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Recommended Articles Abstract Type 1 neurofibromatosis, also known as von Recklinghausen's disease, is one of the most common diseases with autosomal dominant inheritance. The gene of neurofibromatosis, an NF1 tumour suppressor gene, is located on Chromosome Due to the loss of tumour suppressor function, mutation of the gene singlebörse recklinghausen to the development of benign and malignant tumours.

The close association of the two diseases is well known in singlebörse recklinghausen literature, with more than cases reported up to now. GIST associated with neurofibromatosis is a separate entity, and unlike sporadic GIST it is usually multiple and it almost always develops in the small intestine.

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It manifests itself usually at a younger age, with a slight female dominance. Its histological characteristics include spindle cell type, the presence of skeinoid bodies, and frequent S positivity.

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Its low mitotic activity usually suggests a more favourable prognosis. Presumably the pathomechanism of GIST associated with neurofibromatosis singlebörse recklinghausen different from that of the sporadic form, and the occurrence of GIST tumour is a part of the clinical spectrum of neurofibromatosis.

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Imatinib that has revolutionized GIST therapy is not efficient in this group of patients; however there are no sufficient data available yet.

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