Raras
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Glioblastoma de células gigantes
ORPHA:251579CID-10 · C71.9CID-11 · 2A00.00DOENÇA RARA

Uma variação rara de glioblastoma (um tipo de câncer cerebral de grau 4, o mais agressivo, de acordo com a OMS), caracterizada pela predominância de células gigantes com aparência estranha e múltiplos núcleos. Às vezes, também apresenta uma rede abundante de fibras de suporte (reticulina) e é comum encontrar mutações (alterações) no gene TP53.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Uma variação rara de glioblastoma (um tipo de câncer cerebral de grau 4, o mais agressivo, de acordo com a OMS), caracterizada pela predominância de células gigantes com aparência estranha e múltiplos núcleos. Às vezes, também apresenta uma rede abundante de fibras de suporte (reticulina) e é comum encontrar mutações (alterações) no gene TP53.

Pesquisas ativas
1 ensaio
341 total registrados no ClinicalTrials.gov
Publicações científicas
143 artigos
Último publicado: 2026 Mar 5

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.02
Europe
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C71.9
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico143PubMed
Últimos 10 anos58publicações
Pico20217 papers
Linha do tempo
2026Hoje · 2026🧪 1991Primeiro ensaio clínico📈 2021Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

13 genes identificados com associação a esta condição. Padrão de herança: Not applicable.

PPARGPeroxisome proliferator-activated receptor gammaBiomarker tested inModerado
FUNÇÃO

Ligand-activated transcription factor that forms obligate heterodimers with the retinoic acid receptor and acts as a key regulator of biological processes, such as adipocyte differentiation, lipid metabolism, glucose homeostasis and beta-oxidation of fatty acids (PubMed:16150867, PubMed:20829347, PubMed:23525231, PubMed:8702406, PubMed:8706692, PubMed:9065481). Activated by lipid ligands: binds peroxisome proliferators, such as hypolipidemic drugs, and fatty acids, such as prostaglandin J2 metab

LOCALIZAÇÃO

NucleusCytoplasm

VIAS BIOLÓGICAS (7)
Regulation of PTEN gene transcriptionTranscriptional regulation of white adipocyte differentiationPPARA activates gene expressionMECP2 regulates transcription factorsNuclear Receptor transcription pathway
EXPRESSÃO TECIDUAL(Ubíquo)
Tecido adiposo
111.2 TPM
Adipose Visceral Omentum
100.8 TPM
Mama
71.3 TPM
Cólon transverso
21.6 TPM
Fibroblastos
20.4 TPM
OUTRAS DOENÇAS (6)
type 2 diabetes mellitusPPARG-related familial partial lipodystrophyinherited obesitygliosarcoma
HGNC:9236UniProt:P37231
ROS1Proto-oncogene tyrosine-protein kinase ROSPart of a fusion gene inTolerante
FUNÇÃO

Receptor tyrosine kinase (RTK) that plays a role in epithelial cell differentiation and regionalization of the proximal epididymal epithelium. NELL2 is an endogenous ligand for ROS1. Upon endogenous stimulation by NELL2, ROS1 activates the intracellular signaling pathway and triggers epididymal epithelial differentiation and subsequent sperm maturation (By similarity). May activate several downstream signaling pathways related to cell differentiation, proliferation, growth and survival including

LOCALIZAÇÃO

Cell membrane

EXPRESSÃO TECIDUAL(Tecido-específico)
Pulmão
11.1 TPM
Fibroblastos
3.0 TPM
Testículo
1.0 TPM
Córtex cerebral
0.8 TPM
Brain Frontal Cortex BA9
0.8 TPM
OUTRAS DOENÇAS (2)
cholangiocarcinomagiant cell glioblastoma
HGNC:10261UniProt:P08922
IDH1Isocitrate dehydrogenase [NADP] cytoplasmicBiomarker tested inTolerante
FUNÇÃO

Catalyzes the NADP(+)-dependent oxidative decarboxylation of isocitrate (D-threo-isocitrate) to 2-ketoglutarate (2-oxoglutarate), which is required by other enzymes such as the phytanoyl-CoA dioxygenase (PubMed:10521434, PubMed:19935646). Plays a critical role in the generation of NADPH, an important cofactor in many biosynthesis pathways (PubMed:10521434). May act as a corneal epithelial crystallin and may be involved in maintaining corneal epithelial transparency (By similarity)

LOCALIZAÇÃO

Cytoplasm, cytosolPeroxisome

VIAS BIOLÓGICAS (3)
NADPH regenerationNFE2L2 regulating TCA cycle genesPeroxisomal protein import
MECANISMO DE DOENÇA

Glioma

Gliomas are benign or malignant central nervous system neoplasms derived from glial cells. They comprise astrocytomas and glioblastoma multiforme that are derived from astrocytes, oligodendrogliomas derived from oligodendrocytes and ependymomas derived from ependymocytes.

EXPRESSÃO TECIDUAL(Ubíquo)
Glândula adrenal
266.5 TPM
Fígado
100.4 TPM
Esôfago - Mucosa
99.8 TPM
Tecido adiposo
81.8 TPM
Próstata
77.7 TPM
OUTRAS DOENÇAS (7)
Maffucci syndromemetaphyseal chondromatosis with D-2-hydroxyglutaric aciduriaacute myeloid leukemia with multilineage dysplasiaOllier disease
HGNC:5382UniProt:O75874
FGFR3Fibroblast growth factor receptor 3Part of a fusion gene inAltamente restrito
FUNÇÃO

Tyrosine-protein kinase that acts as a cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of cell proliferation, differentiation and apoptosis. Plays an essential role in the regulation of chondrocyte differentiation, proliferation and apoptosis, and is required for normal skeleton development. Regulates both osteogenesis and postnatal bone mineralization by osteoblasts. Promotes apoptosis in chondrocytes, but can also promote cancer cell proliferat

LOCALIZAÇÃO

Cell membraneCytoplasmic vesicleEndoplasmic reticulumSecreted

VIAS BIOLÓGICAS (2)
Signaling by FGFR3 in diseaset(4;14) translocations of FGFR3
MECANISMO DE DOENÇA

Achondroplasia

A frequent form of short-limb dwarfism. It is characterized by a long, narrow trunk, short extremities, particularly in the proximal (rhizomelic) segments, a large head with frontal bossing, hypoplasia of the midface and a trident configuration of the hands. ACH is an autosomal dominant disease.

EXPRESSÃO TECIDUAL(Ubíquo)
Skin Not Sun Exposed Suprapubic
364.6 TPM
Skin Sun Exposed Lower leg
356.5 TPM
Esôfago - Mucosa
199.7 TPM
Brain Caudate basal ganglia
148.4 TPM
Brain Nucleus accumbens basal ganglia
135.4 TPM
OUTRAS DOENÇAS (19)
nevus, epidermalsevere achondroplasia-developmental delay-acanthosis nigricans syndromelacrimoauriculodentodigital syndrome 2testicular germ cell tumor
HGNC:3690UniProt:P22607
MGMTMethylated-DNA--protein-cysteine methyltransferaseBiomarker tested inTolerante
FUNÇÃO

Involved in the cellular defense against the biological effects of O6-methylguanine (O6-MeG) and O4-methylthymine (O4-MeT) in DNA. Repairs the methylated nucleobase in DNA by stoichiometrically transferring the methyl group to a cysteine residue in the enzyme. This is a suicide reaction: the enzyme is irreversibly inactivated

LOCALIZAÇÃO

Nucleus

VIAS BIOLÓGICAS (1)
MGMT-mediated DNA damage reversal
EXPRESSÃO TECIDUAL(Ubíquo)
Fígado
38.0 TPM
Testículo
25.7 TPM
Tecido adiposo
25.1 TPM
Mama
24.2 TPM
Bladder
23.6 TPM
OUTRAS DOENÇAS (3)
gliosarcomafamilial melanomagiant cell glioblastoma
HGNC:7059UniProt:P16455
TACC3Transforming acidic coiled-coil-containing protein 3Part of a fusion gene inTolerante
FUNÇÃO

Plays a role in the microtubule-dependent coupling of the nucleus and the centrosome. Involved in the processes that regulate centrosome-mediated interkinetic nuclear migration (INM) of neural progenitors (By similarity). Acts as a component of the TACC3/ch-TOG/clathrin complex proposed to contribute to stabilization of kinetochore fibers of the mitotic spindle by acting as inter-microtubule bridge. The TACC3/ch-TOG/clathrin complex is required for the maintenance of kinetochore fiber tension (P

LOCALIZAÇÃO

CytoplasmCytoplasm, cytoskeleton, microtubule organizing center, centrosomeCytoplasm, cytoskeleton, spindleCytoplasm, cytoskeleton, spindle pole

VIAS BIOLÓGICAS (2)
Negative regulation of NOTCH4 signalingNOTCH3 Activation and Transmission of Signal to the Nucleus
EXPRESSÃO TECIDUAL(Ubíquo)
Testículo
122.0 TPM
Linfócitos
98.8 TPM
Sangue
80.8 TPM
Baço
44.0 TPM
Fibroblastos
29.4 TPM
OUTRAS DOENÇAS (2)
giant cell glioblastomagliosarcoma
HGNC:11524UniProt:Q9Y6A5
LZTR1Leucine-zipper-like transcriptional regulator 1Disease-causing somatic mutation(s) inTolerante
FUNÇÃO

Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex that mediates ubiquitination of Ras (K-Ras/KRAS, N-Ras/NRAS and H-Ras/HRAS) (PubMed:30442762, PubMed:30442766, PubMed:30481304). Is a negative regulator of RAS-MAPK signaling that acts by controlling Ras levels and decreasing Ras association with membranes (PubMed:30442762, PubMed:30442766, PubMed:30481304)

LOCALIZAÇÃO

Endomembrane systemRecycling endosomeGolgi apparatus

MECANISMO DE DOENÇA

Glioma

Gliomas are benign or malignant central nervous system neoplasms derived from glial cells. They comprise astrocytomas and glioblastoma multiforme that are derived from astrocytes, oligodendrogliomas derived from oligodendrocytes and ependymomas derived from ependymocytes.

EXPRESSÃO TECIDUAL(Ubíquo)
Pituitária
76.5 TPM
Nervo tibial
68.8 TPM
Útero
67.8 TPM
Cervix Ectocervix
65.1 TPM
Cervix Endocervix
61.7 TPM
OUTRAS DOENÇAS (9)
Noonan syndrome 10RASopathyNoonan syndrome 2cafe au lait spots, multiple
HGNC:6742UniProt:Q8N653
NFKBIANF-kappa-B inhibitor alphaBiomarker tested inAltamente restrito
FUNÇÃO

Inhibits the activity of dimeric NF-kappa-B/REL complexes by trapping REL (RELA/p65 and NFKB1/p50) dimers in the cytoplasm by masking their nuclear localization signals (PubMed:1493333, PubMed:36651806, PubMed:7479976). On cellular stimulation by immune and pro-inflammatory responses, becomes phosphorylated promoting ubiquitination and degradation, enabling the dimeric RELA to translocate to the nucleus and activate transcription (PubMed:7479976, PubMed:7628694, PubMed:7796813, PubMed:7878466)

LOCALIZAÇÃO

CytoplasmNucleus

VIAS BIOLÓGICAS (10)
RIP-mediated NFkB activation via ZBP1TRAF6 mediated NF-kB activationTAK1-dependent IKK and NF-kappa-B activation NF-kB is activated and signals survivalCLEC7A (Dectin-1) signaling
MECANISMO DE DOENÇA

Ectodermal dysplasia and immunodeficiency 2

A form of ectoderma dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. This form of ectodermal dysplasia is associated with decreased production of pro-inflammatory cytokines and certain interferons, rendering patients susceptible to infection. EDAID2 inheritance is autosomal dominant.

EXPRESSÃO TECIDUAL(Ubíquo)
Pulmão
497.5 TPM
Nervo tibial
450.8 TPM
Baço
352.8 TPM
Ovário
332.9 TPM
Skin Not Sun Exposed Suprapubic
320.1 TPM
OUTRAS DOENÇAS (5)
ectodermal dysplasia and immunodeficiency 2ectodermal dysplasia and immune deficiencygiant cell glioblastomanasopharyngeal carcinoma
HGNC:7797UniProt:P25963
EGFREpidermal growth factor receptorPart of a fusion gene inRestrito
FUNÇÃO

Receptor tyrosine kinase binding ligands of the EGF family and activating several signaling cascades to convert extracellular cues into appropriate cellular responses (PubMed:10805725, PubMed:27153536, PubMed:2790960, PubMed:35538033). Known ligands include EGF, TGFA/TGF-alpha, AREG, epigen/EPGN, BTC/betacellulin, epiregulin/EREG and HBEGF/heparin-binding EGF (PubMed:12297049, PubMed:15611079, PubMed:17909029, PubMed:20837704, PubMed:27153536, PubMed:2790960, PubMed:7679104, PubMed:8144591, PubM

LOCALIZAÇÃO

Cell membraneEndoplasmic reticulum membraneGolgi apparatus membraneNucleus membraneEndosomeEndosome membraneNucleusSecreted

VIAS BIOLÓGICAS (10)
Signaling by EGFRInhibition of Signaling by Overexpressed EGFRSignaling by ERBB2Signaling by ERBB4Signaling by ERBB2 TMD/JMD mutants
MECANISMO DE DOENÇA

Lung cancer

A common malignancy affecting tissues of the lung. The most common form of lung cancer is non-small cell lung cancer (NSCLC) that can be divided into 3 major histologic subtypes: squamous cell carcinoma, adenocarcinoma, and large cell lung cancer. NSCLC is often diagnosed at an advanced stage and has a poor prognosis.

EXPRESSÃO TECIDUAL(Ubíquo)
Skin Sun Exposed Lower leg
78.3 TPM
Skin Not Sun Exposed Suprapubic
75.9 TPM
Fibroblastos
60.6 TPM
Nervo tibial
43.1 TPM
Vagina
40.6 TPM
OUTRAS DOENÇAS (4)
inflammatory skin and bowel disease, neonatal, 2lung cancergliosarcomagiant cell glioblastoma
HGNC:3236UniProt:P00533
FGFR1Fibroblast growth factor receptor 1Part of a fusion gene inAltamente restrito
FUNÇÃO

Tyrosine-protein kinase that acts as a cell-surface receptor for fibroblast growth factors and plays an essential role in the regulation of embryonic development, cell proliferation, differentiation and migration. Required for normal mesoderm patterning and correct axial organization during embryonic development, normal skeletogenesis and normal development of the gonadotropin-releasing hormone (GnRH) neuronal system. Phosphorylates PLCG1, FRS2, GAB1 and SHB. Ligand binding leads to the activati

LOCALIZAÇÃO

Cell membraneNucleusCytoplasm, cytosolCytoplasmic vesicle

VIAS BIOLÓGICAS (2)
Epithelial-Mesenchymal Transition (EMT) during gastrulationFormation of paraxial mesoderm
MECANISMO DE DOENÇA

Pfeiffer syndrome

A syndrome characterized by the association of craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly of the fingers and toes. Three subtypes are known: mild autosomal dominant form (type 1); cloverleaf skull, elbow ankylosis, early death, sporadic (type 2); craniosynostosis, early demise, sporadic (type 3).

EXPRESSÃO TECIDUAL(Ubíquo)
Aorta
144.8 TPM
Ovário
142.9 TPM
Artéria tibial
134.1 TPM
Fallopian Tube
122.3 TPM
Cérebro - Hemisfério cerebelar
122.0 TPM
OUTRAS DOENÇAS (20)
Hartsfield-Bixler-Demyer syndromeencephalocraniocutaneous lipomatosisosteoglophonic dysplasiaPfeiffer syndrome
HGNC:3688UniProt:P11362
TACC1Transforming acidic coiled-coil-containing protein 1Part of a fusion gene inTolerante
FUNÇÃO

Involved in transcription regulation induced by nuclear receptors, including in T3 thyroid hormone and all-trans retinoic acid pathways (PubMed:20078863). Might promote the nuclear localization of the receptors (PubMed:20078863). Likely involved in the processes that promote cell division prior to the formation of differentiated tissues

LOCALIZAÇÃO

CytoplasmNucleusCytoplasm, cytoskeleton, microtubule organizing center, centrosomeMidbodyMembrane

VIAS BIOLÓGICAS (1)
Signaling by plasma membrane FGFR1 fusions
EXPRESSÃO TECIDUAL(Ubíquo)
Nervo tibial
142.9 TPM
Esôfago - Muscular
140.6 TPM
Útero
124.4 TPM
Esôfago - Junção
122.5 TPM
Tecido adiposo
119.2 TPM
OUTRAS DOENÇAS (2)
giant cell glioblastomagliosarcoma
HGNC:11522UniProt:O75410
TP53Cellular tumor antigen p53Biomarker tested inAltamente restrito
FUNÇÃO

Multifunctional transcription factor that induces cell cycle arrest, DNA repair or apoptosis upon binding to its target DNA sequence (PubMed:11025664, PubMed:12524540, PubMed:12810724, PubMed:15186775, PubMed:15340061, PubMed:17317671, PubMed:17349958, PubMed:19556538, PubMed:20673990, PubMed:20959462, PubMed:22726440, PubMed:24051492, PubMed:24652652, PubMed:35618207, PubMed:36634798, PubMed:38653238, PubMed:9840937). Acts as a tumor suppressor in many tumor types; induces growth arrest or apop

LOCALIZAÇÃO

CytoplasmNucleusNucleus, PML bodyEndoplasmic reticulumMitochondrion matrixCytoplasm, cytoskeleton, microtubule organizing center, centrosome

VIAS BIOLÓGICAS (10)
TP53 Regulates Metabolic GenesRegulation of TP53 ExpressionRegulation of TP53 DegradationOncogene Induced SenescenceOxidative Stress Induced Senescence
EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
73.0 TPM
Skin Sun Exposed Lower leg
37.0 TPM
Skin Not Sun Exposed Suprapubic
35.2 TPM
Fibroblastos
32.9 TPM
Ovário
32.4 TPM
OUTRAS DOENÇAS (29)
Li-Fraumeni syndromenasopharyngeal carcinoma, susceptibility to, 1hepatocellular carcinomafamilial pancreatic carcinoma
HGNC:11998UniProt:P04637
SEPTIN14Septin-14Part of a fusion gene inTolerante
FUNÇÃO

Filament-forming cytoskeletal GTPase (Probable). Involved in the migration of cortical neurons and the formation of neuron leading processes during embryonic development (By similarity). Plays a role in sperm head formation during spermiogenesis, potentially via facilitating localization of ACTN4 to cell filaments (PubMed:33228246)

LOCALIZAÇÃO

CytoplasmCytoplasm, cytoskeletonCell projection, axonCell projection, dendritePerikaryonCytoplasm, perinuclear regionCytoplasmic vesicle, secretory vesicle, acrosome

OUTRAS DOENÇAS (2)
gliosarcomagiant cell glioblastoma
HGNC:33280UniProt:Q6ZU15

Variantes genéticas (ClinVar)

1,796 variantes patogênicas registradas no ClinVar.

🧬 SEPTIN14: NM_207366.3(SEPTIN14):c.372-3del ()
🧬 SEPTIN14: Single allele ()
🧬 SEPTIN14: GRCh37/hg19 7p22.3-q36.3(chr7:10365-159119707)x3 ()
🧬 SEPTIN14: Single allele ()
🧬 SEPTIN14: GRCh37/hg19 7p22.3-q36.3(chr7:43361-159119707)x3 ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 7 variantes classificadas pelo ClinVar.

7
Patogênica (100.0%)
VARIANTES MAIS SIGNIFICATIVAS
PIK3R1: NM_181523.3(PIK3R1):c.1357_1362del (p.Asn453_Thr454del) [Likely pathogenic]
PTEN: NM_000314.8(PTEN):c.275A>T (p.Asp92Val) [Pathogenic]
PIK3CA: NM_006218.4(PIK3CA):c.325GAA[1] (p.Glu110del) [Likely pathogenic]
PTEN: NM_000314.8(PTEN):c.783_784del (p.Asn262fs) [Pathogenic]
PIK3CA: NM_006218.4(PIK3CA):c.263G>A (p.Arg88Gln) [Pathogenic]

Vias biológicas (Reactome)

121 vias biológicas associadas aos genes desta condição.

PPARA activates gene expression Transcriptional regulation of white adipocyte differentiation Nuclear Receptor transcription pathway SUMOylation of intracellular receptors Regulation of PTEN gene transcription MECP2 regulates transcription factors MLL4 and MLL3 complexes regulate expression of PPARG target genes in adipogenesis and hepatic steatosis Transcriptional regulation of brown and beige adipocyte differentiation by EBF2 alectinib sensitve ALK mutants ceritinib-resistant ALK mutants ceritinib-resistant ALK mutants don't bind ceritinib ALK mutants bind type I TKIs Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate NADPH regeneration Neutrophil degranulation Peroxisomal protein import NFE2L2 regulating TCA cycle genes PI3K Cascade PIP3 activates AKT signaling Signaling by activated point mutants of FGFR3 FGFR3b ligand binding and activation FGFR3c ligand binding and activation t(4;14) translocations of FGFR3 Constitutive Signaling by Aberrant PI3K in Cancer Phospholipase C-mediated cascade; FGFR3 SHC-mediated cascade:FGFR3 FRS-mediated FGFR3 signaling PI-3K cascade:FGFR3 Negative regulation of FGFR3 signaling Signaling by FGFR3 in disease RAF/MAP kinase cascade PI5P, PP2A and IER3 Regulate PI3K/AKT Signaling Signaling by FGFR3 fusions in cancer MGMT-mediated DNA damage reversal NOTCH3 Activation and Transmission of Signal to the Nucleus Negative regulation of NOTCH4 signaling Activation of NF-kappaB in B cells RIP-mediated NFkB activation via ZBP1 Downstream TCR signaling NF-kB is activated and signals survival FCERI mediated NF-kB activation TAK1-dependent IKK and NF-kappa-B activation SUMOylation of immune response proteins IkBA variant leads to EDA-ID CLEC7A (Dectin-1) signaling Ub-specific processing proteases Interleukin-1 signaling TRAF6 mediated NF-kB activation SARS-CoV-1 activates/modulates innate immune responses Turbulent (oscillatory, disturbed) flow shear stress activates signaling by PIEZO1 and integrins in endothelial cells Dengue virus modulates apoptosis Signaling by ERBB2 Constitutive Signaling by Ligand-Responsive EGFR Cancer Variants Signaling by ERBB4 SHC1 events in ERBB2 signaling PLCG1 events in ERBB2 signaling Signaling by EGFR GRB2 events in EGFR signaling GAB1 signalosome SHC1 events in EGFR signaling EGFR downregulation GRB2 events in ERBB2 signaling PI3K events in ERBB2 signaling EGFR interacts with phospholipase C-gamma EGFR Transactivation by Gastrin Signal transduction by L1 Constitutive Signaling by EGFRvIII Inhibition of Signaling by Overexpressed EGFR ERBB2 Regulates Cell Motility ERBB2 Activates PTK6 Signaling Cargo recognition for clathrin-mediated endocytosis Clathrin-mediated endocytosis PTK6 promotes HIF1A stabilization Downregulation of ERBB2 signaling TFAP2 (AP-2) family regulates transcription of growth factors and their receptors Extra-nuclear estrogen signaling Signaling by FGFR1 amplification mutants Signaling by activated point mutants of FGFR1 FGFR1b ligand binding and activation FGFR1c ligand binding and activation FGFR1c and Klotho ligand binding and activation NCAM signaling for neurite out-growth Phospholipase C-mediated cascade: FGFR1 Downstream signaling of activated FGFR1 SHC-mediated cascade:FGFR1 PI-3K cascade:FGFR1 FRS-mediated FGFR1 signaling Negative regulation of FGFR1 signaling Signaling by FGFR1 in disease Signaling by plasma membrane FGFR1 fusions Epithelial-Mesenchymal Transition (EMT) during gastrulation Formation of paraxial mesoderm Activation of NOXA and translocation to mitochondria Activation of PUMA and translocation to mitochondria Pre-NOTCH Transcription and Translation Oxidative Stress Induced Senescence Formation of Senescence-Associated Heterochromatin Foci (SAHF) Oncogene Induced Senescence DNA Damage/Telomere Stress Induced Senescence SUMOylation of transcription factors Autodegradation of the E3 ubiquitin ligase COP1 Association of TriC/CCT with target proteins during biosynthesis Pyroptosis TP53 Regulates Metabolic Genes Ovarian tumor domain proteases Recruitment and ATM-mediated phosphorylation of repair and signaling proteins at DNA double strand breaks Interleukin-4 and Interleukin-13 signaling TP53 Regulates Transcription of DNA Repair Genes TP53 Regulates Transcription of Genes Involved in Cytochrome C Release TP53 regulates transcription of several additional cell death genes whose specific roles in p53-dependent apoptosis remain uncertain TP53 Regulates Transcription of Caspase Activators and Caspases TP53 Regulates Transcription of Death Receptors and Ligands TP53 Regulates Transcription of Genes Involved in G2 Cell Cycle Arrest TP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertain TP53 Regulates Transcription of Genes Involved in G1 Cell Cycle Arrest Regulation of TP53 Expression Regulation of TP53 Activity through Phosphorylation Regulation of TP53 Degradation Regulation of TP53 Activity through Acetylation Regulation of TP53 Activity through Association with Co-factors Regulation of TP53 Activity through Methylation

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Pesquisa e ensaios clínicos

341 ensaios clínicos encontrados, 1 ativos.

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Publicações mais relevantes

Timeline de publicações
57 papers (10 anos)
#1

Clinicopathologic, molecular and tumor immune microenvironment features of mismatch repair-deficient glioblastomas in Lynch syndrome: a multicenter study of 29 cases with therapeutic implications.

Acta neuropathologica communications2026 Mar 05

Glioblastoma (GBM) is a relatively rare manifestation of Lynch syndrome (LS), and its defining characteristics are not yet fully defined. This study employs an integrated analysis of the clinicopathological, molecular, and tumor immune microenvironment features of LS-GBMs to elucidate its distinct biology and inform diagnostic and therapeutic strategies. We collected GBM samples from 29 LS patients across multiple medical centers. Germline MMR gene testing confirmed the following mutations among the 29 LS-GBM cases: MSH2 (16 cases, 55.2%), MLH1 (6 cases, 20.7%), MSH6 (4 cases, 13.8%), and PMS2 (1 case, 3.4%). Two cases (6.9%) exhibited no detectable pathogenic germline variants. Patients presented at a mean age of  45.7 years (range 10–69), significantly younger than those with IDH-mutant astrocytoma (WHO grade 4) (P < 0.05) or conventional IDH-wildtype GBMs (IDH-wt cGBMs; P < 0.001). Notably, IDH-wt LS-GBMs demonstrated superior overall survival compared to IDH-wt cGBMs (P < 0.05). Histopathologically, 96.6% (28/29) of cases displayed multinucleated giant cells, with 89.7% (26/29) exhibiting a wreath-like nuclear pattern. Additionally, 58.6% (17/28) demonstrated areas with oligodendroglioma-like characteristics. Molecular profiling revealed high-frequency mutations in TP53 (82.8%, 24/29) and SETD2 (53.6%, 15/28), suggesting concomitant dysregulation of cell cycle control and chromatin remodeling pathways. Furthermore, frequent pathogenic mutations were observed in NF1 (64.3%, 18/28), along with activating mutations in PDGFRA (39.3%, 11/28) and EGFR (32.1%, 9/29), suggesting tumor proliferation and invasion driven by receptor tyrosine kinase (RTK) signaling, such as the Ras/MAPK pathway. Moreover, the MMR-deficient state results in a high tumor mutational burden (100%, 16/16, ≥ 10 muts/Mb) and an inflamed tumor microenvironment with abundant CD8+ T-cell and CD163+ macrophages infiltration. Our findings establish LS-GBMs as a distinct molecular subtype of GBM, driven by convergent defects in DNA repair, cell cycle regulation, and RTK signaling, and highlighted by an immunogenic microenvironment. An integrated diagnostic approach is crucial for its identification, and tailored therapeutic strategies, including immune checkpoint inhibitors and targeted agents, should be explored. The online version contains supplementary material available at 10.1186/s40478-026-02246-6.

#2

Pediatric Oncology Patients With Germline Pathogenic Variants in Adult-Onset Cancer Predisposition Genes.

JCO precision oncology2025 Dec

Cancer predisposition syndromes caused by germline pathogenic variants (GPV) in adult-onset cancer predisposition genes (aoCPG) are those for which there is low risk of cancer in children, with genetic testing and screening for these conditions typically deferred until adulthood. GPV in aoCPG have been identified in pediatric oncology patients, but in these cases the potential contribution of the aoCPG to cancer development is often unknown. We investigated the role GPV in aoCPG may play in childhood cancer development. Results of paired tumor-germline sequencing from pediatric oncology patients enrolled from May 2012 to October 2023 were analyzed for frequency of GPV in aoCPG. Germline testing included analysis of up to 182 cancer predisposition genes. Tumor loss-of-heterozygosity, presence of second somatic pathogenic variant, immunohistochemical stain for protein expression, and/or tumor mutation burden were used to determine possible causation. Of the 954 participants, 42 (4.4%) had GPV in aoCPG. Six (14.3%) of these 42 participants had tumor findings indicating their GPV in an aoCPG likely contributed to cancer development: three patients with Lynch syndrome (two anaplastic astrocytomas, one giant cell glioblastoma) and one each with GPV in ATM (craniopharyngioma and diffuse high-grade glioma), BRIP1 (atypical teratoid rhabdoid tumor), and CHEK2 (mixed germ cell tumor of pineal gland). These findings contribute to the literature suggesting that, rarely, GPV in aoCPG may contribute to cancer diagnoses in children, raising the question of how tumors in these cases may present differently in children than adults. Increased knowledge about potential childhood cancer risks related to what have historically been considered aoCPG could modify predictive genetic testing recommendations for children and enhance existing cancer screening protocols.

#3

A Novel Germline MUTYH Mutation (p.W156∗) in High-Grade Astrocytoma, IDH Mutant.

Human mutation2025

Germline mutations in the DNA repair gene E. coli MutY homolog (MUTYH) are established predisposing factors for colorectal polyposis, colorectal carcinoma, and various extracolonic malignancies. Nevertheless, the association between MUTYH mutations and central nervous system (CNS) tumorigenesis remains poorly characterized. In this study, we reported the first identification of a novel c.467G > A (p.W156∗) MUTYH variant in two patients with high-grade astrocytoma, IDH mutant, which was classified as pathogenic. Histopathological evaluation revealed tumor morphologies consistent with either diffuse glioma or giant cell glioblastoma. Comparative analysis with mismatch repair (MMR)-deficient tumors demonstrated that patients carrying MUTYH mutations exhibited microsatellite stability, relatively low tumor mutation burden (TMB), and an immunosuppressive microenvironment, indicating difficulties in benefiting from immunotherapy. Fortunately, gain of Chromosome 7, in association with amplification of the MET gene, was detected, underscoring the possible application of targeted drugs. Integrating previous studies, we summarized germline MUTYH mutations in 11 cases of high-grade neuroepithelial tumors (eight gliomas and three medulloblastomas). This cohort demonstrated a predilection for pediatric and young adult populations without significant gender predominance. Our findings suggested a potential association between germline MUTYH mutations and CNS tumor susceptibility.

#4

EPR25-124: Epidemiology of Giant Cell Glioblastoma: A Surveillance, Epidemiology, and End Results (SEER) Analysis.

Journal of the National Comprehensive Cancer Network : JNCCN2025 Mar 28
#5

DNA methylation-based analysis reveals accelerated epigenetic aging in giant cell-enriched adult-type glioblastoma.

Clinical epigenetics2024 Dec 11

Giant cell (gc)-enriched glioblastoma (gcGB) represents a distinct histological variant of isocitrate dehydrogenase wild-type adult-type glioblastoma with notable enlarged mono- or multinuclear tumor cells. While some studies suggest a survival advantage for gcGB patients, the underlying causes remain elusive. GcGBs are associated with TP53 mutations, and gcs were shown to accumulate DNA double-strand breaks and show deficient mitosis, potentially triggering cellular senescence programs. Epigenetic clocks have emerged as valuable tools for assessing tumor-induced age acceleration (DNAMethAgeAcc), which has lately proved itself as prognostic biomarker in glioblastoma. Our study aimed to comprehensively analyze the methylome and key metabolic proteins of gcGBs, hypothesizing that they undergo cellular aging programs compared to non-gcGBs. A total of 310 epigenetically classified GBs, including 26 gcGBs, and nine adults with malignant gliomas allocating to pediatric high-grade glioma molecular subclasses (summarized as "pediatric GB") were included. DNAMethAgeAcc was computed by subtraction of chronological patient ages from DNA methylome-derived age estimations and its increase was associated with better survival within gcGB and non-gcGB. GcGBs were significantly more often allocated to the subgroup with increased DNAMethAgeAcc and demonstrated the highest DNAMethAgeAcc. Hypothetical senescence/aging-induced changes of the tumor microenvironment were addressed by tumor deconvolution, which was able to identify a cluster enriched for tumors with increased DNAMethAgeAcc. Key metabolic protein expression did not differ between gcGB and non-gcGB and tumor with versus without increased DNAMethAgeAcc but for elevated levels of one single mitochondrial marker, anti-mitochondrial protein MT-C02, in gcGBs. With its sped-up epigenetic aging, gcGB presented as the epigenetic oldest GB variant in our cohort. Whereas the correlation between accelerated tumor-intrinsic epigenetic aging and cellular senescence in gcGB stays elusive, fostering epigenetic aging programs in GB might be of interest for future exploration of alternative treatment options in GB patients.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC88 artigos no totalmostrando 57

2026

Clinicopathologic, molecular and tumor immune microenvironment features of mismatch repair-deficient glioblastomas in Lynch syndrome: a multicenter study of 29 cases with therapeutic implications.

Acta neuropathologica communications
2025

Pediatric Oncology Patients With Germline Pathogenic Variants in Adult-Onset Cancer Predisposition Genes.

JCO precision oncology
2025

A Novel Germline MUTYH Mutation (p.W156∗) in High-Grade Astrocytoma, IDH Mutant.

Human mutation
2025

EPR25-124: Epidemiology of Giant Cell Glioblastoma: A Surveillance, Epidemiology, and End Results (SEER) Analysis.

Journal of the National Comprehensive Cancer Network : JNCCN
2024

DNA methylation-based analysis reveals accelerated epigenetic aging in giant cell-enriched adult-type glioblastoma.

Clinical epigenetics
2024

Long-term survival in a patient with Li-Fraumeni syndrome-associated giant cell glioblastoma treated with nivolumab: illustrative case.

Journal of neurosurgery. Case lessons
2024

Case report: Germline CHEK2 mutation is associated with a giant cell glioblastoma.

Frontiers in oncology
2024

A case report with discussion on the treatment of giant cell glioblastoma.

Asian journal of surgery
2024

Giant cell glioblastoma with lipogenic differentiation in a patient with neurofibromatosis type 1: A case report.

Neuropathology : official journal of the Japanese Society of Neuropathology
2023

"De novo replication repair deficient glioblastoma, IDH-wildtype" is a distinct glioblastoma subtype in adults that may benefit from immune checkpoint blockade.

Acta neuropathologica
2023

Ultra High-plex Spatial Proteogenomic Investigation of Giant Cell Glioblastoma Multiforme Immune Infiltrates Reveals Distinct Protein and RNA Expression Profiles.

Cancer research communications
2023

Clinicopathological and Immunohistochemistry Study of a Long Survivor of Giant Cell Glioblastoma in a Patient With Neurofibromatosis 1: Case Report.

Cureus
2023

Glioblastoma with Unusual Features: Presentation with Intracerebral Hematoma, Diagnosis with CT Perfusion and Subsequent Cerebral Venous Sinus Thrombosis with Resultant New Hemorrhage.

Journal of clinical practice and research
2023

Not All Monstrous Cells Indicate Glioblastoma: A Neuropathological Case Report of Pleomorphic Xanthoastrocytoma Misdiagnoses As Giant Cell Glioblastoma.

Cureus
2022

Morphogenetic and Imaging Characteristics in Giant Cell Glioblastoma.

Current oncology (Toronto, Ont.)
2022

Gliosarcoma with unusual glial components: Two case reports.

Neuropathology : official journal of the Japanese Society of Neuropathology
2022

Glioblastoma: Changing concepts in the WHO CNS5 classification.

Indian journal of pathology &amp; microbiology
2022

Case report: Awake craniotomy during pregnancy for resection of glioblastoma.

Clinical neurology and neurosurgery
2021

Pediatric Giant Cell Glioblastoma Presenting with Intracranial Dissemination at Diagnosis: A Case Report.

NMC case report journal
2021

IDH-wild type glioblastomas featuring at least 30% giant cells are characterized by frequent RB1 and NF1 alterations and hypermutation.

Acta neuropathologica communications
2021

Epidemiologic Features, Survival, and Prognostic Factors Among Patients With Different Histologic Variants of Glioblastoma: Analysis of a Nationwide Database.

Frontiers in neurology
2021

SMARCAL1 loss and alternative lengthening of telomeres (ALT) are enriched in giant cell glioblastoma.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2021

Primary Intracranial Leiomyosarcoma Secondary to Glioblastoma: Case Report and Literature Review.

Frontiers in oncology
2021

Cyclin D1 expression in ganglioglioma, pleomorphic xanthoastrocytoma and pilocytic astrocytoma.

Experimental and molecular pathology
2020

Near haploidization is a genomic hallmark which defines a molecular subgroup of giant cell glioblastoma.

Neuro-oncology advances
2023

Giant cell glioblastoma multiforme presents as acute pathological nontraumatic subdural haematoma.

British journal of neurosurgery
2020

Giant cell glioblastoma in 6-year-old kid: Report of an unusual case.

Clinical case reports
2021

Differentiating Giant Cell Glioblastoma from Classic Glioblastoma With Diffusion-Weighted Imaging.

World neurosurgery
2020

Characterization and Histological Examination of a Rare Giant Cell Glioblastoma.

Cureus
2020

TP53, ATRX alterations, and low tumor mutation load feature IDH-wildtype giant cell glioblastoma despite exceptional ultra-mutated tumors.

Neuro-oncology advances
2020

Update on Circumscribed Gliomas and Glioneuronal Tumors.

Surgical pathology clinics
2020

Giant cell glioblastoma is a distinctive subtype of glioma characterized by vulnerability to DNA damage.

Brain tumor pathology
2019

Subgaleal and brain abscesses due to Salmonella enteritidis following craniotomy for giant cell glioblastoma multiforme: A case report and literature review.

Surgical neurology international
2019

Association between giant cell glioblastoma and glioblastoma multiforme in the United States: A retrospective cohort study.

Brain and behavior
2019

An Unusual Presentation of Spinal Giant Cell Glioblastoma in a 21-Year-Old Female.

Journal of investigative medicine high impact case reports
2019

Prognostic Factors and Treatment Patterns in the Management of Giant Cell Glioblastoma.

World neurosurgery
2019

Whole-exome sequencing revealed mutational profiles of giant cell glioblastomas.

Brain pathology (Zurich, Switzerland)
2018

Giant cell glioblastoma with spinal and spinal leptomeningeal metastasis in a child: A rare presentation of a rare tumor.

Journal of craniovertebral junction &amp; spine
2019

Germline mutation p.N363K in POLE is associated with an increased risk of colorectal cancer and giant cell glioblastoma.

Familial cancer
2018

Glioblastoma in the setting of prior lower grade gliomas - insights from SEER database.

Oncotarget
2018

Imaging findings in the progression of a giant cell glioblastoma.

Radiology case reports
2018

Acute lymphoblastic leukemia following temozolomide treatment in a patient with glioblastoma: A case report and review of the literature.

Oncology letters
2017

Giant Cell Glioblastoma in a Child with Clinical and Family History of Neurofibromatosis.

Asian journal of neurosurgery
2017

IDH-mutant giant cell glioblastoma: A neglected tumor variant?

Clinical neuropathology
2017

Risk of subsequent cancer among pediatric, adult and elderly patients following a primary diagnosis of glioblastoma multiforme: a population-based study of the SEER database.

The International journal of neuroscience
2017

Intraventricular gliosarcoma with dual sarcomatous differentiation: A unique case.

Neuropathology : official journal of the Japanese Society of Neuropathology
2017

Incidence, survival, pathology, and genetics of adult Latino Americans with glioblastoma.

Journal of neuro-oncology
2017

Radiologic Dilemma in an Extra-Axial Petroclival Lesion: Low Grade versus High Grade.

World neurosurgery
2016

MGMT Promoter Methylation and BRAF V600E Mutations Are Helpful Markers to Discriminate Pleomorphic Xanthoastrocytoma from Giant Cell Glioblastoma.

PloS one
2016

Intraoperative Squash Cytologic Features of Subependymal Giant Cell Astrocytoma.

Journal of laboratory physicians
2015

Giant cell glioblastoma with calcification and long-term survival.

Indian journal of cancer
2016

Genetic Alterations in Gliosarcoma and Giant Cell Glioblastoma.

Brain pathology (Zurich, Switzerland)
2015

A spontaneously occurring malignant pituicytoma in a male sprague dawley rat.

Journal of toxicologic pathology
2015

Pediatric Giant Cell Glioblastoma Mimicking Hemorrhage Secondary to Ischemic Stroke.

Pediatric neurology
2015

Giant cells glioblastoma: case report and pathological analysis from this uncommon subtype of glioma.

Rare tumors
2015

Adult classical glioblastoma with a BRAF V600E mutation.

World journal of surgical oncology
2014

[Gliosarcoma of cerebral hemispheres: a clinicopathologic study of 10 cases].

Zhonghua bing li xue za zhi = Chinese journal of pathology
Ver todos os 88 no EuropePMC

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Clinicopathologic, molecular and tumor immune microenvironment features of mismatch repair-deficient glioblastomas in Lynch syndrome: a multicenter study of 29 cases with therapeutic implications.
    Acta neuropathologica communications· 2026· PMID 41782063mais citado
  2. Pediatric Oncology Patients With Germline Pathogenic Variants in Adult-Onset Cancer Predisposition Genes.
    JCO precision oncology· 2025· PMID 41325557mais citado
  3. A Novel Germline MUTYH Mutation (p.W156&#x2217;) in High-Grade Astrocytoma, IDH Mutant.
    Human mutation· 2025· PMID 40469416mais citado
  4. EPR25-124: Epidemiology of Giant Cell Glioblastoma: A Surveillance, Epidemiology, and End Results (SEER) Analysis.
    Journal of the National Comprehensive Cancer Network : JNCCN· 2025· PMID 40159141mais citado
  5. DNA methylation-based analysis reveals accelerated epigenetic aging in giant cell-enriched adult-type glioblastoma.
    Clinical epigenetics· 2024· PMID 39663543mais citado
  6. Long-term survival in a patient with Li-Fraumeni syndrome-associated giant cell glioblastoma treated with nivolumab: illustrative case.
    J Neurosurg Case Lessons· 2024· PMID 39586078recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:251579(Orphanet)
  2. MONDO:0016682(MONDO)
  3. GARD:20705(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q18555280(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Glioblastoma de células gigantes
Compêndio · Raras BR

Glioblastoma de células gigantes

ORPHA:251579 · MONDO:0016682
Prevalência
<1 / 1 000 000
Herança
Not applicable
CID-10
C71.9 · Neoplasia maligna do encéfalo, não especificado
CID-11
Ensaios
1 ativos
Início
Adult
Prevalência
0.02 (Europe)
MedGen
UMLS
C0334588
Repurposing
2 candidatos
carmustineDNA alkylating agent|DNA inhibitor
temozolomideDNA alkylating agent
EuropePMC
Wikidata
Papers 10a
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