OBJECTIVE AND IMPORTANCE: Xanthogranulomas of the central nervous system are rare, and asymptomatic lesions are often identified in autopsies. We report the first case of a multicentric, cystic, intraparenchymal xanthogranuloma, involving both the supra- and infratentorial compartments, in a 16-month-old girl. CLINICAL PRESENTATION: The clinical presentation consisted of a focal motor seizure in an otherwise healthy infant. INTERVENTION: Gross total removal of the infratentorial lesion was accomplished via a midline suboccipital craniotomy. CONCLUSION: Intracranial xanthogranulomas are rare, and limited experience with the diagnosis of these lesions has been reported. Although most cases are asymptomatic, this case involves a symptomatic, multicentric, intraparenchymal xanthoma in a pediatric patient. Our review of the literature provides further insights regarding the clinical, radiological, and pathological behavior of these lesions and examines the available treatment strategies.
OBJECTIVE AND IMPORTANCE: Xanthogranulomas of the central nervous system are rare, and asymptomatic lesions are often identified in autopsies. We report the first case of a multicentric, cystic, intraparenchymal xanthogranuloma, involving both the supra- and infratentorial compartments, in a 16-month-old girl. CLINICAL PRESENTATION: The clinical presentation consisted of a focal motor seizure in an otherwise healthy infant. INTERVENTION: Gross total removal of the infratentorial lesion was accomplished via a midline suboccipital craniotomy. CONCLUSION: Intracranial xanthogranulomas are rare, and limited experience with the diagnosis of these lesions has been reported. Although most cases are asymptomatic, this case involves a symptomatic, multicentric, intraparenchymal xanthoma in a pediatric patient. Our review of the literature provides further insights regarding the clinical, radiological, and pathological behavior of these lesions and examines the available treatment strategies.