ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2017
Unique findings of subependymal giant cell astrocytoma within cortical tubers in patients with tuberous sclerosis complex: a histopathological evaluation.
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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6 citing papers in PubMed, 13 citations in OpenAlex.
- Surgical treatment of subependymal giant cell astrocytoma in patients with tuberous sclerosis complex-an institutional experience and results.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Modeling human neurodevelopmental diseases with brain organoids.Cell regeneration (London, England) · 2022Review
- MRI-based diagnosis and treatment of pediatric brain tumors: is tissue sample always needed?Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2021Review
- Neuroimaging in tuberous sclerosis complex.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020Review
- Recent advances in human stem cell-based modeling of Tuberous Sclerosis Complex.Molecular autism · 2020Review
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10 authors at 6 institutions in 2 countries.
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Abstract
introductionTuberous sclerosis is associated with three central nervous system pathologies: cortical/subcortical tubers, subependymal nodules (SENs), and subependymal giant cell astrocytomas (SEGAs). Tubers are associated with epilepsy, which is often medication-resistant and often leads to resective surgery. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be effective reducing seizure burden in some patients with tuberous sclerosis complex (TSC)-related refractory epilepsy. mTORi have also been shown to be an alternative for surgery treating SEGAs. We describe several cases of resected tubers that contained SEGA tissue without an intraventricular SEGA.
methodsAfter institutional review board (IRB) protocol approval, we retrospectively reviewed the surgical-pathological data for all TSC patients who underwent cortical resections for treatment of refractory epilepsy at NYU Langone Medical Center and Tel Aviv Medical Center between 2003 and 2013. Data included demographics, epilepsy type, MRI characteristics, epilepsy outcome, and histopathological staining.
resultsWe reviewed cortical resections from 75 patients with complete pathological studies. In three patients, cortical lesions demonstrated histopathological findings consistent with a SEGA within the resected tuber tissue, with no intraventricular SEGA. All lesions were cortically based and none had any intraventricular extension. No patient had been treated before surgery with an mTORi. Two of the three patients remain Engel grade I-II. All lesions stained positive for glial fibrillary acidic protein (GFAP), synaptophysin, and neuronal nuclear antigen (NeuN).
conclusionThis is the first description of cortical tubers harboring SEGA tissue. This observation though preliminary may suggest a subgroup of patients with intractable epilepsy in whom mTORi may be considered before surgical intervention.
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