Dysembryoplastic neuroepithelial tumors are unique and benign congenital tumors occurring frequently in children and adolescents. Differentiation from other low-grade tumors is important for management. Five patients with confirmed dysembryoplastic neuroepithelial tumors were studied with positron emission tomography using glucose and protein metabolic uptake in an attempt to categorize these tumors metabolically. Functional brain mapping also was obtained to aid in operative management. Results of the study conclude that dysembryoplastic neuroepithelial tumors, although having similar neuroimgaing characteristics to other low-grade tumors, are distinguished by a unique metabolic profile. They are inactive tumors with no significant glucose or protein metabolic activity. The combination of preoperative positron emission tomographic metabolic studies with functional brain mapping allowed for prediction of tumor type, defined eloquent areas of cortical function, and improved approach and resection of the tumors with minimal risk of neurologic impairment.
2 patients with Dandy-Walker malformations (DWM) and cardiac anomalies are presented. The types of cardiac defects with septal involvement are suggestive of an early embryonic abnormality occurring before 6 weeks’ gestational age. This correlates with the postulated timing of the development of the DWM and suggests a complex developmental anomaly. Awareness of the associated systemic and central nervous system anomalies in patients with the DWM in regard to management and prognosis is emphasized.
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