Pulmonary sarcoma complicated with neurofibromatosis type 1 (NF1)

We report here a case of primary pulmonary sarcoma complicated with neurofibromatosis type 1 (NF1). A 60-year-old man was urgently admitted to Tokai University Hospital, Japan, due to severe right chest pain. Skin inspection showed subcutaneous tumor lesions and café-au-lait spots, and therefore a diagnosis of neurofibromatosis type 1 (NF1) was made. Chest X-ray showed marked fluid accumulation in the right pleural cavity, and he was diagnosed as having empyema by thoracic drainage. Chest X-ray showed a large round mass lesion (80 mm in diameter) in the right lower lung field. Percutaneous needle biopsy of the tumor revealed that the empyema was caused by actinomycotic infection, and then he underwent right middle and lower lobectomy. Histopathological examination of the surgical specimens revealed features of spindle cell sarcoma. Several postoperative systemic examinations showed no other lesions except in the right lung. We concluded that this was a complicated case of primary pulmonary sarcoma with NF1.