[Primary pulmonary rabdomyosarcoma: clinical-pathological and immunohistochemical findings]

Pulmonary rhabdomyosarcona is a rare entity and the histopatological differential diagnosis can be difficult. We report on a case of a 48-year-old male patient with a neoplasm located at the hilum of the right lung. The histological study of the lobectomy specimen allowed the diagnosis of embryonal rhabdomyosarcoma to be made. Given the absence of tumor lesions in other sites, it was classified as primary pulmonary neoplasm. The patient underwent chemotherapy and subsequently a completion pneumonectomy for recurrence of the tumor. One year after the initial surgery, he presented a metastasis in the right adrenal gland. He died 20 months after the original diagnosis. The importance of immunohistochemistry in the diagnosis is emphasized and the different theories that attempt to explain the histogénesis of these tumors in unusual sites are analyzed.