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Neurofibromatosis type 1 complicated with malignant transformation and diffuse pulmonary disease
Received Date : 03 Jul 2016
Accepted Date : 05 Sep 2016
Nuri Karadurmus a , Yalcin Basaran b , Galip Buyukturan c , Mukerrem Safali d
a Department of Medical Oncology, Gulhane School of Medicine, Ankara, Turkey
b Department of Endocrinology, Gulhane School of Medicine, Ankara, Turkey
c Department of Internal Medicine, Gulhane School of Medicine, Ankara, Turkey d Department of Pathology, Gulhane School of Medicine, Ankara, Turkey
Doi: https://doi.org/10.1016/j.jons.2016.09.001 - Article's Language: EN
ABSTRACT
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.
Keywords: Neurofibromatosis type 1; Neurofibrosarcoma; Diffuse pulmonary disease
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