MCID: GLM004
MIFTS: 49

Gliomatosis Cerebri

Categories: Rare diseases, Neuronal diseases, Cancer diseases

Aliases & Classifications for Gliomatosis Cerebri

MalaCards integrated aliases for Gliomatosis Cerebri:

Name: Gliomatosis Cerebri 12 50 56 52 14 69
Neoplasms, Neuroepithelial 42
Astrocytosis Cerebri 12

Characteristics:

Orphanet epidemiological data:

56
gliomatosis cerebri
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 56  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:6128
MeSH 42 D018302
NCIt 47 C4318
SNOMED-CT 64 26138003
Orphanet 56 ORPHA251582
UMLS via Orphanet 70 C0334576
ICD10 via Orphanet 34 C71.0
UMLS 69 C0334576

Summaries for Gliomatosis Cerebri

NIH Rare Diseases : 50 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. because this type of cancer can be found in many areas of the brain, it can be challenging to treat. treatment might include surgery to remove areas of the brain affected (when possible), radiation therapy, and chemotherapy. last updated: 7/21/2016

MalaCards based summary : Gliomatosis Cerebri, also known as neoplasms, neuroepithelial, is related to hypogonadotropic hypogonadism-frontoparietal alopecia syndrome and papillary glioneuronal tumor, 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 Phospholipase-C Pathway and Downstream signaling events of B Cell Receptor (BCR). The drugs Valproic Acid and chloroquine have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and pituitary, and related phenotypes are Increased cell death HMECs cells and digestive/alimentary

Wikipedia : 72 Gliomatosis cerebri (infiltrative diffuse astrocytosis) is a rare primary brain tumor. It is commonly... 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 186)
id Related Disease Score Top Affiliating Genes
1 hypogonadotropic hypogonadism-frontoparietal alopecia syndrome 10.7 GFAP IDH1
2 papillary glioneuronal tumor 10.6 GFAP IDH1
3 early myoclonic encephalopathy 10.6 IDH1 PTEN
4 ovarian mucinous cystadenofibroma 10.6 GFAP PTEN
5 epidermal appendage tumor 10.6 GFAP PTEN
6 mixed oligodendroglioma-astrocytoma 10.6 IDH1 TP53
7 pilocytic astrocytoma of cerebellum 10.6 IDH1 TP53
8 congenital disorder of glycosylation, type if 10.6 IDH1 TP53
9 macrocystic neurilemmoma 10.6 GFAP IDH1
10 pancoast tumor 10.6 GFAP TP53
11 brain stem astrocytic neoplasm 10.6 GFAP TP53
12 renal pelvis urothelial papilloma 10.6 GFAP TP53
13 tendon sheath lipoma 10.6 GFAP TP53
14 rh isoimmunization 10.5 GFAP TP53
15 third cranial nerve disease 10.5 GFAP TP53
16 pleural empyema 10.5 GFAP TP53
17 childhood choroid plexus cancer 10.5 GFAP TP53
18 brachial plexus neuritis 10.5 GFAP TP53
19 subependymal giant cell astrocytoma 10.5 GFAP TP53
20 breast myoepithelial neoplasm 10.5 PTEN TP53
21 pineoblastoma 10.5 GFAP TP53
22 chest wall lymphoma 10.5 PTEN TP53
23 reactive arthritis 10.5 GFAP MDM2
24 brenner tumor of the vagina 10.5 PTEN TP53
25 juxtacortical chondrosarcoma 10.5 IDH1 MDM2
26 brenner tumor of ovary 10.5 PTEN TP53
27 bannayan-riley-ruvalcaba syndrome 10.5 EGFR PTEN
28 cataract 24, anterior polar 10.5 GFAP TP53
29 plasmodium ovale malaria 10.4 GFAP TP53
30 atypical choroid plexus papilloma 10.4 GFAP TP53
31 pacinian tumor 10.4 GFAP TP53
32 split-hand/foot malformation 4 10.4 PTEN TP53
33 esophagus squamous cell carcinoma 10.4 PTEN TP53
34 large intestine lipoma 10.4 GFAP TP53
35 pediatric infratentorial ependymoma 10.4 EGFR TP53
36 rnase t2-deficient leukoencephalopathy 10.4 MDM2 TP53
37 acute cholinergic dysautonomia 10.4 MDM2 TP53
38 peritoneal benign neoplasm 10.4 GFAP TP53
39 lung leiomyosarcoma 10.4 MDM2 PTEN
40 rectum squamous cell carcinoma 10.4 MDM2 TP53
41 vulvar liposarcoma 10.4 MDM2 TP53
42 lichen nitidus 10.4 MDM2 TP53
43 childhood infratentorial neoplasm 10.4 EGFR TP53
44 peritoneal serous adenocarcinoma 10.4 IDH1 TP53
45 breast fibrosarcoma 10.4 EGFR TP53
46 endodermal sinus tumor 10.4 MDM2 TP53
47 congenital mesoblastic nephroma 10.3 EGFR TP53
48 extragonadal nonseminomatous germ cell tumor 10.3 EGFR TP53
49 secondary pulmonary hemosiderosis 10.3 MDM2 TP53
50 iris disease 10.3 GFAP INA

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

GenomeRNAi Phenotypes related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased cell death HMECs cells GR00103-A-0 8.92 EGFR MDM2 PTEN TP53

MGI Mouse Phenotypes related to Gliomatosis Cerebri:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.65 PTEN TP53 EGFR GFAP MDM2
2 muscle MP:0005369 9.55 EGFR GFAP MDM2 PTEN TP53
3 normal MP:0002873 9.43 EGFR GFAP INA MDM2 PTEN TP53
4 pigmentation MP:0001186 8.92 EGFR MDM2 PTEN TP53

Drugs & Therapeutics for Gliomatosis Cerebri

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

(show top 50) (show all 90)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Valproic Acid Approved, Investigational Phase 3,Phase 2 99-66-1 3121
2
chloroquine Approved, Vet_approved Phase 3 54-05-7 2719
3
Dacarbazine Approved, Investigational Phase 3 4342-03-4 5351166
4
Temozolomide Approved, Investigational Phase 3 85622-93-1 5394
5
Cyproheptadine Approved Phase 3 129-03-3 2913
6
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
7 Dermatologic Agents Phase 3,Phase 1
8 Anticonvulsants Phase 3,Phase 2
9 Anti-Infective Agents Phase 3,Phase 2
10 Antimanic Agents Phase 3,Phase 2
11 Central Nervous System Depressants Phase 3,Phase 2
12 GABA Agents Phase 3,Phase 2
13 Neurotransmitter Agents Phase 3,Phase 2
14 Psychotropic Drugs Phase 3,Phase 2
15 Tranquilizing Agents Phase 3,Phase 2
16 Alkylating Agents Phase 3
17 Analgesics Phase 3
18 Analgesics, Non-Narcotic Phase 3
19 Anthelmintics Phase 3
20 Anti-Inflammatory Agents Phase 3,Phase 1
21 Anti-Inflammatory Agents, Non-Steroidal Phase 3
22 Antimalarials Phase 3
23 Antiparasitic Agents Phase 3
24 Antiprotozoal Agents Phase 3
25 Antirheumatic Agents Phase 3
26 Chloroquine diphosphate Phase 3 50-63-5
27 Peripheral Nervous System Agents Phase 3,Phase 1
28 Pharmaceutical Solutions Phase 3
29 Anti-Allergic Agents Phase 3
30 Antipruritics Phase 3
31 Gastrointestinal Agents Phase 3,Phase 1
32 Histamine Antagonists Phase 3
33 Histamine H1 Antagonists Phase 3
34
Histamine Phosphate Phase 3 51-74-1 65513
35
Serotonin Phase 3 50-67-9 5202
36 Serotonin Agents Phase 3
37 Serotonin Antagonists Phase 3
38
Gefitinib Approved, Investigational Phase 1, Phase 2 184475-35-2 123631
39
nivolumab Approved Phase 2 946414-94-4
40
Benzocaine Approved Phase 2 1994-09-7, 94-09-7 2337
41
Bevacizumab Approved, Investigational Phase 2 216974-75-3
42
Acetaminophen Approved Phase 2 103-90-2 1983
43
Dexamethasone Approved, Investigational, Vet_approved Phase 2,Phase 1 50-02-2 5743
44
Diphenhydramine Approved Phase 2 58-73-1, 147-24-0 3100
45
Promethazine Approved Phase 2 60-87-7 4927
46
Crizotinib Approved Phase 1, Phase 2 877399-52-5 11626560 10366136 10366137 10366138 10366139 10366140 10366141
47 tannic acid Approved, Nutraceutical Phase 2
48
Iodine Investigational Phase 2 7553-56-2 807
49 Protein Kinase Inhibitors Phase 1, Phase 2
50 Micronutrients Phase 2,Phase 1

Interventional clinical trials:

(show all 13)

id Name Status NCT ID Phase Drugs
1 International Cooperative Phase III Trial of the HIT-HGG Study Group (HIT-HGG-2013) Not yet recruiting NCT03243461 Phase 3 Temozolomide + Valproic Acid;Temozolomide + Chloroquine
2 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
3 Gefitinib and Radiation Therapy in Treating Children With Newly Diagnosed Gliomas Completed NCT00042991 Phase 1, Phase 2 gefitinib
4 Immune Checkpoint Inhibitor Nivolumab in People With Select Rare CNS Cancers Recruiting NCT03173950 Phase 2 Nivolumab
5 Valproic Acid and Radiation Followed by Maintenance Valproic Acid and Bevacizumab in Children With High Grade Gliomas or Diffuse Intrinsic Pontine Glioma Active, not recruiting NCT00879437 Phase 2 Valproic acid;Bevacizumab
6 Iodine I 131 Monoclonal Antibody 3F8 in Treating Patients With Central Nervous System Cancer or Leptomeningeal Cancer Active, not recruiting NCT00445965 Phase 2
7 Crizotinib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Anaplastic Large Cell Lymphoma Active, not recruiting NCT00939770 Phase 1, Phase 2 crizotinib
8 Photodynamic Therapy With Porfimer Sodium in Treating Patients With Refractory Brain Tumors Unknown status NCT00002647 Phase 1 verteporfin
9 Imetelstat Sodium in Treating Young Patients With Refractory or Recurrent Solid Tumors or Lymphoma Completed NCT01273090 Phase 1 imetelstat sodium
10 Palbociclib Isethionate in Treating Younger Patients With Recurrent, Progressive, or Refractory Central Nervous System Tumors Recruiting NCT02255461 Phase 1 palbociclib isethionate
11 Oncolytic HSV-1716 in Treating Younger Patients With Refractory or Recurrent High Grade Glioma That Can Be Removed By Surgery Terminated NCT02031965 Phase 1 dexamethasone
12 18F-FDOPA PET/CT or PET/MRI in Measuring Tumors in Patients With Newly-Diagnosed or Recurrent Gliomas Terminated NCT02175745 18F-fluoro-dihydroxyphenylalanine
13 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

MalaCards organs/tissues related to Gliomatosis Cerebri:

39
Brain, Spinal Cord, Pituitary, Breast

Publications for Gliomatosis Cerebri

Articles related to Gliomatosis Cerebri:

(show top 50) (show all 234)
id Title Authors Year
1
Gliomatosis cerebri: Prognosis based on current molecular markers. ( 28539209 )
2017
2
Gliomatosis cerebri in a 10-year-old male patient. ( 28484570 )
2017
3
Management and Survival Patterns of Patients with Gliomatosis Cerebri: A SEER-Based Analysis. ( 28366748 )
2017
4
An Adolescent Presenting With Seizures as a Symptom of Gliomatosis Cerebri. ( 28486262 )
2017
5
Molecular alterations in pediatric gliomatosis cerebri are similar to those in less invasive forms of pediatric diffuse glioma. ( 28421462 )
2017
6
Gliomatosis Cerebri: Current Understanding and Controversies. ( 28824876 )
2017
7
Bevacizumab for Patients with Recurrent Gliomas Presenting with a Gliomatosis Cerebri Growth Pattern. ( 28353668 )
2017
8
Gliomatosis cerebri in children shares molecular characteristics with other pediatric gliomas. ( 26744350 )
2016
9
Gliomatosis cerebri: A consensus summary report from the First International Gliomatosis cerebri Group Meeting, March 26-27, 2015, Paris, France. ( 27466787 )
2016
10
Pediatric Gliomatosis Cerebri: A Review of 15 Years. ( 26239491 )
2016
11
Perioperative risk assessment of patients with gliomatosis cerebri. ( 27836704 )
2016
12
Nanostructured lipid carriers based temozolomide and gene co-encapsulated nanomedicine for gliomatosis cerebri combination therapy. ( 26017099 )
2016
13
Radiology Case of the Month: Gliomatosis Cerebri. ( 27598892 )
2016
14
Gliomatosis cerebri: no evidence for a separate brain tumor entity. ( 26493382 )
2016
15
Pediatric gliomatosis cerebri mimicking tubercular encephalitis. ( 26962350 )
2015
16
Gliomatosis cerebri in a 12-year-old patient. ( 25662824 )
2015
17
Gliomatosis cerebri having a poor performance status without recurrence after radiotherapy: a single institutional experience. ( 25569296 )
2015
18
Gliomatosis cerebri in L-2-hydroxyglutaric aciduria. ( 25997888 )
2015
19
Gliomatosis cerebri mimicking encephalitis evaluated using fluorine-18 fluorodeoxyglucose: Positron emission tomography/computed tomography. ( 25589818 )
2015
20
Corticosteroid sensitivity in gliomatosis cerebri delays diagnosis. ( 25922538 )
2015
21
Gliomatosis cerebri with spinal metastasis presenting with chronic meningitis in two boys. ( 26318497 )
2015
22
Gliomatosis cerebri: Case series of six cases with review of literature. ( 26752659 )
2015
23
"Fungimitosis": Invasive fulminant Aspergillus brain infection mimicking gliomatosis cerebri. ( 26053824 )
2015
24
Clinical manifestations and imaging characteristics of gliomatosis cerebri with pathological confirmation. ( 24969874 )
2014
25
A comparison of clinical, magnetic resonance imaging and pathological findings in dogs with gliomatosis cerebri, focusing on cases with minimal magnetic resonance imaging changes(a8!) ( 24945683 )
2014
26
Exclusive radiotherapy for gliomatosis cerebri: long-term follow-up at a single institution. ( 24474572 )
2014
27
Nocardia abscesses mimicking tumor progression in gliomatosis cerebri responding to temozolomide. ( 25189790 )
2014
28
Gliomatosis cerebri mimicking acute viral encephalitis and with malignant transformation of partial lesions: A case report. ( 25120625 )
2014
29
Gliomatosis Cerebri Type 1 with Extensive Involvement of the Spinal Cord and BRAF V600E Mutation. ( 24353007 )
2014
30
Procarbazine, CCNU, and Vincristine Chemotherapy in Gliomatosis Cerebri. ( 25408934 )
2014
31
Gliomatosis cerebri mimicking viral encephalitis in a 4-year-old child. ( 24717581 )
2014
32
Patterns of presentation and failure in patients with gliomatosis cerebri treated with partial-brain radiation therapy. ( 24845411 )
2014
33
Gliomatosis cerebri: a review. ( 24390814 )
2014
34
Response: gliomatosis cerebri. ( 23495379 )
2013
35
Teaching NeuroImages: diffuse cerebral neurosarcoidosis mimicking gliomatosis cerebri. ( 23940026 )
2013
36
Gliomatosis cerebri: clinical characteristics, management, and outcomes. ( 23341100 )
2013
37
Uncal decompression in gliomatosis cerebri. ( 23618524 )
2013
38
Juvenile parkinsonism as an initial manifestation of gliomatosis cerebri. ( 24158273 )
2013
39
Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases. ( 23306961 )
2013
40
18F FDG PET/CT in a child with gliomatosis cerebri. ( 23122744 )
2013
41
Infiltration of the Optic Chiasm, Nerve, and Disc by Gliomatosis Cerebri. ( 23287703 )
2013
42
Letter to the editor: gliomatosis cerebri. ( 23198839 )
2013
43
Bilateral ptosis as initial presentation of gliomatosis cerebri: case report. ( 23427031 )
2013
44
Presentation patterns and outcome of gliomatosis cerebri. ( 22740882 )
2012
45
Marked response of gliomatosis cerebri to temozolomide and whole brain radiotherapy. ( 22341931 )
2012
46
Subcutaneous tumor seeding after biopsy in gliomatosis cerebri. ( 21837541 )
2012
47
Mutated IDH1 is a favorable prognostic factor for type 2 gliomatosis cerebri. ( 21929658 )
2012
48
An initial exploration of surgery following radiotherapy for the treatment of gliomatosis cerebri. ( 23253734 )
2012
49
A rare case of gliomatosis cerebri presenting as dementia. ( 22690056 )
2012
50
MRI features of gliomatosis cerebri in a dog. ( 22413939 )
2012

Variations for Gliomatosis Cerebri

Expression for Gliomatosis Cerebri

Search GEO for disease gene expression data for Gliomatosis Cerebri.

Pathways for Gliomatosis Cerebri

Pathways related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

(show all 37)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.85 EGFR MDM2 PTEN TP53
2
Show member pathways
12.51 EGFR MDM2 PTEN TP53
3 12.46 EGFR MDM2 PTEN TP53
4 12.41 EGFR MDM2 PTEN TP53
5
Show member pathways
12.33 EGFR MDM2 TP53
6
Show member pathways
12.32 EGFR PTEN TP53
7
Show member pathways
12.26 EGFR MDM2 TP53
8 12.23 EGFR MDM2 PTEN TP53
9
Show member pathways
12.21 MDM2 PTEN TP53
10
Show member pathways
12.18 MDM2 PTEN TP53
11
Show member pathways
12.11 EGFR MDM2 TP53
12 12.1 EGFR MDM2 TP53
13
Show member pathways
12.06 MDM2 PTEN TP53
14
Show member pathways
11.93 EGFR MDM2 PTEN
15
Show member pathways
11.92 EGFR MDM2 PTEN TP53
16
Show member pathways
11.9 EGFR MDM2 PTEN
17 11.88 MDM2 PTEN TP53
18 11.81 EGFR GFAP TP53
19 11.78 MDM2 PTEN TP53
20
Show member pathways
11.75 GFAP INA NES
21 11.75 EGFR MDM2 PTEN TP53
22
Show member pathways
11.73 EGFR GFAP MDM2
23 11.7 MDM2 PTEN TP53
24 11.61 EGFR MDM2 PTEN
25 11.58 EGFR MDM2 PTEN TP53
26 11.47 EGFR MDM2 TP53
27 11.31 MDM2 TP53
28 11.3 EGFR MDM2 PTEN TP53
29 11.28 MDM2 PTEN TP53
30 11.23 PTEN TP53
31 11.17 EGFR TP53
32 11.16 MDM2 PTEN TP53
33 11.15 MDM2 TP53
34 11.12 MDM2 TP53
35 10.95 MDM2 TP53
36
Show member pathways
10.94 MDM2 TP53
37 10.74 EGFR IDH1 PTEN TP53

GO Terms for Gliomatosis Cerebri

Cellular components related to Gliomatosis Cerebri according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 9.33 GFAP INA NES
2 intermediate filament cytoskeleton GO:0045111 8.8 GFAP INA NES
3 neurofilament GO:0005883 8.65 INA

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

(show all 11)
id Name GO ID Score Top Affiliating Genes
1 protein deubiquitination GO:0016579 9.65 MDM2 PTEN TP53
2 negative regulation of apoptotic process GO:0043066 9.62 EGFR MDM2 PTEN TP53
3 response to steroid hormone GO:0048545 9.46 IDH1 MDM2
4 response to antibiotic GO:0046677 9.43 MDM2 TP53
5 response to organic cyclic compound GO:0014070 9.43 EGFR IDH1 PTEN
6 cellular response to gamma radiation GO:0071480 9.4 MDM2 TP53
7 positive regulation of protein export from nucleus GO:0046827 9.37 MDM2 TP53
8 phosphatidylinositol-mediated signaling GO:0048015 9.33 EGFR PTEN TP53
9 long-term synaptic potentiation GO:0060291 9.19 PTEN
10 cellular response to hypoxia GO:0071456 9.13 MDM2 PTEN TP53
11 cellular response to actinomycin D GO:0072717 8.62 MDM2 TP53

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

id Name GO ID Score Top Affiliating Genes
1 enzyme binding GO:0019899 9.26 EGFR MDM2 PTEN TP53
2 disordered domain specific binding GO:0097718 9.16 MDM2 TP53
3 identical protein binding GO:0042802 9.02 EGFR GFAP MDM2 PTEN TP53

Sources for Gliomatosis Cerebri

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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