Volume 29, Issue 1 (3-2005)                   Research in Medicine 2005, 29(1): 70-77 | Back to browse issues page

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Malignant transformation of neurofibromatosis lesions in Von Recklinghausen Syndrome
Abstract:   (18393 Views)
Neurofibromatosis (Von Recklinghausen syndrome) is an autosomal dominant genetic disease caused by a mutation in the gene expressing neurofibromin. It is characterized by café-au-lait macules, axillary or inguinal freckling, multiple neurofibromas, and developmental delay. Malignant transformation or sarcomatosis may occur in as many as 1.5-15% of patients. Case report: We present a patient with Von Recklinghausen syndrome showing malignant transformation 20 years after the initial manifestations of the disease.
Keywords: KEYWORDS: Neurofibromatosis, Von Recklinghausen syndrome
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Type of Study: General | Subject: Interdisciplinary (Educational Management, Educational research, Statistics, Medical education
Received: 2003/11/27 | Published: 2005/03/15
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