Pulmonary artery sarcoma with extensive leiomyosarcoma differentiation and heterologous osteosarcomatous elements

Pulmonary artery sarcomas are exceptionally unusual. Their clinic, diagnosis and treatment play a very important role in the ultimate outcome and long-term survival. We present the case of a 70-year-old gentleman diagnosed with a leiomyosarcoma of the pulmonary artery with osteosarcoma differentiation that underwent surgical resection and subsequent chemotherapy, with good recovery at 9 months follow-up. Late diagnosis and incomplete surgical resection will worsen the short- and long-term prognosis of these patients.