Primary congenital intracranial lipoma with extracranial extension in a pediatric patient: A case report and literature review.

Background: Congenital intracranial lipomas are rare, representing only 0.1-0.5% of brain tumors, and are typically asymptomatic. An extracranial component is exceedingly rare, usually reported in slow-growing adult cases. This report presents a unique case of a rapidly growing congenital intracranial lipoma with extracranial extension in a pediatric patient. Case Description: A 3-year-old girl with a progressively enlarging forehead mass, present since birth, was found to have a large interhemispheric lipoma (12 × 10 × 9 cm) extending into the left lateral ventricle and connected to an extracranial mass (17 × 10 × 10 cm) through a frontal bone defect. Additional findings included corpus callosal agenesis and venous drainage from the scalp lesion into the superior sagittal sinus. The microsurgical intervention involved the excision of the extracranial lipoma and subtotal resection of the intracranial component, preserving critical neurovascular structures. Conclusion: This case of rapid lipoma growth in a pediatric patient is atypical, differing from slow-growing, asymptomatic presentations in adults. High-magnification microsurgery and Doppler guidance facilitated safe resection with a positive outcome. This case highlights the need for further research into developmental factors and rapid growth mechanisms in pediatric intracranial lipomas with extracranial components, potentially representing a distinct clinical entity.