Type 1 neurofibromatosis (von Recklinghausen disease).

Unilateral and multiple nodular plexiform Neurofibromas: An uncommon presentation of Neurofibromatosis type 1

Plexiform neurofibroma is a rare variant but pathognomonic of von Recklinghausen disease. We report an uncommon presentation of neurofibromatosis type I in a girl. This 11-year old girl had multiple plexiform neurofibromas on her left hand, and also several Lisch’s nodules. Classification and clinical features are discussed briefly with emphasis on the possibility this condition may not be give...

von Recklinghausen disease complicated by pulmonary hypertension.

Two patients with von Recklinghausen disease (neurofibromatosis type 1) were admitted to the hospital because of progressive heart failure. Both patients had prominent pulmonary hypertension revealed on cardiac catheterization. A lung perfusion scan did not show any gross defect. There were no underlying causes of pulmonary hypertension in either patient, such as chronic lung disease, congenita...

Neurofibromatosis, type 1 (von Recklinghausen disease).

Neurofibroma of the mandible in an adolescent with von Recklinghausen's disease.

Neurofibromatosis, as identified by yon Recklinghausen, is an autosomal dominant, neurocutaneous syndrome, characterized by multiple neurofibromas, cafe-au-lait spots, and iris Lisch nodules.1, 2 This disorder has also been referred to as the "Elephant Man" disease, and was the subject of a play and movie depicting the life of Joseph Merrick, who suffered severely from neurofibromatosis. 2 Othe...

Primary adenocarcinoma of the lung in a case of von Recklinghausen neurofibromatosis

Von Recklinghausen disease (neurofibromatosis, NF-1) is the most common inherited syndrome predisposing to neoplasia, particularly neural crest-derived tumors. However, occurrence of primary lung adenocarcinoma in association with NF-1 is not common. We present a 42-year old man with NF-1 and primary adenocarcinoma of the lung to discuss the linkage between these two entities. [Turk J Cancer 20...