/suppl_59/PA4300.full
SO  - Eur Respir J2015 Sep 01; 46
AB  - Background. Pulmonary artery sarcoma (PAS) is rare. So the analysis of each case is especially important for the practice.Objectives. Review of the literature and analysis of clinical course of 4 our patients determined the main symptoms of PAS and optimal treatment strategy.Methods: The 1stand 2nd patients were subjected to right pneumonectomy with resection and pericardiac repair of truncus pulmonalis. Resection of the truncus with prostetic repair was done in the 3rd patient. Sternotomy and cardiac arrest were performed. Endarterecomy of the relapsed tumor was performed in the 1st patient 8 years after first operation. In the 2nd patient laser ablation of the metastases was done 2 years later in the left lung followed by five courses of A1 regimen chemotherapy. In the 4th case right pneumonectomy was performed in emergency due to intrapleural bleeding.Results: Three characteristic symptoms of PAS were as follows. 1.Clinic resembled to the acute or chronic pulmonary embolism. 2. Right ventricular disfunction and pulmonary hypertension were presented. Ventilation-perfusion scintiscan showed normal ventilation and severe decreased perfusion. 3. Chest CT showed endoluminal mass in the truncus pulmonalis near valves and spread distally to one or both arteries. By this way we disclosed PAS before the operation in three patients. Survival 8, 3 and 6 years was achieved due to radical surgery and adjuvant chemotherapy. The 4th patient died 4 months after the operation.Conclusions. Clinical manifestations and proper diagnostics allow timely recognize PAS. Prompt diagnosis and a complete removal of the tumor can improve the prognosis. Sporadic observations prevent from trails to select the adjuvant methods of the treatment.