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Neurofibromatosis type 1 can be severe and associated with progressive course and death. Although it has been rarely reported, a subset of patients with neurofibromatosis type 1 may develop interstitial lung disease. However, no case of neurofibrosarcoma and lung involvement together has been reported so far. We report a case of a 45-year-old male who was previously diagnosed as having neurofibromatosis type 1, 12 years later after the initial diagnosis he was histologically confirmed to have malignant transformation, and 3 years later he had associated diffuse lung damage

Neurofibromatosis type 1 complicated with malignant transformation and diffuse pulmonary disease