Malignant Schwannoma in patients with von Recklinghausen disease: report of two cases
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چکیده
منابع مشابه
Malignant Schwannoma in patients with von Recklinghausen disease: report of two cases.
Von Recklinghausen’s disease is characterized by the presence of cafe-au-lait spots, multiple neurofibromas and hamartomas. It is a dominant autosomal disease with high penetrance and variable expression, involving the head and neck in 22 to 47% of the patients. It is known as type I neurofibromatosis, in order to differentiate it from central involvement, especially that of the eight cranial n...
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Type I Neurofibromatosis (NF1) is an autosomal-dominant inheritable disorder, with an incidence of 1:3,000, and a prevalence of 1:4,000 to 5,000. Pathogenesis is based on mutations of the NF1 gene, a tumor suppressor gene encoding a cytoplasmic protein named neurofibromin that controls cellular proliferation. Patients affected by NF1 typically present with cutaneous neurofibromas, cafè au lait ...
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Adrenal tumours are frequently incidental discoveries, and their therapy is a subject of controversial discussions. Herein we describe two cases of adrenal schwannoma discovered during autopsy. The accidental observation of two benign schwannomas of the adrenal gland on asymptomatic patients led to the hypothesis that the real frequency of this type of lesion was underestimated. Furthermore, th...
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Background: Schwannoma is a benign tumor which usually presents as a solitary slow-growing painless mass. Approximately 25–48% of all schwannomas occurs in the head and neck region and is found rarely in oral cavity, about 1% of head and neck cases. Tongue is the most common site, followed by buccal mucosa, palate, floor of the mouth, jaw, gingiva and lips. This tumor may prese...
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ژورنال
عنوان ژورنال: Brazilian Journal of Otorhinolaryngology
سال: 2009
ISSN: 1808-8694
DOI: 10.1016/s1808-8694(15)30851-x