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. Research in Medicine. 2005; 29 (1) :70-77
URL: http://pejouhesh.sbmu.ac.ir/article-1-62-en.html
Abstract:   (15173 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.
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