Olgu sunumu: Glioblastoma ile seyreden bir nöroblastom olgusu

Neurofibromatosis ( NF ), is one of the most common otosomal dominant traits; its incidence has been estimated at 1 per 4000 births. There are two distinct forms of NF : NF-1 and NF-2. NF-1 is the most prevalent type ( 90 % ). The NF-1 gene on chromosome region 17q 11.2 encodes a protein ( neurofibromin ) that is thought to be involved in the regulation of the protooncogene ras. Cafe-au-lait spots are the hallmark of NF and are present in almost 100% of patients. Malignant neoplasms are also a significant problem in patients with NF-1. A 4,5 years old male patient was admitted to emergency room with symptoms of somnolence and headache. The patient had cafe-au-lait spots which made us to think that he had NF. For the diagnosis of associated CNS tumors cranial MRI was done. Cranial MRI revealed 7.5 cm x 5.5 cm x 5 cm intraaxial mass. Pathologic evaluation showed glioblastome multiforme. We present this case to emphasize the association of NF with intracranial tumors.