Subependymal giant-cell astrocytoma: A surgical review in the modern era of mTOR inhibitors

Danassegarane, G.; Tinois, Julien; Sahler, Y.; Aouaissia, S.; Riffaud, Laurent

doi:10.1016/j.neuchi.2022.07.003

Cited by 6 publications

(6 citation statements)

References 49 publications

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“…The low number of cases available from our tertiary high-volume institution re ects the rarity of SEGA, which presents in only 10-25% of patients with TSC [5,6], and is in line with numbers presented in most other surgical series [11,13,15,17,22,23,26,27,30,42]. Studies from larger samples of patients are rare [25,29,31], particularly after the introduction of medical treatment with mTOR inhibitors [43].…”

Section: Discussionmentioning

confidence: 65%

“…The rationale for the treatment of SEGA is primarily to alleviate the mass effect and secondary hydrocephalus caused by the tumour. Although surgical treatment of symptomatic or growing SEGA remains a valid treatment strategy, the risk of signi cant neurological morbidity reported in older publications (5-50%) is still considered its main limitation [25,30,44]. The risk of complications appears particularly high in large SEGA [31], in which the use of alternative treatment methods -such as laser interstitial thermal therapy (LITT) and mTOR inhibitors -is limited.…”

Section: Discussionmentioning

confidence: 99%

“…In the most recent literature review of 18 studies published between 1980 and 2021 counting a total of 263 patients [30], the surgical morbidity was 4.9% and morbidity 33.6%, while ventriculoperitoneal was needed in 30.8% of cases. Although comparison is di cult due to low number of cases in the present series, our results are more in line with own data from the same study, showing few severe complications and negligible impact of surgical removal of SEGA on the grade of epilepsy in the identical number of patients [30].…”

Section: Discussionmentioning

confidence: 99%

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Surgical treatment of subependymal giant cell astrocytoma in patients with tuberous sclerosis complex – an institutional experience and results

Tuft,

Berger,

Marthinsen

et al. 2024

Preprint

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Objective Subependymal giant cell astrocytoma (SEGA) present in patients with tuberous sclerosis complex (TSC), occasionally requiring surgical removal. The study aimed to analyze the results from our series of children undergoing surgery for SEGA. Methods We retrospectively identified children with TSC undergoing resection of SEGA at Oslo University Hospital between 1982 to 2016. Patient charts, radiological images, epilepsy and neuropsychological reports were reviewed. Results Out of 208 patients with TSC, 18 (9%) underwent resection of SEGA. Due to missing data, we could only analyze results from 14 surgeries in 11 children (median age 6 years, range 0–19; male/female ratio 2.7:1). The tumours were bilateral in four (36%) patients. The tumor diameter was median 19 mm (10–104 mm). The surgical approach was transcortical in eight (57%) and transcallosal in six surgeries (43%). Gross total resection was achieved in 12 (86%) of surgeries. There was no mortality or major morbidity related to surgery, but one patient developed complications related to the ventriculoperitoneal shunt. During the follow-up (median 11 years, range 1–21), three patients (27%) underwent repeated surgery. We could not document any significant impact of the surgery on patients` cognitive functioning or the grade of epilepsy. Conclusions Resection of SEGA in children with TSC was associated with a low complication rate. We could not document any impact of surgery on patients` cognitive functioning or grade of epilepsy. However, the neuropsychological data were limited in most cases. Neuropsychological assessment should be performed before the surgery and be a part of follow-up after surgery.

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Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Surgical treatment of subependymal giant cell astrocytoma in patients with tuberous sclerosis complex – an institutional experience and results

Tuft,

Berger,

Marthinsen

et al. 2024

Preprint

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“…Among low-grade tumours, there are subependymal giant cell astrocytomas (SEGAs), rare brain tumours that primarily affect individuals with a genetic condition known as tuberous sclerosis complex (TSC). Treatment of SEGAs involves a combination of surgical resection and targeted medical therapy with MTOR inhibitors such as everolimus that inhibits the mTOR pathway which is overactive in TSC and contributes to tumour growth [ 40 , 41 ].…”

Section: Histological Subtypes and Molecular Characterizationmentioning

confidence: 99%

Central nervous system tumours in neonates: what should the neonatologist know?

Toniutti,

Sasso,

Carai

et al. 2024

Eur J Pediatr

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Central nervous system (CNS) tumours in neonates are relatively rare and present differently when compared with those occurring later in childhood in terms of aetiology, clinical features, location, histology and prognosis. The clinical presentation is extremely variable. Even if the most frequent clinical sign is a macrocephaly, there are many other non-specific symptoms associated. The prognosis is usually poor with overall survival of less than 30%. Surgery continues to be the primary treatment for neonatal CNS tumours, aiming for a gross total resection, directly correlated with prognosis and the overall outcome. The chemotherapy is the only adjuvant therapy whereas the radiotherapy is avoided under three years of age because of the severe sequelae. Hence the importance of molecular characterization of these neoplasms in order to improve the accuracy of the diagnosis and identify new therapeutic targets. The aim of this review is to describe the main characteristics of these tumours and the recent advances in their treatment in order to recognize these pathologies in the prenatal period and create a multidisciplinary team providing the best possible treatment while minimising the risk of long-term complications. Neonatologists play a key role in the early detection, diagnostic evaluation, management and supportive care of these neonates. Conclusion: The aim of this review is to describe the main characteristics of these tumours and the recent advances in their treatment in order to ensure the essential knowledge that will help the neonatologist identify them and create a multidisciplinary team providing the best possible treatment while minimising the risk of long-term complications. What is Known:• Neonatal CNS tumours are relatively rare and their early identification is important to identify the best diagnostic-therapeutic management.• Surgery is the main treatment of neonatal CNS tumours. The extent of surgical resection directly correlates with prognosis and outcome. What is New:• Predisposing conditions such as Cancer Predisposition Syndromes must be considered.• Targeted drugs and other therapeutic strategies can be identified through molecular characterization

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“…SEGAs are thought to arise from subependymal nodules, which are typically asymptomatic. The transformation into SEGAs is not fully understood but is believed to be associated with mutations in either the TSC1 or TSC2 genes, which are responsible for the regulation of the mTOR (mammalian target of rapamycin) pathway [ 3 ]. Dysregulation of this pathway leads to uncontrolled cell growth and proliferation, contributing to tumor development.…”

Section: Introductionmentioning

confidence: 99%

Beyond Benign: A Case of Subependymal Giant Cell Astrocytomas Provoking Hydrocephalus in Tuberous Sclerosis Complex

Navarro-Ballester,

Álvaro-Ballester,

Lara-Martínez

2024

AML

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22-year-old male diagnosed with Tuberous Sclerosis Complex (TSC), a genetic disorder characterized by benign tumors in various organs, with a focus on neurological implications. Central to the study is the development of Subependymal Giant Cell Astrocytomas (SEGAs), leading to hydrocephalus in the patient. The diagnosis of TSC was made in the patient’s childhood, and he was monitored regularly. The study highlights a significant growth in a subependymal nodule, leading to monoventricular hydrocephalus. MRI scans played a crucial role in identifying the progression of SEGAs and the subsequent hydrocephalus. The treatment approach involved endoscopic surgical removal of the SEGA, with histopathology confirming the diagnosis. Post-surgical outcomes over an eight-year follow-up period showed a normalization in ventricular size and the stability of other subependymal nodules, without any complications. This case underscores the importance of regular monitoring for TSC patients, early intervention for complications like hydrocephalus, and the need for a multidisciplinary treatment approach. The case study provides valuable insights into the management of neurodevelopmental disorders and the complexities surrounding TSC and SEGAs.

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Subependymal giant-cell astrocytoma: A surgical review in the modern era of mTOR inhibitors

Cited by 6 publications

References 49 publications

Surgical treatment of subependymal giant cell astrocytoma in patients with tuberous sclerosis complex – an institutional experience and results

Surgical treatment of subependymal giant cell astrocytoma in patients with tuberous sclerosis complex – an institutional experience and results

Central nervous system tumours in neonates: what should the neonatologist know?

Beyond Benign: A Case of Subependymal Giant Cell Astrocytomas Provoking Hydrocephalus in Tuberous Sclerosis Complex

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