Abstract
A case of primary pulmonary rhabdomyosarcoma occurring in a 62-year-old man is reported, and a review of the literature is presented. The tumor affected the left upper lobe and involved the mediastinal lymph nodes. Immunohistochemical and ultrastructural studies supported the myogenic phenotype of the neoplasm. A left pneumonectomy was performed with complete surgical removal of the tumor. Postoperative radiotherapy was carried out. The patient is currently alive and free of disease 9 months after operation. Despite the rarity of primary pulmonary rhabdomyosarcoma, this tumor should be differentiated from other poorly differentiated pulmonary neoplasms and from metastatic sarcomas.