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Chirurgia 2000 October-December;13(5-6):277-82

language: Italian

Neurofibrosarcoma of the median nerve associated with neurofibromatosis type 1

Tonante A., Gagliano E., D'Alia C., Lo Schiavo M. G., Speciale G., Sturniolo G.


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An interesting case of neurofibroma of the median nerve degenerating in a neurofibrosarcoma, associated to a Von Recklinghausen disease is reported and a survey of the literature is made. The quick growth of the neoplasia is stressed: only 2 weeks since the clinical beginning; furthermore the effectiveness of the radiological exams (CT, NMR and angiography) is underlined in order to define the morphology and the anatomical relationship of the neoplasia. In conclusion, considering the morphological and histological peculiarity of this neoplasia, it is pointed out that the reported case has needed a conservative surgery of the nerve without a postoperative radiotherapy. The effectiveness of this therapy has been confirmed by the results of periodical clinical and radiological follow-up which at 1 year after the surgery did not show any reccurrent or metastatic disease.

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