Subependymal giant cell astrocytoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2], Sujit Routray, M.D. [3]

Subependymal giant cell astrocytoma is a benign tumor arising within the ventricles of the brain. It is almost exclusively associated with tuberous sclerosis, an autosomal dominant disorder associated with the inactivation of the tumor suppressor genes, TSC1 and/or TSC2. Subependymal giant cell astrocytoma is believed to arise from a subependymal nodule present in the ventricular wall of a patient with tuberous sclerosis. On microscopic histopathological analysis, it is characterized by pleomorphic multinuleated eosinophilic cells with abundant cytoplasm, arranged in a perivascular pseudopallisading pattern. Subependymal giant cell astrocytoma is a disease that tends to affect the pediatric, adolescent, and young adult population. Males are more commonly affected than females. According to the International Tuberous Sclerosis Complex Consensus, screening for subependymal giant cell astrocytoma by MRI is recommended every 1-3 years among patients with tuberous sclerosis, even in the absence of symptoms. If left untreated, patients with subependymal giant cell astrocytoma may progress to develop seizures, occlusion of the foramen of Monro with subsequent elevated intracranial pressure and obstructive hydrocephalus, infection, stroke, and death. Common complications of subependymal giant cell astrocytoma include obstructive hydrocephalus, intratumoral hemorrhage, and infection. Symptoms of subependymal giant cell astrocytoma include headache, seizures, vision loss, changes in speech, weakness in limbs, and sensory loss. Common physical examination findings of subependymal giant cell astrocytoma include papilledema, vision field defects, developmental delay, aphasia, sensory loss, and hemiparesis. Head CT scan and brain MRI may be helpful in the diagnosis of subependymal giant cell astrocytoma. On head CT scan, subependymal giant cell astrocytoma is characterized by an intraventricular mass near the foramen of Monro, which is iso- or slightly hypoattenuating to the grey matter. Accompanying hydrocephalus may be present. There is marked enhancement on contrast administration. On MRI, subependymal giant cell astrocytoma is characterized by hypo- to isointensity on T1-weighted imaging and hyperintensity on T2-weighted imaging. There may be marked enhancement on contrast administration. Surgical resection is the mainstay treatment of subependymal giant cell astrocytoma but in certain cases, medical therapy such as everolimus is used.

In 2012, subependymal giant cell astrocytoma was described at the International Tuberous Sclerosis Complex Consensus Conference as a lesion located in the caudothalamic groove having a size of >1 cm in any direction or a subependymal lesion that has shown serial growth on consecutive imaging regardless of size and location.

There is no classification system established for subependymal giant cell astrocytoma.

Subependymal giant cell astrocytoma is almost exclusively associated with tuberous sclerosis complex, which is an autosomal dominant disorder. It is associated with inactivation of the tumor suppressor genes, TSC1 and/or TSC2. It is also believed to arise from a subependymal nodule present in the ventricular wall of a patient with tuberous sclerosis. Some of the common findings seen on microscopic pathology include pleomorphic multinuleated eosinophilic cells, streams of elongated tumor cells with abundant cytoplasm, and clustered cells arranged in a perivascular pseudopallisading pattern. On immunohistochemistry, the tumor cells are positive for glial fibrillary acidic protein, microtubule-associated protein 2, synaptophysin, S-100, neurofilament, and neuron-specific enolase.

Subependymal giant cell astrocytoma is predominantly seen in patients with tuberous sclerosis complex which is caused by a mutation in the TSC1 and TSC2 tumor suppressor genes.

Subependymal giant cell astrocytoma must be differentiated from ependymoma, meningioma, tuberculoma, intraventricular hemorrhage, glioblastoma multiforme, primary CNS lymphoma, and cerebral metastases.

Subependymal giant cell astrocytoma is the most common central nervous system tumor in patients with tuberous sclerosis complex. Approximately 10-20% of patients with tuberous sclerosis develop subependymal giant cell astrocytoma. It is a disease that tends to commonly affect the pediatric population with males being more affected than females.

The most potent risk factor in the development of subependymal giant cell astrocytoma is tuberous sclerosis.

According to the International Tuberous Sclerosis Complex Consensus, screening for subependymal giant cell astrocytoma by MRI is recommended every 1-3 years among patients with tuberous sclerosis, even in the abscence of symptoms.

Subependymal giant cell astrocytoma is generally located in the caudothalamic groove adjacent to the foramen of Monro and it presents commonly in the first two decades of life. It can lead to a few complications such as obstructive hydrocephalus, intratumoral hemorrhage, and death. Although the prognosis may be poor, patients who undergo surgical resection and those below the age of 18 have a better prognosis.

There is no single diagnostic study of choice for the diagnosis of subependymal giant cell astrocytoma, but subependymal giant cell astrocytoma can be diagnosed based on contrast enhanced MRI and CT scan.

Patients with subependymal giant cell astrocytoma may have a positive history of tuberous sclerosis, seizures, and personality changes. Some common symptoms that may be present include headaches, nausea, vomiting, and cognitive decline.

Common physical examination findings in patients with subependymal giant cell astrocytoma include hypomelanotic macules, retinal hamartomas, sensory deficits, and muscle weakness. Because subependymal giant cell astrocytoma is predominantly seen in people with tuberous sclerosis, the examination findings listed are those seen in tuberous sclerosis patients.

There are no diagnostic lab findings associated with subependymal giant cell astrocytoma.

Head CT scan may be helpful in the diagnosis of subependymal giant cell astrocytoma. On head CT, some of the findings that are suggestive of subependymal giant cell astrocytoma include a heterogenous mass with uniform post contrast enhancement, enlargement of the ventricles, and iso- or slightly hypoattenuating to grey matter.

Brain MRI may be helpful in the diagnosis of subependymal giant cell astrocytoma. On MRI, some of the findings suggestive of subependymal giant cell astrocytoma include T1 isointense and hypointense signal enhancement, T2 isointense and hyperintense signal enhancement, and enlargement of ventricles.

There are no ultrasound findings associated with subependymal giant cell astrocytoma.

There are no other imaging findings associated with subependymal giant cell astrocytoma.

There are no other diagnostic studies associated with subependymal giant cell astrocytoma.

The mainstay therapy for subependymal giant cell astrocytoma is surgery, but medical therapy is preferred in some cases. Mammalian target of rapamycin (mTOR) inhibitors, everolimus and rapamycin, are the medications used. They are capable of reducing the size of the tumor and in some cases, the tumors grow back after upon cessation of use. The most common side effects associated with the use of mTOR inhibitors are stomatitis and upper respiratory tract infections.

The mainstay of treatment for subependymal giant cell astrocytoma is surgery with medical therapy used in some cases.

Surgery is the first line therapy for subependymal giant cell astrocytoma. It is preferably indicated in cases such as tumor growth, acute hydrocephalus, and worsened seizure burden. The tumors that have invaded neighboring structures, those located bilaterally, and growing residual tumors are difficult to treat surgically. Medical therapy is favored in these cases. Some of the complications of surgical resection include transient memory loss, infection, and death. Gamma knife radiosurgery may also be used to treat subependymal giant cell astrocytoma with the risk of causing radiation-induced secondary tumor.

There is no established method for prevention of subependymal giant cell astrocytoma.

Effective measures for the secondary prevention of subependymal giant cell astrocytoma include brain imaging, preferably magnetic resonance imaging with and without contrast, which should be performed every 1 to 3 years until the age of 25 years in every patient with tuberous sclerosis.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2], Ifeoma Odukwe, M.D. [3]

Subependymal giant cell astrocytoma was described as a lesion located in the caudothalamic groove having a size of >1 cm in any direction or a subependymal lesion that has shown serial growth on consecutive imaging regardless of size and location.

• In 2012, experts at the International Tuberous Sclerosis Complex Consensus Conference described subependymal giant cell atroctyoma as a lesion located in the caudothalamic groove having a size of >1 cm in any direction or a subependymal lesion that has shown serial growth on consecutive imaging regardless of size and location.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

There is no classification system established for subependymal giant cell astrocytoma.

There is no classification system established for subependymal giant cell astrocytoma.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2], Sujit Routray, M.D. [3]

Subependymal giant cell astrocytoma is almost exclusively associated with tuberous sclerosis complex, which is an autosomal dominant disorder. It is associated with inactivation of the tumor suppressor genes, TSC1 and/or TSC2. It is also believed to arise from a subependymal nodule present in the ventricular wall of a patient with tuberous sclerosis. Some of the common findings seen on microscopic pathology include pleomorphic multinuleated eosinophilic cells, streams of elongated tumor cells with abundant cytoplasm, and clustered cells arranged in a perivascular pseudopallisading pattern. On immunohistochemistry, the tumor cells are positive for glial fibrillary acidic protein, microtubule-associated protein 2, synaptophysin, S-100, neurofilament, and neuron-specific enolase.

• Subependymal giant cell astrocytoma is a rare, benign tumor predominantly associated with tuberous sclerosis complex, although a few cases have been reported in patients without evidence of tuberous sclerosis.^[1]
• It is classified as a WHO grade I central nervous system tumor.
• It is of glioneuronal origin and typically arises from the caudothalamic groove adjacent to the foramen of monro.^[2][3]
• The inactivation of the tumor suppressor genes TSC1 (on chromosome 9q34) and/or TSC2 (on chromosome 16p13) results in the formation of subependymal giant cell astrocytoma in people with tuberous sclerosis.^[1]
• TSC1 and TSC2 encodes the proteins tuberin and hamartin, respectively. The tuberin/hamartin complex suppresses Ras homolog enriched in brain (RHES) which functions as a direct activator of the mammalian target of rapamycin (mTOR). The complex also inhibits cyclin-dependent kinase inhibitor 1B, which regulates cell cycle progression. The activation of mTOR and progression of the cell cycle from the loss of upstream inhibition leads to protein translation, cell growth, and proliferation.^[1]
• It is believed that a subependymal nodule, which are common brain masses seen in tuberous sclerosis, can transform to subependymal giant cell astrocytoma.
• It is commonly located in the ventricles but a few may have extraventricular locations.^[2]
• Subependymal giant cell astrocytoma is a major cause of tuberous sclerosis complex-related morbidity and mortality during the pediatrics age, as it is seen in 10 to 20% of these patients.^[4]
• It is believed to arise from a subependymal nodule but this is controversial because subependymal giant cell astrocytomas are located in the caudothalamic groove while subependymal nodules are located in the ependymal lining of the lateral ventricles along the caudate nucleus.^[4]
• On immunohistochemistry, the tumor cells test positive for the glial fibrillary acidic protein and microtubule-associated protein 2.^[4]

Genes involved in the pathogenesis of subependymal giant cell astrocytoma include:^[5]

• TSC1
• TSC2

Conditions associated with subependymal giant cell astrocytoma include:^[5]

• Tuberous sclerosis

On microscopic histopathological analysis, subependymal giant cell astrocytoma is characterized by:^{[4][6][7][8][1][9][9]}

• Pleomorphic multinuleated eosinophilic cells
• Streams of elongated tumor cells with abundant cytoplasm
• Clustered cells arranged in a perivascular pseudopallisading pattern
• Evenly distributed granular chromatin
• Frequent binucleation and multinucleation
• Vesicular nuclei
• Occasional distinct to prominent nucleoli
• On rare occasions, there can be atypical features such as vascular endothelial proliferations, mitosis, and necrosis
• Tumor cells are positive on immunohistochemistry for glial fibrillary acidic protein, microtubule-associated protein 2, synaptophysin, S-100, neurofilament, and [[neuron-specific enolase

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2], Sujit Routray, M.D. [3]

Subependymal giant cell astrocytoma is predominantly seen in patients with tuberous sclerosis complex which is caused by a mutation in the TSC1 and TSC tumor suppressor genes.

Subependymal giant cell astrocytoma is predominantly seen in patients with tuberous sclerosis complex. Tuberous sclerosis is caused by a mutation in the TSC1 and TSC2 tumor suppressor genes on chromosome 9 and 16, respectively.^[1]

Cardiovascular	No underlying causes
Chemical/Poisoning	No underlying causes
Dental	No underlying causes
Dermatologic	No underlying causes
Drug Side Effect	No underlying causes
Ear Nose Throat	No underlying causes
Endocrine	No underlying causes
Environmental	No underlying causes
Gastroenterologic	No underlying causes
Genetic	TSC1 and TSC2 mutation on chromosome 9 and 16, respectively
Hematologic	No underlying causes
Iatrogenic	No underlying causes
Infectious Disease	No underlying causes
Musculoskeletal/Orthopedic	No underlying causes
Neurologic	No underlying causes
Nutritional/Metabolic	No underlying causes
Obstetric/Gynecologic	No underlying causes
Oncologic	No underlying causes
Ophthalmologic	No underlying causes
Overdose/Toxicity	No underlying causes
Psychiatric	No underlying causes
Pulmonary	No underlying causes
Renal/Electrolyte	No underlying causes
Rheumatology/Immunology/Allergy	No underlying causes
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Miscellaneous	No underlying causes

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D., Ifeoma Odukwe, M.D. [2], Sujit Routray, M.D. [3]

Subependymal giant cell astrocytoma must be differentiated from ependymoma, meningioma, tuberculoma, intraventricular hemorrhage, glioblastoma multiforme, primary CNS lymphoma, and cerebral metastases.

Diseases	Clinical manifestations					Para-clinical findings			Gold standard	Additional findings
	Symptoms				Physical examination
						Lab Findings	MRI	Immunohistopathology
	Head- ache	Seizure	Visual disturbance	Constitutional	Focal neurological deficit
Adult primary brain tumors
Glioblastoma multiforme [1][2][3]	+	+/−	+/−	−	+	−	Supratentorial Irregular ring-nodular enhancing lesions Central necrosis Surrounding vasogenic edema Cross corpus callosum (butterfly glioma)	Astrocyte origin Pleomorphic cell Pseudopalisading appearance GFAP + Necrosis + Hemorrhage + Vascular prolifration +	Biopsy	Highest incidence in fifth and sixth decades of life Most of the time, focal neurological deficit is the presenting sign.
Oligodendroglioma [4][5][6]	+	+	+/−	−	+	−	Almost always in cerebral hemisphers (frontal lobes) Hypointense on T1 Hyperintense on T2 Calcification Chicken wire capillary pattern	Oligodendrocyte origin Calcification + Fried egg cell appearance	Biopsy	Highest incidence is between 40 and 50 years of age. Most of the time, epileptic seizure is the presenting sign.
Meningioma [7][8][9]	+	+/−	+/−	−	+	−	Well circumscribed Extra-axial mass Dural attachment CSF vascular cleft sign Sunburst appearance of the vessels	Arachnoid origin Psammoma bodies Whorled spindle cell pattern	Biopsy	Highest incidence is between 40 and 50 years of age. Most of the time, focal neurological deficit and epileptic seizure are the presenting signs. May be associated with NF-2
Hemangioblastoma [10][11][12][13]	+	+/−	+/−	−	+	−	Infratentorial Cystic lesion with a solid enhancing mural nodule	Blood vessel origin Capillaries with thin walls	Biopsy	Might secret erythropoietin and cause polycythemia May be associated with von hippel-lindau syndrome
Pituitary adenoma [14][15][3]	−	−	+ Bitemporal hemianopia	−	−	Endocrine abnormalities as a result of functional adenomas or pressure effect of non-functional adenomas	Isointense to normal pituitary gland in T1	Endocrine cell hyperplasia	Biopsy	It is associated with MEN1 disease. Initialy presents with upper bitemporal quadrantanopsia followed by bitemporal hemianopsia (pressure on optic chiasma from below)
Schwannoma [16][17][18][19]	−	−	−	−	+	−	Split-fat sign Fascicular sign Often have areas of hemosiderin	Schwann cell origin S100+	Biopsy	It causes hearing loss and tinnitus May be associated with NF-2 (bilateral schwannomas)
Primary CNS lymphoma [20][21]	+	+/−	+/−	−	+	−	Usually deep in the white matter Single mass with ring enhancement	B cell origin Similar to non hodgkin lymphoma (diffuse large B cell)	Biopsy	Usually in young immunocompromised patients (HIV) or old immunocompetent person.
Childhood primary brain tumors
Pilocytic astrocytoma [22][23][24]	+	+/−	+/−	−	+	−	Infratentorial Solid and cystic component Mostly in posterior fossa Usually in cerebellar hemisphers and vermis	Glial cell origin Solid and cystic component GFAP +	Biopsy	Most of the time, cerebellar dysfunction is the presenting signs.
Medulloblastoma [25][26][27]	+	+/−	+/−	−	+	−	Infratentorial Mostly in cerebellum Non communicating hydrocephalus	Neuroectoderm origin Homer wright rosettes	Biopsy	Drop metastasis (metastasis through CSF)
Ependymoma [28][3]	+	+/−	+/−	−	+	−	Infratentorial Usually found in 4th ventricle Mixed cystic/solid lesion Hydrocephalus	Ependymal cell origin Perivascular pseudorosette	Biopsy	Causes an unusually persistent, continuous headache in children.
Craniopharyngioma [29][30][31][3]	+	+/−	+ Bitemporal hemianopia	−	+	Hypopituitarism as a result of pressure effect on pituitary gland	Calcification Lobulated contour Motor-oil like fluid within tumor	Ectodermal origin (Rathkes pouch) Calcification +	Biopsy	Initialy presents with lower bitemporal quadrantanopsia followed by bitemporal hemianopsia (pressure on optic chiasma from above)
Pinealoma [32][33][34]	+	+/−	+/−	−	+ vertical gaze palsy	B-hCG rise leads to precocious puberty in males	Hydrocephalus (compression of cerebral aqueduct)	Similar to testicular seminoma	Biopsy	May cause prinaud syndrome (vertical gaze palsy, pupillary light-near dissociation, lid retraction and convergence-retraction nystagmus
Vascular
AV malformation [35][36][3]	+	+	+/−	−	+/−	−	Supratentorial: ~85% Flow voids on T2 weighted images	We do not perform biopsy for AVM	Angiography	We may see bag of worms appearance in CT angiography
Brain aneurysm [37][38][39][40][41]	+	+/−	+/−	−	+/−	−	In magnetic resonance angiography, we may see aneurysm mostly in anterior circulation (~85%)	We do not perform biopsy for brain aneurysm	MRA and CTA	It is associated with autosomal dominant polycystic kidney disease, Ehlers-Danlos syndrome, pseudoxanthoma elasticum and Bicuspid aortic valve (Angiography is reserved for patients who have negative MRA and CTA)
Infectious
Bacterial brain abscess [42][43]	+	+/−	+/−	+	+	Leukocytosis Elevated ESR Blood culture may be positive for underlying organism	Central hypodense signal and surrounding ring-enhancement in T1 Central hyperintense area surrounded by a well-defined hypointense capsule with surrounding edema in T2	We do not perform biopsy for brain abscess	History/ imaging	The most common causes of brain abscess are Streptococcus and Staphylococcus.
Tuberculosis [44][3][45]	+	+/−	+/−	+	+	Positive acid-fast bacilli (AFB) smear in CSF specimen Positive CSF nucleic acid amplification testing Hyponatremia (inappropriate secretion of antidiuretic hormone) Mild anemia Leukocytosis	Hydrocephalus combined with marked basilar meningeal enhancement	We do not perform biopsy for brain tuberculosis	Lab data/ Imaging	It is associated with HIV infection
Toxoplasmosis [46][47]	+	+/−	+/−	−	+	Normal CSF	Multifocal masses with ring enhancement Mostly in basal ganglia, thalami, and corticomedullary junction.	We do not perform biopsy for brain toxoplasmosis	History/ imaging	It is associated with HIV infection
Hydatid cyst [48][3]	+	+/−	+/−	+/−	+	Positive serology (Antibody detection for E. granulosus)	Honeycomb appearance Necrotic area	We do not perform biopsy for hydatid cysts	Imaging	Brain, eye, and splenic cysts may not produce detectable amount of antibodies
CNS cryptococcosis [49]	+	+/−	+/−	+	+	Positive CSF antigen testing (coccidioidomycosis) CSF lymphocytic pleocytosis Elevated CSF proteins and lactate Low CSF glucose	Dilated perivascular spaces Basal ganglia pseudocysts Soap bubble brain lesions (cryptococcus neoformans)	We may see numerous acutely branching septate hyphae	Lab data/ Imaging	It is the most common brain fungal infection It is associated with HIV, immunosuppressive therapies, and organ transplants In may happen in immunocompetent patients undergoing invasive procedures ( neurosurgery) or exposed to contaminated devices or drugs Since brain biopsies are highly invasive and may may cause neurological deficits, we diagnose CNS fungal infections based on laboratory and imaging findings
CNS aspergillosis [50]	+	+/−	+/−	+	+	Positive galactomannan antigen testing (aspergillosis) CSF lymphocytic pleocytosis Elevated CSF proteins and lactate Low CSF glucose	Multiple abscesses Ring enhancement Peripheral low signal intensity on T2	We may see numerous acutely branching septate hyphae	Lab data/ Imaging	It is associated with HIV, immunosuppressive therapies, and organ transplants In may happen in immunocompetent patients undergoing invasive procedures ( neurosurgery) or exposed to contaminated devices or drugs Since brain biopsies are highly invasive and may may cause neurological deficits, we diagnose CNS fungal infections based on laboratory and imaging findings
Other
Subependymal giant cell astrocytoma [51][52]	+	+/−	+/−	–	+	−	T1 isointense and hypointense signal enhancement T2 isointense and hyperintense signal enhancement Homogenous postcontrast enhancement Enlargement of ventricles	Glial fibrillary acidic protein+ Microtubule-associated protein 2+ Pleomorphic multinuleated eosinophilic cells Streams of elongated tumor cells with abundant cytoplasm Clustered cells arranged in a perivascular pseudopallisading pattern	MRI	It is predominantly associated with tuberous sclerosis complex It commonly presents in the first two decades of life.
Brain metastasis [53][3]	+	+/−	+/−	+	+	−	Multiple lesions Vasogenic edema	Based on the primary cancer type we may have different immunohistopathology findings.	History/ imaging	Most common primary tumors that metastasis to brain: Lung cancer Renal cell carcinoma Breast cancer Melanoma Gastrointestinal tract If there is any uncertainty about etiology, biopsy should be performed

ABBREVIATIONS

CNS=Central nervous system, AV=Arteriovenous, CSF=Cerebrospinal fluid, NF-2=Neurofibromatosis type 2, MEN-1=Multiple endocrine neoplasia, GFAP=Glial fibrillary acidic protein, HIV=Human immunodeficiency virus, BhCG=Human chorionic gonadotropin, ESR=Erythrocyte sedimentation rate, AFB=Acid fast bacilli, MRA=Magnetic resonance angiography, CTA=CT angiography

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2], Sujit Routray, M.D. [3]

Subependymal giant cell astrocytoma is the most common central nervous system tumor in patients with tuberous sclerosis complex. Approximately 10-20% of patients with tuberous sclerosis develop subependymal giant cell astrocytoma. It is a disease that tends to commonly affect the pediatric population with males being more affected than females.

• The incidence of subependymal giant cell astrocytoma is approximately 0.027 per 100,000 person-years.^[1]

• Subependymal giant cell astrocytoma is the most common central nervous system tumor in patients with tuberous sclerosis complex.^[2]
• Approximately 10-20% of patients with tuberous sclerosis develop subependymal giant cell astrocytoma.^[3]

• Subependymal giant cell astrocytoma is a disease that tends to affect the pediatric, adolescent, and young adult population.^[4]
• The mean age at diagnosis is 13 years.^[5]
• It commonly presents in the first two decades of life.^[6]
• It becomes symptomatic, clinically and pathologically, between 8 to 19 years of age.^[2]

• There is no racial predilection to subependymal giant cell astrocytoma.

• Males are more commonly affected with subependymal giant cell astrocytoma than females.^[2][4][7]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2], Ifeoma Odukwe, M.D. [3]

The most potent risk factor in the development of subependymal giant cell astrocytoma is tuberous sclerosis.

The most potent risk factor in the development of subependymal giant cell astrocytoma is tuberous sclerosis.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2], Ifeoma Odukwe, M.D. [3]

According to the International Tuberous Sclerosis Complex Consensus, screening for subependymal giant cell astrocytoma by MRI is recommended every 1-3 years among patients with tuberous sclerosis, even in the abscence of symptoms.

According to the International Tuberous Sclerosis Complex Consensus, screening for subependymal giant cell astrocytoma by MRI is recommended every 1-3 years among patients with tuberous sclerosis, even in the abscence of symptoms.^[1][2]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2], Sujit Routray, M.D. [3]

Subependymal giant cell astrocytoma is generally located in the caudothalamic groove adjacent to the foramen of Monro and it presents commonly in the first two decades of life. It can lead to a few complications such as obstructive hydrocephalus, intratumoral hemorrhage, and death. Although the prognosis may be poor, patients who undergo surgical resection and those below the age of 18 have a better prognosis.

• Subependymal giant cell astrocytoma’s generally found in the caudothalamic groove adjacent to the foramen of Monro.^[1]
• It commonly presents in the first two decades of life.^[2]
• It is usually benign and slow growing but can progress to occluding the foramen of monro leading to obstructive hydrocephalus with symptoms of increased intracranial pressure.^[3]
• New tumors hardly arise after 20-25 years of age.^[1]

Common complications of subependymal giant cell astrocytoma include:^[4][5]

• Obstructive hydrocephalus
• Brain herniation
• Intratumoral hemorrhage
• Chronic ventriculoperitoneal shunt placement
• Stroke
• Sudden death

• Prognosis of subependymal giant cell astrocytoma is generally poor.^[6]
• It could be lethal, it is shown to be responsible for 25% of the excess mortality caused by the tuberous sclerosis complex.^[7]
• Surgical resection and age under 18 years are significant positive prognostic factors.^[8]
• Poor prognostic factors for subependymal giant cell astrocytoma include:^[1]

• Invasion to neighboring structures (fornix, hypothalamus, basal ganglia, or genu of internal capsule)
• Large sized tumors
• Recurrent tumors

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