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Extraventricular subependymal giant cell tumor in a child with tuberous sclerosis complex
Robert J Bollo1, Jonathan L Berliner, Ingeborg Fischer
1Department of Neurosurgery, New York University School of Medicine and NYU Langone Medical Center, New York, New York 10016, USA. robert.bollo@nyumc.org
Insights
Subependymal giant cell tumors (SGCTs) are a common complication in tuberous sclerosis complex (TSC). This case highlights an extraventricular SGCT in a child with TSC, successfully treated with surgical excision, resolving seizures.
Area of Science:
- Neuro-oncology
- Pediatric Neurology
- Genetics
Background:
- Subependymal giant cell tumors (SGCTs) occur in 5-20% of patients with tuberous sclerosis complex (TSC).
- SGCTs contribute significantly to neurological morbidity in TSC patients, accounting for approximately 25% of cases.
- Tuberous sclerosis complex is a genetic disorder characterized by the growth of hamartomas in multiple organs.
Observation:
- A 7-year-old girl with TSC and multiple cortical tubers presented with escalating seizures.
- Imaging revealed a rapidly growing, cystic, calcified, extraventricular SGCT in the right frontal lobe.
- The lesion was initially misidentified as a cortical tuber.
Findings:
- Surgical excision of the extraventricular SGCT and surrounding tubers was performed.
- Post-operative resolution of clinical seizures was achieved.
- This represents the first reported case of an extraventricular SGCT in a pediatric TSC patient outside the neonatal period.
Implications:
- Early recognition and surgical intervention for extraventricular SGCTs in pediatric TSC patients can lead to favorable outcomes.
- This case expands the understanding of SGCT presentation and management in tuberous sclerosis complex.
- Further research into the specific characteristics and growth patterns of extraventricular SGCTs in children with TSC is warranted.
Abstract:
Subependymal giant cell tumors (SGCTs) are observed in 5-20% of patients with tuberous sclerosis complex (TSC) but account for approximately 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.
