Extraventricular subependymal giant cell tumor in a child with tuberous sclerosis complex

Case report

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  • 1 Departments of Neurosurgery,
  • | 2 Pathology, and
  • | 3 Neurology (Comprehensive Epilepsy Center), New York University School of Medicine and NYU Langone Medical Center, New York, New York; and
  • | 4 Pediatric Epilepsy Service, Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts
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Subependymal giant cell tumors (SGCTs) are observed in 5–20% of patients with tuberous sclerosis complex (TSC) but account for ~ 25% of neurological morbidity. The authors report the case of a 7-year-old girl with TSC and multiple cortical tubers who presented with worsening seizures in the context of the rapid growth of a cystic, calcified, extraventricular SGCT in the right frontal lobe, initially thought to represent a cortical tuber. The tumor and surrounding tubers were excised, and clinical seizures resolved. This is the first report of an extraventricular SGCT in a child with TSC outside the neonatal period.

Abbreviations used in this paper:

EEG = electroencephalography; TSC = tuberous sclerosis complex; SGCT = subependymal giant cell tumor.

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