Management of recurrent pilocytic astrocytoma with leptomeningeal dissemination in childhood

Neurofibromatoses are genetic disorders of the nervous system that primarily affect the development and growth of neural cell tissues. These disorders produce other abnormalities such as skin changes and bone deformities. Neurofibromatoses occur in both sexes and in all races and ethnic groups. Scientists have classified these disorders as neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2). Other types of neurofibromatoses may exist, but are not yet identified. Nearly 50% of the cases shows a well definited familiarity for the disease, according to an autosomal dominant transmission, while the other 50% of patients shows a negative history of familiarity, according to a new chromosomal mutation interesting the same autosomal genes of the dominant transmission. Oral localization is more rare showing an incidence ranging from 4 to 7% in most series of different authors. The most frequent involvement site in oral neurofibromatosis is the tongue, followed by the oral mucosa and floor of the mouth; palate and maxillary-mandibular bones are a rare localization of the disease. In the present, the clinical, radiological, histopathological and therapeutical aspects of a clinical case of neurofibromatosis, presenting as mandibular tumor, are examined. The clinical case reported, a 37 year old man, was essentially characterized by a positive family history for the disease, a neurofibroma of the oral mucosa.