| Summary: | The case of a 24-year-old male patient who had a history of neurofibromatosis type 1 and was admitted with headache, vomiting and dysarthria is reported. It was determined the existence of a supratentorial tumor, with a diagnosis of glial tumor of the type diffuse fibrillary astrocytoma grade IV. The report of various tumors associated with Von Recklinghausen's disease, and the imaging and morphological characteristics described, motivated a review the classification of the World Health Organization for tumors of the central nervous system and the classification of St Anne-Mayo for hemispheric tumors, allowing its proper location and its differentiation from other intracranial neoplasms that have been described in patients with the disease. The survival of the patient, for 20 months to date, coincides with the location of the tumor as a slower growing tumor that has undergone an anaplastic transformation, which has a relatively better prognosis.
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