A case report of dysembryoplastic neuroepithelial tumor associated with neurofibromatosis type 1

Dysembryoplastic neuroepithelial tumor (DNT) in a newly proposed mixed neuroglial tumor in the cerebral cortex. However, DNT associated with phacomatosis has mostly been considered exceptional. In this paper, a case of DNT associated with neurofibromatosis type 1 is reported. A 23-year-old male was admitted to our hospital complaining of intractable complex-partial seizure. He had a history of neurofibromatosis type 1 (NF - 1) and pituitary dwarfism. On general physiological examination, many cafe au lait spots and freckling could be noted, showing that the case was neurofibromatosis type 1. In addition, neurological examination showed no abnormal findings. MR images revealed a small area of abnormal intensity on the right temporal. This region showed high intensity on T2 weighted image and low intensity without enhancement on T1 weighted image. On electroencephalography (EEG), an epileptic spike focus was demonstrated in the right temporal lobe. So, lobectomy was perfomed for control of epilepsy. Postoperative course was uneventful and without seizure. On histological examination, the tumor was composed of three different components: specific glioneural element, foci of oligodendrocyte-like cell, and cortical dysplasia. All of these findings were consistent with the definition of DNT by Daumas-Duport et al, except for the association with NF - 1. Although reported cases of DNT associated with FN - 1 are exceptionally rare, both DNT and NF - 1 originate from mal-development of the fetal central nervous system. It is very interesting that our case indicated the possibility of co-existence of both diseases.