MULTICENTRIC PARENCHYMAL XANTHOGRANULOMA IN A CHILD:CASE REPORT AND REVIEW OF THE LITERATURE Maciej S. Lesniak, M.D. Department of Neurosurgery, The Johns Hopkins Hospital, Baltimore, Maryland Michael P. Viglione, M.D., Ph.D. Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland Jon Weingart, M.D. Department of Neurosurgery, The Johns Hopkins Hospital, Baltimore, Maryland Reprint requests: Maciej S. Lesniak, M.D., Department of Neurosurgery, The Johns Hopkins Hospital, Meyer 8-161, 600 North Wolfe Street, Baltimore, MD 21287. Received, February 27, 2002. Accepted, June 13, 2002. 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 asymptom- atic, 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. KEY WORDS: Intracranial, Multicentric, Pediatric, Seizure, Xanthogranuloma Neurosurgery 51:1493-1498, 2002 DOI: 10.1227/01.NEU.0000036623.16853.93 www.neurosurgery-online.com I ntracranial xanthogranulomas, which have been reported to arise exclusively within the choroid plexus or the dura, are asymptomatic lesions that almost always oc- cur in adults (6, 64). Symptomatic xanthogran- ulomas are particularly rare; when they occur, the symptoms are usually secondary to ob- struction of cerebrospinal fluid (CSF) path- ways or mass effect. Occasionally, these xan- thomatous tumors occur in association with diseases such as Hand-Schuller-Christian dis- ease, Weber-Christian disease, histiocytosis X, or malignant fibrous histiocytoma or as com- plications of other metabolic or storage dis- eases. We present the first case of a multicen- tric, cystic, symptomatic, intraparenchymal xanthogranuloma in a child without a history of an underlying medical disorder. CASE REPORT A previously healthy, 16-month-old, female infant was brought to the emergency room with a history of a new focal motor seizure. The physical examination revealed nothing abnormal. The neurological examination was unremarkable. The pupils were round, equal, and reactive. The optic discs were sharp. Mo- tor strength was equal and symmetrical in all extremities. Computed tomographic scans revealed a left frontal cystic mass and a right cerebellar cystic mass. On magnetic resonance imaging (MRI) scans, these lesions appeared isointense to hyperintense on T1-weighted scans and hy- pointense on T2-weighted scans (Fig. 1, A and B). Post-contrast scans revealed homogeneous enhancement of the left frontal and right cer- ebellar lesion (Fig. 1, C and D). In addition, a previously undetected small mass in the right putamen was noted. The ventricles were en- larged and, although no mass effect or shift resulted from any of the supratentorial le- sions, the cerebellar lesion caused distortion of the fourth ventricle. Laboratory investigation results were nor- mal and demonstrated no evidence of hyper- cholesterolemia or hyperlipoproteinemia. Fur- ther genetic screening results were negative for any evidence of a storage or metabolic disease. The patient underwent a midline suboccip- ital craniotomy for removal of the cerebellar lesion. The tumor was encapsulated by hard fibrous tissue and was removed without com- plications. No invasion of the surrounding brain tissue or the fourth ventricle was ob- served. The postoperative course was un- eventful; however, the child required place- ment of a ventriculoperitoneal shunt, for NEUROSURGERY VOLUME 51 | NUMBER 6 | DECEMBER 2002 | 1493