[The diagnostic challenge of a rare pulmonary neoplasm]

Primary pulmonary sarcomas account for less than 0.5% of all thoracic neoplasms. They're aggressive tumors arising in mesenchymal cells from the bronchial walls, vessels or pulmonary interstitium. The authors present a patient in which the diagnostic pathway ended with the diagnosis of a primary pulmonary leiomyosarcoma. This case was a true challenge, illustrative of the difficulty associated with this type of neoplasm, but also regarding its aggressiveness, considering its rapid and fatal progression.