MCID: ANP007
MIFTS: 29

Anaplastic Ganglioglioma

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Anaplastic Ganglioglioma

MalaCards integrated aliases for Anaplastic Ganglioglioma:

Name: Anaplastic Ganglioglioma 49 55 69

Classifications:

Orphanet: 55  
Rare neurological diseases


External Ids:

Orphanet 55 ORPHA251957
UMLS via Orphanet 70 C0431112
UMLS 69 C0431112

Summaries for Anaplastic Ganglioglioma

NIH Rare Diseases : 49 Anaplastic ganglioglioma (AGG) is a very rare type of brain tumor that is a type of ganglioglioma. In general, gangliogliomas are classified as grade I or low grade tumors, meaning that they grow slowly and are considered benign. Anaplastic gangliogliomas, however, are considered grade III or high grade tumors, which means that they are usually aggressive, malignant tumors. The main treatment is removal of the entire tumor during surgery.  If the entire tumor is not removed, it has the potential to recur and may require additional surgery or treatments, such as radiation therapy or chemotherapy.  Unfortunately, because gangliogliomas are quite rare, there is limited information to show that radiation therapy or chemotherapy are effective treatments for this condition. Last updated: 10/18/2011

MalaCards based summary : Anaplastic Ganglioglioma is related to ganglioglioma and gliosarcoma. An important gene associated with Anaplastic Ganglioglioma is ACTC1 (Actin, Alpha, Cardiac Muscle 1), and among its related pathways/superpathways are Cytoskeleton remodeling Neurofilaments and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Trametinib and Dabrafenib have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and myeloid, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Related Diseases for Anaplastic Ganglioglioma

Diseases related to Anaplastic Ganglioglioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 55)
# Related Disease Score Top Affiliating Genes
1 ganglioglioma 30.6 GFAP NES
2 gliosarcoma 28.9 ACTC1 GFAP NES
3 neuronitis 10.0
4 central nervous system teratoma 9.9 GFAP NES
5 dermatofibrosarcoma protuberans 9.9 ACTC1 NES
6 central nervous system germ cell tumor 9.9 GFAP NES
7 central nervous system primitive neuroectodermal neoplasm 9.9 GFAP NES
8 dysembryoplastic neuroepithelial tumor 9.9 GFAP NES
9 ependymoblastoma 9.9 GFAP NES
10 astroblastoma 9.9 GFAP NES
11 subependymoma 9.9 GFAP NES
12 medulloepithelioma 9.9 GFAP NES
13 tanycytic ependymoma 9.9 GFAP NES
14 papillary ependymoma 9.9 GFAP NES
15 gliomatosis cerebri 9.9 GFAP NES
16 brain ischemia 9.9 GFAP NES
17 subependymal giant cell astrocytoma 9.8 GFAP NES
18 central neurocytoma 9.8 GFAP NES
19 gastrointestinal stromal tumor 9.8 ACTC1 NES
20 retinitis pigmentosa 42 9.8 GFAP NES
21 cutaneous leiomyosarcoma 9.8 ACTC1 GFAP
22 brain glioma 9.8 GFAP NES
23 parachordoma 9.8 ACTC1 GFAP
24 neurofibromatosis, type iv, of riccardi 9.8
25 neuroblastoma 9.8
26 aging 9.8
27 leukemia, acute myeloid 9.8
28 leukemia 9.8
29 tuberous sclerosis 9.8
30 colon adenocarcinoma 9.8
31 adenocarcinoma 9.8
32 cerebritis 9.8
33 ganglioneuroblastoma 9.8
34 pleomorphic xanthoastrocytoma 9.8
35 myeloid leukemia 9.8
36 neurofibroma 9.8
37 glioblastoma 9.8
38 extraventricular neurocytoma 9.8
39 optic nerve glioma 9.8 GFAP NES
40 syringocystadenoma papilliferum 9.8 ACTC1 GFAP
41 ependymoma 9.8 GFAP NES
42 angiocentric glioma 9.8 ACTC1 GFAP
43 infratentorial cancer 9.8 GFAP NES
44 neurilemmoma 9.8 GFAP NES
45 binswanger's disease 9.8 ACTC1 GFAP
46 necrotizing sialometaplasia 9.8 ACTC1 GFAP
47 myoepithelioma 9.7 ACTC1 GFAP
48 brain cancer 9.7 GFAP NES
49 pleomorphic adenoma 9.7 ACTC1 GFAP
50 astrocytoma 9.7 GFAP NES

Graphical network of the top 20 diseases related to Anaplastic Ganglioglioma:



Diseases related to Anaplastic Ganglioglioma

Symptoms & Phenotypes for Anaplastic Ganglioglioma

GenomeRNAi Phenotypes related to Anaplastic Ganglioglioma according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-107 9.36 ACTC1
2 Increased shRNA abundance (Z-score > 2) GR00366-A-11 9.36 NES
3 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.36 ACTC1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-123 9.36 NES
5 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.36 ACTC1 NES
6 Increased shRNA abundance (Z-score > 2) GR00366-A-16 9.36 NES
7 Increased shRNA abundance (Z-score > 2) GR00366-A-186 9.36 NES
8 Increased shRNA abundance (Z-score > 2) GR00366-A-196 9.36 NES
9 Increased shRNA abundance (Z-score > 2) GR00366-A-199 9.36 ACTC1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-205 9.36 NES

Drugs & Therapeutics for Anaplastic Ganglioglioma

Drugs for Anaplastic Ganglioglioma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Trametinib Approved Phase 2 871700-17-3 11707110
2
Dabrafenib Approved, Investigational Phase 2 44462760 44516822
3 Protein Kinase Inhibitors Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Pediatric Study With Dabrafenib in Combination With Trametinib in Patients With HGG Recruiting NCT02684058 Phase 2 dabrafenib;trametinib
2 Molecularly-Driven Doublet Therapy for Recurrent CNS Malignant Neoplasms Recruiting NCT03434262 Phase 1 Gemcitabine;ribociclib;sonidegib;trametinib

Search NIH Clinical Center for Anaplastic Ganglioglioma

Genetic Tests for Anaplastic Ganglioglioma

Anatomical Context for Anaplastic Ganglioglioma

MalaCards organs/tissues related to Anaplastic Ganglioglioma:

38
Brain, Spinal Cord, Myeloid, Colon

Publications for Anaplastic Ganglioglioma

Articles related to Anaplastic Ganglioglioma:

(show all 31)
# Title Authors Year
1
Response to the BRAF/MEK inhibitors dabrafenib/trametinib in an adolescent with a BRAF V600E mutated anaplastic ganglioglioma intolerant to vemurafenib. ( 29380516 )
2018
2
Unprecedented response to combination BRAF and MEK inhibitors in adult anaplastic ganglioglioma. ( 29335912 )
2018
3
Ganglioglioma progression to combined anaplastic ganglioglioma and anaplastic pleomorphic xanthoastrocytoma. Case report and literature review. ( 28939541 )
2017
4
Pediatric intracranial primary anaplastic ganglioglioma. ( 27921213 )
2016
5
Progression of Atypical Extraventricular Neurocytoma to Anaplastic Ganglioglioma. ( 27597523 )
2016
6
Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma. ( 27539475 )
2016
7
Anaplastic ganglioglioma: a report of three cases and review of the literature. ( 25862009 )
2015
8
An Unusual Case of Constitutional Mismatch Repair Deficiency Syndrome With Anaplastic Ganglioglioma, Colonic Adenocarcinoma, Osteosarcoma, Acute Myeloid Leukemia, and Signs of Neurofibromatosis Type 1: Case Report. ( 25850602 )
2015
9
Pediatric primary anaplastic ganglioglioma: a case report and review of the literature. ( 22922381 )
2012
10
Anaplastic ganglioglioma of the brainstem in an adult. ( 24028985 )
2012
11
Anaplastic ganglioglioma: a very rare intramedullary spinal cord tumor. ( 22922809 )
2012
12
Anaplastic ganglioglioma in the spinal cord: case report and literature review. ( 23689037 )
2012
13
Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database. ( 21626070 )
2011
14
Anaplastic ganglioglioma with malignant features in both neuronal and glial components--case report. ( 20339274 )
2010
15
Anaplastic ganglioglioma in children. ( 19043777 )
2009
16
Anaplastic ganglioglioma with gliosarcoma component. ( 19900112 )
2009
17
Primary anaplastic ganglioglioma with a small-cell glioblastoma component. ( 18402388 )
2008
18
Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion. Case report. ( 18459885 )
2007
19
Anaplastic ganglioglioma in a middle-aged woman: a case report with a review of the literature. ( 17923741 )
2007
20
Primary spinal anaplastic ganglioglioma. ( 17047418 )
2006
21
Anaplastic ganglioglioma of the cerebellopontine angle. Case report. ( 16308520 )
2005
22
Anaplastic ganglioglioma with sarcomatous component: an immunohistochemical study and molecular analysis of p53 tumor suppressor gene. ( 12030414 )
2002
23
Novel cerebral lesions in the Eker rat model of tuberous sclerosis: cortical tuber and anaplastic ganglioglioma. ( 10744057 )
2000
24
Extracranial metastasis of anaplastic ganglioglioma through a ventriculoperitoneal shunt: a case report. ( 10338084 )
1999
25
Anaplastic ganglioglioma with dissemination to the spinal cord: a case report. ( 9537666 )
1998
26
Anaplastic ganglioglioma: case report and review of the literature. ( 11013696 )
1998
27
Recurrent anaplastic ganglioglioma: pathological characterization of tumor cells. Case report. ( 8847572 )
1996
28
Anaplastic ganglioglioma: case report and review of the literature. ( 7984264 )
1994
29
An immunohistochemical study of peripheral neuroblastoma, ganglioneuroblastoma, anaplastic ganglioglioma, schwannoma and neurofibroma in cattle. ( 7962722 )
1994
30
Anaplastic ganglioglioma of the brain stem demonstrating active neurosecretory features of neoplastic neuronal cells. ( 1575013 )
1992
31
Anaplastic ganglioglioma in an infant: case report and review of the literature. ( 3808232 )
1986

Variations for Anaplastic Ganglioglioma

Expression for Anaplastic Ganglioglioma

Search GEO for disease gene expression data for Anaplastic Ganglioglioma.

Pathways for Anaplastic Ganglioglioma

Pathways related to Anaplastic Ganglioglioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.99 GFAP NES
2 10.52 GFAP NES

GO Terms for Anaplastic Ganglioglioma

Cellular components related to Anaplastic Ganglioglioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 9.16 GFAP NES
2 cell body GO:0044297 8.96 ACTC1 GFAP
3 intermediate filament cytoskeleton GO:0045111 8.62 GFAP NES

Molecular functions related to Anaplastic Ganglioglioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural molecule activity GO:0005198 8.62 GFAP NES

Sources for Anaplastic Ganglioglioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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