Astrocytoma

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2021-01-23

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Genes

NF1, FGFR1, PTPN11, TP53, BRAF, MMP9, HIF1A, MET, HGF, ESR1, MMP3, KIAA1549, NCOA3, NOTCH1, KRAS, LATS2, ACVR1, NOTCH2, LATS1, NTRK2, HEY1, NDRG1, HEY2, AR, DLL3, ESR2, HRAS, CDKN2A, IDH2, IDH1

NF1, FGFR1, PTPN11, TP53, BRAF, MMP9, HIF1A, MET, HGF, ESR1, MMP3, KIAA1549, NCOA3, NOTCH1, KRAS, LATS2, ACVR1, NOTCH2, LATS1, NTRK2, HEY1, NDRG1, HEY2, AR, DLL3, ESR2, HRAS, CDKN2A, IDH2, IDH1, IFNG, TSC2, H3-3A, TSC1, ERBB2, NF2, MLH1, MSH3, MSH2, MSH6, VEGFA, MIB1, TNF, PTEN, PMS2, BRCA2, CTNNB1, H3P10, PDGFRA, AKT1, EGF, GFAP, ATRX, EGFR, POT1, APC, TAC1, IL1B, IL6, CXCL8, MGMT, MMP2, CCND1, MKI67, PPP1R2C, STAT3, CCL2, OLIG2, GJA1, IL1A, MTOR, CDK4, CDK2, ACTB, NGF, H3-3B, PITX1, IGFBP2, ICAM1, BCL2, APOE, SLC2A1, CXCR4, AQP4, TIMP1, OSM, PIK3CA, CDKN2B, GDNF, KDR, MAPK1, LOC110806263, GSTM1, FGF2, CD44, ARHGAP24, CHI3L1, CAV1, ANGPT1, DMBT1, IFNB1, NOS2, SERPINE1, MDM2, PSEN1, ADARB1, EPHB2, PTGS2, PAX6, PCNA, BDNF, PGR, SOX2, NES, TACR1, NOS1, ABCB1, ANXA1, ITIH4, TP73, CCL5, HSPA5, IGF1, S100A4, IL13RA2, CIC, GSTP1, RAC1, MDM4, CDKN1B, TERT, FGFR4, HPGDS, TNC, NPM1, NUP98, NXT1, MLC1, TM7SF2, MRPL28, SRC, ALK, CXCL12, FPR2, ATM, TXN, CXCL10, H3P9, HFE, PRND, TGFA, NEFL, APP, MYC, SUB1, EZH2, GSTT1, FN1, ABCC1, MRC1, AQP1, GAPDH, EGR1, PTHLH, WLS, TNKS, NCOA1, PLG, PLA2G10, PROM1, KLF6, GADL1, PFKFB3, MAPK14, MAPK3, PLA2G2A, AZIN2, SAI1, SPARC, PTN, PEA15, LGALS3, VHL, RAF1, NAT2, SLC6A8, TBC1D9, JAK2, PTK2, POLDIP2, VCAM1, UBE2N, ADM, RNF19A, SLC1A2, PTPN6, LAMP1, P2RX4, SPAG9, HSPB3, ABCC3, TNFRSF1A, IFI27, SFRP1, SETD2, TNFSF10, TNFRSF6B, S100B, TLR3, RGCC, S100A13, IGFBP3, NRP1, TGFBI, IL4, GPR162, ARHGEF7, SPHK1, GLS2, RGS2, TGFB1, MAP2, ILK, RASGRF1, PSMD9, NT5C2, AHSA1, TAT, DCTN6, PIK3CB, YWHAZ, SOX10, SERPINE2, ID4, NFE2L2, NFKB1, SOX11, PECAM1, SNCA, AIMP2, PDGFA, ZNRD2, SLC7A5, NOS3, H3P23, NPY, SPP1, CIB1, P2RY1, XRCC4, NR1I3, PIK3CD, PIK3CG, MBP, ZHX2, NTRK1, AURKB, PROX1, GRAP2, MAPK8, CD99, WIF1, EZR, VIM, MMP1, VTN, RASSF1, PLAU, MMP14, DSTN, PLA2G4A, XRCC3, GLIPR1, MVP, BECN1, H3P40, VCAN, CDKN1A, DSC3, CD40, CD47, GAST, MIR183, MARCKSL1, CD63, RTEL1, AXL, DNMT1, GSTK1, ASCL1, MIR221, ZMYND10, GRIA2, BSG, CCDC88A, EDN1, CALCR, CASR, TICAM2, NDRG2, CASP3, SLC12A9, CALR, CACNA1F, TMED7-TICAM2, CCK, CA9, CCND2, F3, FABP7, MIR106A, PDIK1L, CDH1, CSF2, GSN, CSF3, ANGPT2, TMED7, ASCC1, FOXP3, MAP1LC3B, CRK, GSTM3, COX8A, CCR5, STARD13, CNTF, SIRPA, GSK3B, CRABP2, HES1, HSPB1, HSPE1, HSPB2, SLCO6A1, EMC10, CDK5R1, EGLN3, TSC22D1, ZAR1, HDAC3, NANOS3, AIM2, IQGAP1, NAPSA, MACC1, TNFRSF10B, ATG5, ASPM, TNFRSF11A, QKI, TNFRSF10A, DIRAS1, TAC4, DOK2, LGI1, PLB1, SLC5A8, PLK5, EXO1, NEURL1, PDCD5, AIFM1, SLC16A3, SLC16A4, CITED4, FOLH1B, PTTG1, SGPL1, ADGRG1, RICTOR, SEMA5A, ELFN2, SOCS3, KLF4, CDCA5, NCR1, BTRC, HJV, SNURF, FUBP1, RIPK2, AKR1C3, HES5, UCN, MIR451A, MIR370, MIR335, VEGFB, MIR328, XRCC6P5, MIR17HG, TRPV1, MIR93, XK, MIR25, MIR224, MIR223, XRCC5, MIR22, VAV2, MIR146B, SOHLH1, TYK2, CDKN2B-AS1, ESRG, MIR770, POTEF, PPAN-P2RY11, TPM3, TRAF3, TRH, MIR542, CXADRP1, TWIST1, POU5F1P4, RASSF10, POU5F1P3, MIR497, ZAP70, ZFP36, MIR214, ST8SIA4, CUL3, ATRN, RGS5, PPM1D, CDC14B, MIR132, MKNK1, AKR7A2, MIR128-1, AKAP12, MIR100, SOCS1, EIF3A, MIRLET7G, HRK, RECK, MIR137, NR0B2, ST8SIA2, MIR21, ADAM12, FOSL1, YEATS4, MIR206, MIR195, MIR184, MIR144, MIR17, MIR146A, CD24, LZTR1, USP9X, RBM10, PTGES, PYCARD, RGS6, PID1, CT55, FOCAD, TERF2IP, MMRN1, CD93, TLR9, TPX2, SHC3, NCOA6, RAB23, ACSL5, RASD1, PLCB1, PHLPP1, CAMTA1, NLRP1, ATF5, SIRT1, NEIL3, SPHK2, LINC00470, UBE2C, RAPGEF4, ADAMTS13, PTPRT, CD160, BCCIP, MMP26, MIR4758, PPAN, PCDHGA11, AP5M1, HES6, IMP3, PSD3, GDE1, LHX9, HSPA14, DKK3, LEF1, STARD13-AS, UPK3B, ING4, TFB1M, TNFRSF21, NOX4, NT5C, RBMX, RABGEF1, UBQLN1, SENP1, HIPK2, UHRF1, B3GAT1, ANKRD1, PHF3, NUSAP1, RNF138, PPME1, DNPEP, SH3BP4, MTCO2P12, LSM4, CHST15, PRDX5, WT1-AS, TLR7, PHF20, PHGDH, MYCBP, GNL3, TOP3A, BDH2, METAP2, HDAC9, HDAC5, PDGFD, DNM1L, PTPRU, MINDY3, OPTN, DHDDS, G3BP1, NANOG, MAP9, TRIM13, MCPH1, BIRC7, AKR1A1, TLR6, TUBB3, COL18A1, TANK, WNK1, AKT3, ATP23, PIEZO1, RB1CC1, MELK, AP5Z1, LMLN, KIF14, TMPRSS13, CTNNA3, ATAD3B, SESN2, RASSF5, NR1D2, KAZALD1, WNT5B, YAP1, PRMT5, CHPT1, NUFIP2, PDPN, GPR158, RASL10A, ZMIZ1, SPINT2, MIR3189, CD248, ACKR3, CYSLTR1, ALDH1L1, TUBGCP2, PPP1R13L, HPSE, BLCAP, FASTK, IVNS1ABP, MXD4, TUBGCP3, SEMA6A, RTN4R, P2RY12, GORASP1, LRRN2, CPEB1, HOXB13, ARHGEF28, TFB2M, XYLT1, GOLPH3, NPAS3, PRDM16, TRPV4, NLRC4, HAMP, HOTAIR, A2M, TOP2B, PTK2B, EPAS1, EPHA8, ERBB3, ETS1, EXT1, EYA2, EZH1, F2RL2, FABP5, FKTN, FOSB, FCN2, FEN1, FGFR3, FGFR2, FLI1, FLII, FLT1, FMR1, FOLH1, ENPEP, EMP3, ELK1, EIF4E, DECR1, DES, DIAPH2, DNASE1, DNMT3A, DNMT3B, DRD1, DRD2, ATN1, DUT, DVL3, E2F1, E2F4, ECT2, EDNRA, EDNRB, EFNB1, EFNB2, EPHA2, FOS, FTH1, DCX, IGF1R, HOXD9, HOXD13, HRH1, HSPD1, HTC2, HTR1B, IFI35, IFIT2, IFIT1, IGF2, GABPA, IGFALS, IGFBP4, IL1RN, IL4R, CXCR2, IL12A, IL13, IL17A, ING1, HOXA9, HMOX1, HMMR, HMGA1, GAP43, GATA4, GATA6, GCHFR, GEM, GCLC, GLG1, GLI2, GOLGA4, CXCR3, GRM2, GRM3, GRM5, CXCL1, H2AX, HAS2, HELLS, HLA-DRB4, HLA-G, DMXL1, BRINP1, JUN, AREG, ANXA2, AOX1, BIRC5, AQP8, APOD, APRT, FAS, FASLG, AQP9, RHOA, BST2, ARR3, STS, ATP1B2, ATP2A2, B2M, BAX, BCL2L1, OPN1SW, BMI1, ANPEP, ANG, AMPD1, ALOX5, ACHE, ACTN4, ACTN1, ADAR, ADARB2, ADCY1, ADH1A, ADH1B, ADH1C, ADH4, ADH6, ADK, PARP1, ADRB2, AGT, AHSG, AIF1, AKT2, AKR1B1, BMP1, TSPO, CD55, CS, CETN3, CLU, CCR3, CNP, CNR1, CNR2, COL6A1, CPE, CREB1, CSF3R, C2, CST3, CSTB, CTH, CTNNA1, CTSD, CX3CR1, CXADR, CYP1B1, CYP2D6, CETN2, CDKN3, CDK9, CDK5, C3, VPS51, CA8, CA12, CASP9, CAT, RUNX1, RUNX3, SERPINA6, CCNG2, CCNT1, CCT, CD28, CD34, SCARB2, CD59, CDC20, CDC42, CDH11, IRF7, JUNB, TOP2A, SATB1, RNPEP, ROS1, RPLP0, RPS6, RPS15, RYR2, S100A9, SAA1, SAA2, CCL14, SLC6A12, CXCL6, SDC4, SDHA, SCG5, SIAH1, SIX1, SIX3, SKP2, SLC2A3, BRD2, RIEG2, RGS4, RGS3, KLK6, PSMA5, PTGER3, PTPN13, PTPRA, PTPRZ1, PVT1, PXN, RAB27B, RAC2, RAD51, RAP1A, RARB, PLAAT4, RASA1, RBM3, RBP2, REG1A, RELA, SLC5A2, SMARCA1, PRNP, TFAP2A, TBX2, TBXA2R, TCF4, HNF1A, TCF7, TCF7L2, TRBV20OR9-2, TEK, TERC, NR2F2, SMARCA2, TFR2, TFRC, TGFB1I1, TIMP2, TIMP4, NKX2-1, TLE2, TLR1, TLR2, TAZ, CNTN2, TARBP2P1, TARBP2, SMARCA4, SMN1, SMN2, SNRPN, SOD1, SOD2, SOS1, SOX4, SP1, SPAST, SPG7, RO60, STAT5A, STAT5B, STAT6, AURKA, STX1A, SULT2A1, SYT1, PRPS1, MAP2K7, JUND, CD200, MDK, MFAP1, CIITA, MIP, MMP7, MMP15, MMP16, MMP19, MNAT1, MPST, NBN, MSMB, MST1, MT1A, COX2, MUC1, MXI1, MYB, MYBL1, MYCN, CD46, MCL1, MATN2, MATK, KCNA5, KCNE1, KCNH2, KPNA2, RPSA, STMN1, LDLR, LEP, LGALS4, LIF, LNPEP, LOX, LPA, LPP, LRP1, LSP1, LTA, CYP4F3, MXD1, NAP1L1, NCK1, MAP2K1, PLD2, ENPP1, PFKFB4, PGAM1, PGK1, SLC25A3, PIM1, PIK3R1, PLA2G1B, PLD1, PLEC, NCL, PLP1, SEPTIN4, PODXL, PON1, POU5F1, PPP1R1A, PRKAR1A, PRKCA, PRKDC, PDGFRB, PDGFB, PDE3B, PAX5, SEPTIN2, NEU1, NFIA, NFATC3, NFIB, NGFR, NKX2-2, NME1, CCN3, NOVA1, NPPB, P2RX3, P2RX7, P2RY2, P2RY6, P2RY11, PEBP1, PRDX1, PAX3, RAC3

Drugs

(3R,3aS,9R,9aS,9bS)-3-((dimethylamino)methyl)-9-hydroxy-6,9-dimethyl-3,3a,4,5,7,8,9,9a-octahydroazuleno[4,5-b]furan-2(9bH)-one fumarate, (S)-perillyl alcohol temozolomide, 1,2:5,6-Dianhydrogalactitol

(3R,3aS,9R,9aS,9bS)-3-((dimethylamino)methyl)-9-hydroxy-6,9-dimethyl-3,3a,4,5,7,8,9,9a-octahydroazuleno[4,5-b]furan-2(9bH)-one fumarate, (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, 5,5'-(4-(trifluromethyl)benzylazanediyl)bis(methylene)diquinolin-8-ol, 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 ), Albumin-binding prodrug of doxorubicin, Allogeneic and autologous haptenised and irradiated cells and cell lysates derived from glioma, Altiratinib, Anti-beta1 integrin monoclonal antibody, 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, Bevacizumab ( Equidacent, AVASTIN, Aybintio, MVASI ), 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, Diaziquone, 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 ), Fluorouracil, 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 ), Olaptesed pegol, 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, Recombinant human glutamate oxaloacetate transaminase 1, 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, Tranilast, Veledimex, Vocimagene amiretrorepvec, Zoledronic acid, Zotiraciclib, [a-N-(2'succinyl-paclitaxel)Thr]-Phe-Phe-Tyr-Gly-Gly-Ser-Arg-Gly-[epsilon-N-(2'succinyl-paclitaxel)Lys]-Arg-Asn-Asn-Phe-[epsilon-N-(2'succinyl-paclitaxel)Lys]-Thr-Glu-Glu-Tyr

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A complex group of benign and malignant cerebral tumors arising at any age.

Epidemiology

They are the most frequent cerebral tumors and represent more than half of all primary brain tumors. Incidence is estimated at 1/12,500.

Clinical description

The most frequent benign tumors are juvenile pilocytic astrocytomas (grade I) and diffuse low grade or fibrillary astrocytomas (grade II). The most frequent malignant tumors include anaplastic astrocytomas (grade III), glioblastomas (grade IV, the most severe form of astrocytoma; see this term), giant cell glioblastomas and gliosarcomas. Pleomorphic xanthoastrocytomas can be malignant or benign. These tumors occur at all ages although glioblastomas are more frequent in adults and in elderly people, while pilocytic astrocytomas are more frequent in children and adolescents. Age at diagnosis may influence tumor biology and evolution; the outcome generally being better in children. Speed of tumor growth is related to tumor grade. Symptom duration of less than three months is usually seen with malignant tumors.

Etiology

Apart from cranial irradiation, which may itself induce glioblastomas, the underlying causes of these tumors are not known. The principal genetic predisposing syndromes are: neurofibromatosis type 1, Turcot syndrome and Li-Fraumeni syndrome (see these terms). Familial cases of isolated astrocytomas are very rare.

Diagnostic methods

Diagnosis is made after MRI, which reveals an intraparenchymal lesion associated with a mass effect and compression of the normal structures.

Management and treatment

Treatment first consists of surgery, which aims at removing as much of the tumor as possible. If complete, surgical resection may be the only treatment required for benign astrocytomas. Adjuvant treatment is required for malignant tumors, whatever the quality of the resection. In case of incomplete resection of a benign tumor, adjuvant treatment may also be discussed. Radiation therapy is usually used in adults and in children over 10 years of age. Chemotherapy is preferred for younger children and may even be discussed for adults. The most chemosensitive tumors are pilocytic astrocytomas and anaplastic astrocytomas. Pilocytic tumors are highly curable, while malignant tumors are often fatal. The neurologic and intellectual sequelae of these tumors and their treatment may be severe, especially in young children, elderly people and patients requiring extended irradiation. Patient management requires multidisciplinary teams with expertise in the field of brain neoplasms. The relative rarity of these tumors calls for the management of patients in prospective trials aimed at improving prognosis and quality of survival.

Prognosis

Prognosis depends on tumor histology (benign or malignant) and location (operable or not).