Dysembryoplastic Neuroepithelial Tumours in Pediatric Patients: Long-Term Follow up and Outcome

Keywords: brain tumor, outcome, children, surgery, resection

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     Dysembryoplastic Neuroepithelial Tumour (DNET) is a rare and generally benign pediatric brain tumour whose optimal management and long-term outcome is uncertain.
     Of the 54 patients identified, 32 (59.3%) were males and 50 (92.6%) presented with seizures at an average age of 8.44 years.
     We retrospectively reviewed all patients who were diagnosed with a DNET at the Hospital for Sick Children and Cincinnati Children’s Medical Center from 1987 to 2011. Patient demographics, clinical presentation, operative procedures, pathology, and outcome were determined.
     The most common seizure presentation was partial complex (42%). The most common tumour locations were frontal (38.9%) and temporal (29.6%) lobes. Average duration of post-operative follow up was 4.66 years (range 0-12.8). Of patients with seizures at presentation and =2 years of post-operative follow-up, 21 of 29 patients with complete tumor excision and 4 of 10 patients with residual tumours were seizure-free. One patient received adjuvant radiation for early regrowth, followed by chemotherapy for dissemination, and at repeat resection 4 years later revealed apparent malignant transformation to a rhabdoid tumour. All patients were alive at last follow up.
     This is a retrospective study.
     Pediatric DNETs are rare tumours with an excellent overall prognosis. Gross total excision where possible provides excellent tumor and seizure control.
     Tumours not amenable to complete excision are more problematic and require ongoing surveillance. The need for adjuvant treatment is exceptional and should raise the issue of an atypical DNET that may have different behavior.


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