Long-term recurrence of dysembryoplastic neuroepithelial tumor: Clinical case report

Dysembryoplastic neuroepithelial tumors (DNETs) are rare, benign brain neoplasms that typically arise in children and adolescents and classically present with intractable, partial complex seizures. DNETs are classically associated with a favorable prognosis after complete surgical resection.

We describe a case of long-term recurrence of a DNET, which initially resected and diagnosed as an oligodendroglioma prior to the recognition of DNETs. This patient was seizure-free for 12 years and had no signs of radiologic progression until 24 years after initial resection. On repeat surgical resection, 31 years after the initial surgery, histopathologic evaluation identified the characteristic features of DNET in both specimens.

This patient's 24-year disease-free interval prior to radiologic recurrence demonstrates the longest interval to relapse in the literature for a DNET. This case illustrates the possibility of late recurrence of DNETs decades after radiographical complete resection to emphasize the necessity of thoughtful clinical judgment in adult survivors of low grade pediatric neoplasms who present with seizures after a prolonged seizure-free interval.