Subependymal Giant Cell Astrocytoma Presenting with Tumoral Bleeding: A Case Report

Jung, Tae‐Young; Lee, Kyung‐Hwa; Kim, Seul-Kee

doi:10.14791/btrt.2017.5.1.37

Cited by 15 publications

(3 citation statements)

References 27 publications

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“…Images were taken from a 17year-old boy with TSC and intractable seizures. Reproduced with permission from Kalantari and Salamon [79] morphologic features of giant glial cells, with an expression of GFAP and S-100 protein [77,86,87,90]. These atypical and unusually large cells express brain lipid-binding protein (BLBP) [91,92], which plays a key role during the development of radial glia and migration of immature neurons [93].…”

Section: Human Tsc Brain Pathologymentioning

confidence: 99%

Revisiting Brain Tuberous Sclerosis Complex in Rat and Human: Shared Molecular and Cellular Pathology Leads to Distinct Neurophysiological and Behavioral Phenotypes

et al. 2021

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Tuberous sclerosis complex (TSC) is a dominant autosomal genetic disorder caused by loss-of-function mutations in TSC1 and TSC2, which lead to constitutive activation of the mammalian target of rapamycin C1 (mTORC1) with its decoupling from regulatory inputs. Because mTORC1 integrates an array of molecular signals controlling protein synthesis and energy metabolism, its unrestrained activation inflates cell growth and division, resulting in the development of benign tumors in the brain and other organs. In humans, brain malformations typically manifest through a range of neuropsychiatric symptoms, among which mental retardation, intellectual disabilities with signs of autism, and refractory seizures, which are the most prominent. TSC in the rat brain presents the first-rate approximation of cellular and molecular pathology of the human brain, showing many instructive characteristics. Nevertheless, the developmental profile and distribution of lesions in the rat brain, with neurophysiological and behavioral manifestation, deviate considerably from humans, raising numerous research and translational questions. In this study, we revisit brain TSC in human and Eker rats to relate their histopathological, electrophysiological, and neurobehavioral characteristics. We discuss shared and distinct aspects of the pathology and consider factors contributing to phenotypic discrepancies. Given the shared genetic cause and molecular pathology, phenotypic deviations suggest an incomplete understanding of the disease. Narrowing the knowledge gap in the future should not only improve the characterization of the TSC rat model but also explain considerable variability in the clinical manifestation of the disease in humans.

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Section: Human Tsc Brain Pathologymentioning

confidence: 99%

Revisiting Brain Tuberous Sclerosis Complex in Rat and Human: Shared Molecular and Cellular Pathology Leads to Distinct Neurophysiological and Behavioral Phenotypes

et al. 2021

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“…Because their location and growth potential can cause increased intracranial pressure, obstructive hydrocephalus, focal neurologic deficits, and death [11]. Acute symptoms can develop insidiously by progressive growth of the tumor and abruptly by intratumoral bleeding [8,9,[12][13][14][15][16][17][18][19][20][21][22].…”

Section: Sega and Tuberous Sclerosismentioning

confidence: 99%

Subependymal Giant Cell Astrocytoma Apoplexy: A Case Report and Systematic Review

Turkistani¹,

Fallatah²,

Ghoneim³

et al. 2023

Cureus

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Subependymal giant cell astrocytoma (SEGA) is the most common intracranial tumor in tuberous sclerosis (TS) patients. The tumor generally localizes in the proximity of Monro's foramen; as it grows, it subsequently causes hydrocephalus and increases intracranial pressure (ICP). However, acute symptoms of increased ICP due to intratumoral bleeding rarely manifest in SEGA patients.We present a 27-year-old male with TS who presented due to hemorrhagic complications of SEGA with intratumoral bleeding and vitreous orbital hemorrhage. We then conducted a systematic review with four databases (PubMed, Web of Science, Google Scholar, and Cochrane) to identify similar cases using the following keywords: "Subependymal giant cell astrocytoma," "Hemorrhage," "Haemorrhage," and "Bleeding."Our review identified 12 articles reporting 14 cases of hemorrhagic complications of SEGA in addition to our case report. The median age of diagnosis was 21 (range 5-79) years with unequal gender distribution (M:F ratio, 11:4). Headache was the most presented symptom, followed by hemiparesis, seizure, altered mental status, visual deterioration, and headache accompanied by seizure. TS was seen in most of the cases (80%). Gross total resection (GTR) was achieved in 53.5% of the patients. Regarding the clinical outcome, 66.7% had a good outcome, 20% died, and 13.3% had no report of their outcomes. No tumor recurrence was seen in the cases with a reported duration of follow-up.Catastrophic presentation of SEGA apoplexy is a rare occurrence. We present a case report with a systematic review and discuss SEGA apoplexy's possible pathophysiology and outcome.

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“…SEGAs are considered to be benign lesions and excision of the tumor is considered to be curative in patients presenting with a single lesion. Surgical treatment is indicated in cases of symptomatic SEGA or patients presenting with acute increase in intracranial pressure due to obstructive hydrocephalus [29]. Other alternative treatment options are Gamma Knife radiosurgery [GKR] or the mechanistic target of rapamycin [mTOR] inhibitors which can reduce the size of the mass in TSC.…”

Section: Treatmentmentioning

confidence: 99%

Lesions at the Foramen of Monro Causing Obstructive Hydrocephalus

Chugh¹,

Gotecha²,

Punia³

et al. 2022

Cerebrospinal Fluid

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The foramen of Monro has also been referred to by the name of interventricular foramen. The structures comprising this foramen are the anterior part of the thalamus, the fornix and the choroid plexus. Vital structures surround the foramen, the damage to which can be catastrophic leading to disability either temporary or permanent. In the literature it has been shown that tumors occurring in the area of interventricular foramen are rare and usually cause hydrocephalus. The operative approach depends upon the location of the tumor which can be either in the lateral or the third ventricle. Various pathologies which can lead to foramen of Monro obstruction and obstructive hydrocephalus include colloid cyst, craniopharyngioma, subependymal giant cell astrocytoma [SEGA], Neurocysticercosis, tuberculous meningitis, pituitary macroadenoma, neurocytoma, ventriculitis, multiseptate hydrocephalus, intraventricular hemorrhage, functionally isolated ventricles, choroid plexus tumors, subependymomas and idiopathic foramen of monro stenosis. In this chapter, we will discuss the various lesions at the level of foramen of Monro causing obstructive hydrocephalus and the management and associated complications of these lesions based on their type, clinical picture and their appearance on imaging.

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Subependymal Giant Cell Astrocytoma Presenting with Tumoral Bleeding: A Case Report

Cited by 15 publications

References 27 publications

Revisiting Brain Tuberous Sclerosis Complex in Rat and Human: Shared Molecular and Cellular Pathology Leads to Distinct Neurophysiological and Behavioral Phenotypes

Revisiting Brain Tuberous Sclerosis Complex in Rat and Human: Shared Molecular and Cellular Pathology Leads to Distinct Neurophysiological and Behavioral Phenotypes

Subependymal Giant Cell Astrocytoma Apoplexy: A Case Report and Systematic Review

Lesions at the Foramen of Monro Causing Obstructive Hydrocephalus

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