Subependymal Giant Cell Astrocytoma

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Retrieved

2021-01-18

Source

Wikipedia

Trials

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Genes

BRAF, HGF, HIF1A, NOTCH2, LATS1, NDRG1, DLL3, PTPN11, HEY1, NTRK2, ACVR1, NOTCH1, NF1, HEY2, MMP9, MMP3, MET, KRAS, FGFR1, ESR2, ESR1, LATS2, KIAA1549, AR, NCOA3, TP53, TSC1, TSC2, MTOR, TESC

BRAF, HGF, HIF1A, NOTCH2, LATS1, NDRG1, DLL3, PTPN11, HEY1, NTRK2, ACVR1, NOTCH1, NF1, HEY2, MMP9, MMP3, MET, KRAS, FGFR1, ESR2, ESR1, LATS2, KIAA1549, AR, NCOA3, TP53, TSC1, TSC2, MTOR, TESC, SLC12A3, GLS2, UBASH3B, A1BG, VEGFA, NKX2-1, PKD1, MYC, DPYSL3, DCX, CDK1, ATRX, ANXA6, MIR21

Drugs

(S)-perillyl alcohol temozolomide, 1,2:5,6-Dianhydrogalactitol, 18-(p-[124I]-iodophenyl)octadecyl phosphocholine

(S)-perillyl alcohol temozolomide, 1,2:5,6-Dianhydrogalactitol, 18-(p-[124I]-iodophenyl)octadecyl phosphocholine, 2-hydroxyoleic acid, 4,6,8-trihydroxy-10-(3,7,11-trimethyldodeca-2,6,10-trienyl)-5,10-dihydrodibenzo[b,e][1,4] diazepin-11-one, 4-[123I] iodo-L-phenylalanine, 4-[131I] iodo-L-phenylalanine, 5-aminolevulinic acid hydrochloride ( GLEOLAN, GLIOLAN ), 7-beta-hydroxycholesteryl-3-beta-oleate, Adenoviral vector serotype 5 encoding the human interleukin-12 p70 transgene, Adenovirus serotype 5 containing partial E1A deletion and an integrin-binding domain, Adenovirus-associated vector containing human Fas-c gene, Adenovirus-mediated Herpes Simplex Virus-thymidine kinase (HKSV-tk) gene ( CEREPRO ), Allogeneic and autologous haptenised and irradiated cells and cell lysates derived from glioma, Asunercept, Autologous Tumor-Derived gp96 Heat Shock Protein-Peptide Complex ( ONCOPHAGE ), Autologous dendritic cells pulsed with RNA from glioma stem cells, Autologous dendritic cells pulsed with autologous cell lysate, Autologous dendritic cells pulsed with tumour antigen-derived synthetic peptides (MAGE-1, HER-2, AIM-2, TRP-2, gp-100, and interleukin-13 receptor alpha), Autologous ex-vivo-expanded leucocytes treated with 5-aza-2'-deoxycytidine, Autologous glioma tumour cells treated with antisense molecule directed against the insulin-like growth factor type 1 receptor, Biotinylated anti-tenascin monoclonal antibody for use with 90-Yttrium, Boswellia serrata resin dry extract, Cannabidiol ( EPIDIOLEX, EPIDYOLEX ), Carmustine ( GLIADEL, BICNU, CARMUSTINE OBVIUS ), Carmustine (solution for intratumoral injection), Cilengitide, Combination of carboplatin and sodium valproate, Delta-9-tetrahydrocannabinol and cannabidiol from extracts of the Cannabis sativa L. plant, Doxorubicin (administered after synthetic double-stranded siRNA oligonucleotide directed against claudin-5 complexed with polyethyleneimine), Doxorubicin hydrochloride (drug eluting beads), Dronabinol and cannabidiol, Eflornithine, Enzastaurin hydrochloride, Florilglutamic acid (18F), Fluciclovine (18F), Flucytosine ( ANCOTIL ), Gadodiamide (liposomal), Galunisertib, Gimatecan, Glutathione-pegylated liposomal doxorubicin hydrochloride, H-Arg-Pro-Lys-Pro-Gln-Gln-Phe-2Thi-Gly-Leu-Met(O2)-NH2-DOTA-213-bismuth, H-Arg-Pro-Lys-Pro-Gln-Gln-Phe-2Thi-Gly-Leu-Met(O2)-NH2-DOTA-225-Actinium, Herpes simplex virus lacking infected cell protein 34.5, Human transferrin conjugated to mutant diphtheria toxin, Humanised monoclonal antibody against epidermal growth factor receptor, Humanised recombinant monoclonal antibody against epidermal growth factor receptor conjugated to maleimidocaproyl monomethylauristatin F, IL-12-secreting dendritic cells, loaded with autologous tumour lysate, Iodine (131I) anti-nucleohistone H1 chimeric biotinylated monoclonal antibody, Iodine (131I) anti-tenascin monoclonal antibody 81C6, Iodine (131I) chlorotoxin, Irinotecan hydrochloride (drug eluting beads), L-cysteine, L-leucyl-L-alpha-glutamyl-L-alpha-glutamyl-L-lysyl-L-lysylglycyl-L-asparaginyl-L-tyrosyl-L-valyl-L-valyl-L-threonyl-L-alpha-aspartyl-L-histidyl-S-[1-[(4-carboxycyclohexyl)methyl]-2,5-dioxo-3-pyrrolidinyl]-complex with keyhole limpet haemocyanin, Larotrectinib ( VITRAKVI ), Marizomib, N-(4-Methoxyphenyl)-N,2,6-trimethylfuro[2,3-d]pyrimidin-4-amine, Nimotuzumab ( THERALOC ), Oligonucleotide phosphorothioate (TAAACGTTATAACGTTATGACGTCAT), Onfekafusp alfa, Paclitaxel-succinate-Arg-Arg-Leu-Ser-Tyr-Ser-Arg-Arg-Arg-Phe, Picropodophyllin, Pritumumab, Pseudomonas exotoxin (domains II/III)-Interleukin 13 chimeric protein, Recombinant fusion protein of circulary-permuted IL-4 and pseudomonas exotoxin A, [IL-4(38-37)-PE38KDEL], Recombinant human bone morphogenetic protein 4, Regorafenib ( STIVARGA ), Salmonella typhi Ty21a strain transfected with a plasmid vector encoding the human vascular endothelial growth factor receptor 2, Synthetic double-stranded siRNA oligonucleotide directed against claudin-5 complexed with polyethyleneimine (prior to administration of doxorubicin), Talampanel, Technetium (99mTc) tetrofosmin, Temozolomide ( TEMODAL, TEMODAR ), Topotecan hydrochloride (liposomal), Trabedersen, Veledimex, Vocimagene amiretrorepvec, Zoledronic acid, Zotiraciclib

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Subependymal giant cell astrocytoma (SEGA, SGCA, or SGCT) is a low-grade astrocytic brain tumor (astrocytoma) that arises within the ventricles of the brain. It is most commonly associated with tuberous sclerosis complex (TSC). Although it is a low-grade tumor, its location can potentially obstruct the ventricles and lead to hydrocephalus.

Signs and symptoms

Individuals with this type of tumor may have no symptoms if cerebrospinal fluid (CSF) flow remains open. Obstruction of CSF flow will result in the symptoms associated with increased CSF pressure: nausea, vomiting, headache (often positional), lethargy, blurry or double vision, new or worsened seizures, and personality change.

Diagnosis

MRI of brain with sub-ependymal giant cell astrocytoma

Diagnosis is made by imaging with a contrast-enhanced MRI or CT scan of the brain.

Screening

It is recommended that children with TSC be screened for SEGA with neuroimaging every 1–3 years.

Treatment

Pharmacotherapy

Two related drugs have been shown to shrink or stabilize subependymal giant cell tumors: rapamycin and everolimus. These both belong to the mTOR inhibitor class of immunosuppressants, and are both contraindicated in patients with severe infections.

Rapamycin showed efficacy in five cases of SEGA in TSC patients, shrinking their tumor volumes by an average of 65%. However, after the drug was stopped, the tumors regrew.

Everolimus, which has a similar structure as rapamycin, but with slightly increased bioavailability and shorter half-life, was studied in 28 patients with SEGA. There was a significant reduction in SEGA size in 75% of the patients, and a mild improvement in their seizures. Everolimus was approved for the treatment of SEGA by the US Food and Drug Administration (FDA) in October, 2010.

Surgery

Photograph of a brain section containing a subependymal giant cell astrocytoma

A NIH Consensus Conference report in 1999 recommends that any SEGA that is growing or causing symptoms should be surgically removed. Tumors are also removed in cases where a patient is suffering from a high seizure burden. If a tumor is rapidly growing or causing symptoms of hydrocephalus, deferring surgery may lead to vision loss, need for ventricular shunt, and ultimately death. Total removal of the tumor is curative.

Surgery to remove intraventricular tumors also carries risks of complications or death. Potential complications include transient memory impairment, hemiparesis, infection, chronic ventriculoperitoneal shunt placement, stroke, and death.

Prognosis

After complete surgical removal, a SEGA tumor does not grow back. They do not metastasize to other parts of the body. However, the patient is still at risk for, and often develops, new tumors arising from subependymal nodules elsewhere in the ventricular system.

Epidemiology

SEGAs arise in 5-20% of TSC patients.