Neurofibromatosis Type I in Childhood

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Neurofibromatosis type 1 and malignancy in childhood.

Neurofibromatosis type 1 (NF1) is an autosomal dominant hereditary neurocutaneous syndrome characterized by multi-system involvement and an increased incidence of both benign and malignant tumors. In this study, we evaluated the clinical presentation and prognosis of NF1 and malignancy. Between 1975 and 2013, 26 (5%) of the 473 patients with NF1 at our center developed non-neurofibroma neoplasm...

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Chiari I malformation and neurofibromatosis type 1.

Single case reports exist in the medical literature of patients with tonsillar ectopia, i.e., the Chiari I malformation and neurofibromatosis type 1. However, large series of patients with either of these entities have not been examined for the presence of both defects. We have retrospectively examined two large groups of pediatric patients: Group I, with the primary diagnosis of Chiari I malfo...

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Lung Cancer Associated with Neurofibromatosis Type I

Lung cancer associated with neurofibromatosis type I is considered very rare, and only a few case reports have been described in the literature. There is some evidence that a genetic linkage between neurofibromatosis and carcinogenesis in the lung may exist. We present a 42-year-old female, lifetime nonsmoker with a known history of neurofibromatosis type I, free of respiratory symptoms, who un...

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A review on 17 cases of type I Neurofibromatosis associated with malignancies

Background: Neurofibromatosis 1 (NF1) is a relatively common autosomal dominant condition. Among the complications of the disease, such as neurological disorders, hypertension and skeletal deformities, malignancy is the most serious one and is the main cause of early death in these patients. Objective: Review of cases of NF-1 associated with malignancies. Patents and Methods: In a retrospective...

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Folliculosebaceous Cystic Hamartoma in a Patient with Neurofibromatosis Type I

Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma composed of dilated folliculosebaceous units and mesenchymal elements. It presents as a papule or nodule usually on the face and scalp, rarely on the genital or trunk area. Histologically, FSCH shares several similar features to sebaceous trichofolliculoma. We report one case of FSCH misdiagnosed as a neurofibroma. He was ...

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ژورنال

عنوان ژورنال: Journal of Neuropathology and Experimental Neurology

سال: 1998

ISSN: 0022-3069

DOI: 10.1097/00005072-199804000-00010