MCID: GLM004
MIFTS: 44

Gliomatosis Cerebri

Categories: Cancer diseases, Mental diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Gliomatosis Cerebri

MalaCards integrated aliases for Gliomatosis Cerebri:

Name: Gliomatosis Cerebri 11 19 58 75 53 14 71
Neoplasms, Neuroepithelial 43
Astrocytosis Cerebri 11

Characteristics:


Prevelance:

<1/1000000 (Europe) 58

Age Of Onset:

Adult 58

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 11 DOID:6128
MeSH 43 D018302
NCIt 49 C4318
SNOMED-CT 68 26138003
ICD10 via Orphanet 32 C71.0
UMLS via Orphanet 72 C0334576
Orphanet 58 ORPHA251582
UMLS 71 C0334576

Summaries for Gliomatosis Cerebri

GARD: 19 Gliomatosis cerebri is a type of malignant (cancerous) brain tumor called a glioblastoma that develops from a specific type of brain cell called an astrocyte. It involves at least three sections (lobes) within the main part of the brain (cerebrum). It may also affect both sides of the cerebrum as well as the nerve cells within the brain (grey matter). There are two types of Gliomatosis cerebri. Type 1 (classic form) is characterized by scattered and widespread tumor cells and no apparent mass or tumor, while type 2 has similar characteristics in addition to a detectable mass or tumor. Signs and symptoms of Gliomatosis cerebri may include personality changes, memory disturbance, headaches, and seizures.

MalaCards based summary: Gliomatosis Cerebri, also known as neoplasms, neuroepithelial, is related to glioma susceptibility 1 and oligoastrocytoma, and has symptoms including headache, seizures and sensory manifestations. An important gene associated with Gliomatosis Cerebri is GFAP (Glial Fibrillary Acidic Protein), and among its related pathways/superpathways are Neural Stem Cells and Lineage-specific Markers and Pathways affected in adenoid cystic carcinoma. The drugs Neurotransmitter Agents and Psychotropic Drugs have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and eye, and related phenotypes are cellular and neoplasm

Orphanet: 58 A rare glial tumor characterized by extensive infiltration of the brain, often extending to infratentorial structures and even the spinal cord. The tumor corresponds to WHO grade III and is composed of elongated glial cells typically resembling astrocytes. Cases in which the predominant cell type is oligodendroglial have also been described. Some tumors develop a circumscribed neoplastic mass in addition to the diffuse lesion, usually showing features of high-grade glioma. Clinical symptoms include dementia, headache, seizures, signs of increased intracranial pressure, and a variety of neurological deficits. Prognosis is generally poor.

Disease Ontology: 11 A brain cancer that is characterized by a pattern of diffuse infiltration of the brain that affect various areas of the cerebral lobes and has material basis in abnormally proliferating cells, derives from glial cells.

Wikipedia: 75 Gliomatosis cerebri is a rare primary diffuse brain tumor that has a poor prognosis. It is defined by... more...

Related Diseases for Gliomatosis Cerebri

Diseases related to Gliomatosis Cerebri via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 305)
# Related Disease Score Top Affiliating Genes
1 glioma susceptibility 1 31.2 TP53 IDH2 IDH1 H3-3A
2 oligoastrocytoma 30.6 MGMT IDH2 GFAP
3 glioma 30.4 TP53 PTEN MGMT IDH2 IDH1
4 central nervous system cancer 30.4 TP53 MGMT KDM4C IDH1
5 hydrocephalus 30.1 TP53 PTEN NES GFAP
6 oligodendroglioma 30.1 TP53 PTEN OLIG2 MGMT IDH2 IDH1
7 high-grade astrocytoma 30.0 TP53 PTEN ATRX
8 2-hydroxyglutaric aciduria 30.0 KDM4C IDH2 IDH1
9 l-2-hydroxyglutaric aciduria 30.0 KDM4C IDH2 IDH1
10 neurofibromatosis, type i 29.9 TP53 PTEN KIAA1549 IDH1
11 high grade glioma 29.7 TP53 PTEN NES MGMT KDM4C GFAP
12 malignant astrocytoma 29.5 TP53 PTEN MGMT KDM4C IDH1 ATRX
13 obstructive hydrocephalus 29.4 OLIG2 KIAA1549 H3-3B H3-3A GFAP
14 enchondromatosis, multiple, ollier type 29.3 TP53 KDM4C IDH2 IDH1 ATRX
15 fibrillary astrocytoma 29.2 TP53 LUC7L2 KIAA1549 IDH2 IDH1 H3-3B
16 gemistocytic astrocytoma 29.1 TP53 PTEN MGMT IDH2 IDH1 GFAP
17 glioblastoma 29.0 TP53 PTEN OLIG2 NES MGMT IDH2
18 medulloblastoma 28.9 TP53 PTEN OLIG2 NES MGMT IDH1
19 brain cancer 28.8 TP53 PTEN OLIG2 NES MGMT KDM4C
20 meningioma, familial 28.6 TP53 PTEN OLIG2 MGMT KIAA1549 IDH1
21 diffuse astrocytoma 28.1 TP53 PTEN OLIG2 MGMT KIAA1549 KDM4C
22 anaplastic astrocytoma 27.7 TP53 PTEN OLIG2 NES MGMT KIAA1549
23 nervous system disease 27.5 TP53 PTEN OLIG2 NES MGMT LUC7L2
24 anaplastic oligodendroglioma 27.2 TP53 PTEN OLIG2 NES MGMT KIAA1549
25 pilocytic astrocytoma 27.2 TP53 PTEN OLIG2 NES MGMT KIAA1549
26 spinal cord disease 27.0 TP53 PTEN OLIG2 NES MGMT KIAA1549
27 low grade glioma 26.7 TP53 PTEN OLIG2 NES MGMT KIAA1549
28 glial tumor 10.5
29 intracranial hypertension 10.5
30 rare tumor 10.5
31 encephalitis 10.4
32 parkinsonism 10.3
33 low-grade astrocytoma 10.3
34 urethra transitional cell carcinoma 10.3 TP53 GFAP
35 intracranial hypertension, idiopathic 10.3
36 overgrowth syndrome 10.3
37 enchondroma 10.3 IDH2 IDH1
38 lung oat cell carcinoma 10.3 TP53 PTEN
39 prolactin producing pituitary tumor 10.3 TP53 MGMT
40 spinal cord lipoma 10.3 MGMT GFAP
41 synchronous bilateral breast carcinoma 10.3 TP53 PTEN
42 intracranial chondrosarcoma 10.2 IDH2 IDH1
43 cerebral lymphoma 10.2 MGMT IDH1
44 anaplastic oligodendroglioma, idh-mutant and 1p/19q-codeleted 10.2 IDH2 IDH1
45 periosteal chondrosarcoma 10.2 IDH2 IDH1
46 juxtacortical chondroma 10.2 IDH2 IDH1
47 glioblastoma classical subtype 10.2 MGMT IDH1
48 endometrioid ovary carcinoma 10.2 TP53 PTEN
49 sinonasal undifferentiated carcinoma 10.2 IDH2 IDH1
50 sclerosing hemangioma 10.2 TP53 PTEN

Graphical network of the top 20 diseases related to Gliomatosis Cerebri:



Diseases related to Gliomatosis Cerebri

Symptoms & Phenotypes for Gliomatosis Cerebri

UMLS symptoms related to Gliomatosis Cerebri:


headache; seizures; sensory manifestations

MGI Mouse Phenotypes related to Gliomatosis Cerebri:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.65 ATRX GFAP H3-3A H3-3B IDH2 KDM4C
2 neoplasm MP:0002006 9.63 IDH2 KDM4C MGMT OLIG2 PTEN TP53
3 mortality/aging MP:0010768 9.36 ATRX GFAP H3-3A H3-3B IDH1 KDM4C

Drugs & Therapeutics for Gliomatosis Cerebri

Drugs for Gliomatosis Cerebri (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 71)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Neurotransmitter Agents Phase 3
2 Psychotropic Drugs Phase 3
3 Anticonvulsants Phase 3
4 Pharmaceutical Solutions Phase 3
5
Bevacizumab Approved, Investigational Phase 2 216974-75-3 135329020
6
Benzocaine Approved, Investigational Phase 2 1994-09-7, 94-09-7 2337
7
Tannic acid Approved Phase 2 1401-55-4 16129878 16129778
8
Gefitinib Approved, Investigational Phase 1, Phase 2 184475-35-2 123631
9
Pembrolizumab Approved Phase 2 1374853-91-4 254741536
10
Valproic acid Approved, Investigational Phase 2 99-66-1 3121
11
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
12
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
13 Antineoplastic Agents, Immunological Phase 2
14 Immunoglobulins Phase 2
15 Antibodies, Monoclonal Phase 2
16 Antibodies Phase 2
17 Angiogenesis Inhibitors Phase 1, Phase 2
18 Vaccines Phase 1, Phase 2
19 Adjuvants, Immunologic Phase 1, Phase 2
20 Interleukin-12 Phase 1, Phase 2
21 Anti-Bacterial Agents Phase 2
22 Antibiotics, Antitubercular Phase 2
23 Liposomal doxorubicin Phase 2
24
Lenograstim Approved, Investigational Phase 1 135968-09-1
25
Trametinib Approved Phase 1 871700-17-3 11707110
26
Gemcitabine Approved Phase 1 95058-81-4, 122111-03-9 60750
27
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 1 1177-87-3 3680
28
Dexamethasone Approved, Investigational, Vet_approved Phase 1 50-02-2 3003 5743
29
Palbociclib Approved, Investigational Phase 1 571190-30-2 5330286
30
Mebendazole Approved, Vet_approved Phase 1 31431-39-7 4030
31
Piperazine Approved, Vet_approved Phase 1 110-85-0 4837
32 Protein Kinase Inhibitors Phase 1
33 Antiviral Agents Phase 1
34 Antimetabolites Phase 1
35 Antineoplastic Agents, Hormonal Phase 1
36 Gastrointestinal Agents Phase 1
37 Hormones Phase 1
38 Hormone Antagonists Phase 1
39 Antiemetics Phase 1
40 glucocorticoids Phase 1
41 BB 1101 Phase 1
42 Anti-Inflammatory Agents Phase 1
43 Piperazine citrate Phase 1
44 DMP 777 Phase 1 157341-41-8
45
Dopamine Approved 62-31-7, 51-61-6 681
46
Melphalan Approved Early Phase 1 148-82-3 4053 460612
47
Fludarabine Approved Early Phase 1 75607-67-9, 21679-14-1 30751 657237
48
Alemtuzumab Approved, Investigational Early Phase 1 216503-57-0
49
Etoposide Approved Early Phase 1 33419-42-0 36462
50
Thiotepa Approved, Investigational Early Phase 1 52-24-4 5453

Interventional clinical trials:

(show all 17)
# Name Status NCT ID Phase Drugs
1 International Cooperative Phase III Trial of the HIT-HGG Study Group for the Treatment of High Grade Glioma, Diffuse Intrinsic Pontine Glioma, and Gliomatosis Cerebri in Children and Adolescents < 18 Years.(HIT-HGG-2013) Recruiting NCT03243461 Phase 3 Temozolomide + Valproic Acid
2 A Phase 2 Study of Valproic Acid and Radiation, Followed by Maintenance Valproic Acid and Bevacizumab in Children With Newly Diagnosed High-grade Gliomas or Brainstem Gliomas Completed NCT00879437 Phase 2 Valproic acid;Bevacizumab
3 Phase I/II Trial of Gefitinib and Radiation in Pediatric Patients Newly Diagnosed With Brain Stem Tumors or Incompletely Resected Supratentorial Malignant Gliomas With Phase II Limited to Brain Stem Tumors Completed NCT00042991 Phase 1, Phase 2 gefitinib
4 A Phase II, Multi-center, Open-label Study of a Conditionally Replicative Adenovirus (DNX-2401) With Pembrolizumab (KEYTRUDA®) for Recurrent Glioblastoma or Gliosarcoma (CAPTIVE/KEYNOTE-192) Completed NCT02798406 Phase 2
5 Clinical Study of an Dendritic and Glioma Cells Fusion Vaccine With IL-12 for Treatment-naïve GBM Patients. Recruiting NCT04388033 Phase 1, Phase 2 Interleukin-12;Temozolomide
6 An Open-label, Single-arm, Phase II Study to Evaluate Safety and Efficacy of Doxorubicin in Combination With Radiotherapy, Temozolomide and Valproic Acid in Patients With Glioblastoma Multiforme (GBM) and Diffuse Intrinsic Pontine Glioma (DIPG) Terminated NCT02758366 Phase 2 Doxorubicin
7 A First-in-human Phase I Single-agent Dose-escalation, Food Effect and Dose Expansion Study of Oral ONC206 in Recurrent and Rare Primary Central Nervous System Neoplasms Recruiting NCT04541082 Phase 1 ONC206
8 Phase 1 Trial of Engineered HSV G207 in Children With Recurrent or Refractory Cerebellar Brain Tumors Recruiting NCT03911388 Phase 1
9 Molecularly-Driven Doublet Therapy for All Children With Refractory or Recurrent CNS Malignant Neoplasms and Young Adults With Refractory or Recurrent SHH Medulloblastoma Active, not recruiting NCT03434262 Phase 1 Gemcitabine;ribociclib;sonidegib;trametinib
10 A Phase I Study of Intratumoral/Peritumoral Herpes Simplex Virus-1 Mutant HSV1716 in Patients With Refractory or Recurrent High Grade Gliomas (HGG) Terminated NCT02031965 Phase 1 dexamethasone
11 Phase I Study of CDK 4-6 Inhibitor PD-0332991 (Palbociclib; IBRANCE) in Children With Recurrent, Progressive or Refractory Central Nervous System Tumors Terminated NCT02255461 Phase 1 palbociclib isethionate
12 Untersuchungen Zur Verlaufskontrolle Bei Re-Bestrahlung Von Glioblastompatienten Mittels Kombinierter [18F]FET-PET-Kernspintomographie Unknown status NCT01579253
13 Molecular-guided Therapy for the Treatment of Patients With Relapsed and Refractory Childhood Cancers Active, not recruiting NCT02162732
14 The Expanded Access Use of Vorasidenib in Patients With IDH1 or IDH2 Mutated Glioma Available NCT05592743 Vorasidenib
15 18F-FDOPA PET/CT or PET/MRI in Patients With Gliomas Terminated NCT02175745 18F-fluoro-dihydroxyphenylalanine
16 A Multi-Institutional Pilot Study of Allogeneic Hematopoietic Stem Cell Transplantation for Patients With Malignant Neuro-Epithelial and Other Solid Tumors Terminated NCT02653196 Early Phase 1 Keratinocyte Growth Factor;Alemtuzumab;Thiotepa;Etoposide;Fludarabine;Melphalan;Tacrolimus;Cyclosporine A;Mycophenolate mofetil
17 Efficacy of 68Ga-DOTATOC Positron Emission Tomography (PET) CT in Children and Young Adults With Brain Tumors Withdrawn NCT02194452

Search NIH Clinical Center for Gliomatosis Cerebri

Cochrane evidence based reviews: neoplasms, neuroepithelial

Genetic Tests for Gliomatosis Cerebri

Anatomical Context for Gliomatosis Cerebri

Organs/tissues related to Gliomatosis Cerebri:

MalaCards : Brain, Spinal Cord, Eye, Temporal Lobe, Cerebellum, Pituitary, Breast

Publications for Gliomatosis Cerebri

Articles related to Gliomatosis Cerebri:

(show top 50) (show all 391)
# Title Authors PMID Year
1
Clinicopathological study of cellular proliferation and invasion in gliomatosis cerebri: important role of neural cell adhesion molecule L1 in tumour invasion. 53 62
15677537 2005
2
Analysis of TP53 and PTEN in gliomatosis cerebri. 53 62
12734658 2003
3
Assessment of proliferative potential in gliomatosis cerebri. 53 62
1856741 1991
4
Glioblastoma with Gliomatosis Cerebri Growth Pattern Presenting as Rapidly Progressive Dementia. 62
35419153 2022
5
T2/FLAIR Hyperintensity in Mesial Temporal Lobe: Challenging Differential Diagnosis. 62
34931987 2022
6
Multifocal oligodendroglioma with callosal and brainstem involvement. 62
36324958 2022
7
Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates. 62
35733513 2022
8
MRI findings in a young dog with gliomatosis cerebri. 62
34318514 2022
9
A rare case of gliomatosis cerebri lurking beneath the shadows of a stroke mimic. 62
34345333 2021
10
Nimotuzumab therapy in the treatment of pediatric central nervous system tumors: single-center experience. 62
34151393 2021
11
Leber's hereditary optic neuropathy with diffuse white matter changes mimicking gliomatosis cerebri: illustrative case. 62
35854899 2021
12
Fluctuation of Clinical Signs With Near-Syncopal Episodes in a Dog With Gliomatosis Cerebri: A Diagnostic Challenge. 62
33434679 2021
13
Canine Gliomatosis Cerebri: Morphologic and Immunohistochemical Characterization Is Supportive of Glial Histogenesis. 62
33357125 2021
14
Analysis of preventability of malignancy-related maternal death from the nationwide registration system of maternal deaths in Japan. 62
30999803 2021
15
Gliomatosis Cerebri Growth Pattern: Association of Differential First-Line Treatment with Overall Survival in WHO Grade II and III Gliomas. 62
33472203 2021
16
A pediatric case of anaplastic astrocytoma with a gliomatosis cerebri; the growth pattern and changes in serum VEGF-121 levels after bevacizumab treatment. 62
33222957 2020
17
Predictive factors for overall survival in surgical cases of gliomatosis cerebri from the National Cancer Database. 62
33222914 2020
18
A phase I trial of lenalidomide and radiotherapy in children with diffuse intrinsic pontine gliomas or high-grade gliomas. 62
33040274 2020
19
A case of pediatric gliomatosis cerebri harboring H3F3A K27 mutation. 62
32965767 2020
20
Gliomatosis cerebri mimicking diffuse demyelinating disease: Case Report. 62
32742528 2020
21
Gliomatosis cerebri (GC) or GC-like? A picture to be reconsidered in neuro-oncology based on large retrospective analysis of GC series. 62
32114667 2020
22
Multimodal neuroimaging of gliomatosis cerebri: a case series of four patients. 62
32913666 2020
23
Gliomatosis cerebri and Rasmussen's encephalitis: Two different entities causing refractory epilepsy. Comparison through two clinical cases. 62
32439411 2020
24
Outcomes of presumed malignant glioma treated without pathological confirmation: a retrospective, single-center analysis. 62
33282325 2020
25
Mortal consequences of a cooperative action between Takotsubo syndrome and increased intracranial pressure. 62
32142222 2020
26
Clinical relevance of molecular subgrouping of gliomatosis cerebri per 2016 WHO classification: a clinicopathological study of 89 cases. 62
31435963 2020
27
Adult H3K27M-mutant diffuse midline glioma with gliomatosis cerebri growth pattern: Case report and review of the literature. 62
32145563 2020
28
Imaging characteristics of adult H3 K27M-mutant gliomas. 62
31731269 2019
29
Radiological Characteristics and Natural History of Adult IDH-Wildtype Astrocytomas with TERT Promoter Mutations. 62
30407589 2019
30
Gliomatosis Cerebri: A Unique Presentation with Accompanying Clinical Nuance. 62
31528509 2019
31
Corticosteroid-induced immunodeficiency in a patient with gliomatosis cerebri: Are corticosteroids indicated in all brain tumors? 62
30964431 2019
32
Integrated diagnostics of diffuse astrocytic and oligodendroglial tumors. 62
31025086 2019
33
Gliomatosis Cerebri Among Children and Adolescents: An Individual-Patient Data Meta-analysis of 182 Patients. 62
30887873 2019
34
[Integrated diagnostics of diffuse astrocytic and oligodendroglial tumors. German version]. 62
30790013 2019
35
Wide Range of Clinical Outcomes in Patients with Gliomatosis Cerebri Growth Pattern: A Clinical, Radiographic, and Histopathologic Study. 62
30097523 2019
36
Heterogeneity of glioblastoma with gliomatosis cerebri growth pattern on diffusion and perfusion MRI. 62
30565029 2019
37
An autopsy case of widespread brain dissemination of glioblastoma unnoticed by magnetic resonance imaging after treatment with bevacizumab. 62
31528472 2019
38
An Adolescent Presenting With Seizures as a Symptom of Gliomatosis Cerebri. 62
28486262 2018
39
Prognostic Factors and Survival of Gliomatosis Cerebri: A Systematic Review and Meta-Analysis. 62
30172970 2018
40
Clinical, neuroimaging and histopathological features of gliomatosis cerebri: a systematic review based on synthesis of published individual patient data. 62
30117023 2018
41
Gliomatosis cerebri: a consensus summary report from the Second International Gliomatosis cerebri Group Meeting, June 22-23, 2017, Bethesda, USA. 62
29998396 2018
42
Incidence and survival of gliomatosis cerebri: a population-based cancer registration study. 62
29464663 2018
43
Magnetic resonance imaging features of canine gliomatosis cerebri. 62
29110365 2018
44
WHO 2016 Classification of gliomas. 62
28815663 2018
45
Gliomatosis Cerebri: A Rare Brain Tumor. 62
29459616 2018
46
Astroblastoma - reviewing literature and one case report. 62
30845307 2018
47
Lactoferrin- and RGD-comodified, temozolomide and vincristine-coloaded nanostructured lipid carriers for gliomatosis cerebri combination therapy. 62
29861635 2018
48
A Pediatric Case of Diffuse Glioma Diagnosed at Autopsy. 62
31240015 2017
49
Feline glioma: a retrospective study and review of the literature. 62
28156189 2017
50
2016 Updates to the WHO Brain Tumor Classification System: What the Radiologist Needs to Know. 62
29028423 2017

Variations for Gliomatosis Cerebri

Expression for Gliomatosis Cerebri

Search GEO for disease gene expression data for Gliomatosis Cerebri.

Pathways for Gliomatosis Cerebri

GO Terms for Gliomatosis Cerebri

Biological processes related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 NADP metabolic process GO:0006739 9.46 IDH2 IDH1
2 isocitrate metabolic process GO:0006102 9.26 IDH2 IDH1
3 glyoxylate cycle GO:0006097 8.92 IDH2 IDH1

Molecular functions related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 isocitrate dehydrogenase (NADP+) activity GO:0004450 8.92 IDH2 IDH1

Sources for Gliomatosis Cerebri

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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