Background: pulmonary artery sarcoma is a rare mesenchymal tumor. The prognosis of this very infrequent disease remains poor,and surgical resection is important for obtaining the best outcome.
Case Presentation: A 51-year-old male patient presented to our clinic due to the finding of the isolated lung mass in the left lower lobe of the lung. Preoperative pulmonary artery computed tomographic angiography scans showed a mass with the in the left pulmonary artery and an isolated mass in the left dorsal segment of the lung (RS6).Under extracorporeal circulation the left pneumonectomy was performed, the surgical resection margins were confirmed to be tumor-free on frozen section and all lymph nodes were free of tumor. Histopathology, immunohistochemistry, as well as fluorescence in situ hybridization(FISH) indicated the diagnosis of pleomorphic rhabdomyosarcoma
Conclusion: This case report describes a PAS which was successfully resected with pneumonectomy and concludes that biopsy may play a limited role in confirming the pathological type and pneumonectomy should be considered in the surgical treatment of PAS.