RESUMO
Neurofibromatosis also referred to as von Recklinghausen`s disease, is an autosomal dominant disease, which is characterized by cutaneous neurofibromas, cafe-au-lait spot, and axillary freckles. Although neruofibromatosis is a congenital disorder, the pulmonary manifestation become evident in adulthood. Approximately 15percent of patients with cutaneous lesions have intrathoracic neurofibromas. In some patients with neurofibromatosis, the lungs are the seat of interstitial fibrosis, leiomyoma, and bullous lesion, meningocele or less commonly lung cancer. The association of neurofibromatosis and lung cancer is rare, A 47-year-old man, who was a heavy smoker, was admitted to our hospital because of hemoptysis. Since cafe-au-lait sopts, diffuse pigmentation and multiple neurofibromatosis were observed on the skin, he was considered to have von Recklinghausen`s disease. Chest X-ray film showd extensive hazy density affecting all of his lung field, and minimal peribronchial infiltratin in his left lower lung field. Chest CT showed that near total consolidative lesions were present of right middle lobe. Also, some patchy infiltration were present in his right lower lobe and some of left lower lobe. Histoathological examination of the percutaneous lung biopsy specimen demonstrated that he had undifferentiated large cell carcinoma. The Authors have experienced a case of neurofibromatosis combined with undifferntiated large cell carcinoma. A brief review of related literature was conducted.
