MCID: ANP007
MIFTS: 31

Anaplastic Ganglioglioma

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Anaplastic Ganglioglioma

MalaCards integrated aliases for Anaplastic Ganglioglioma:

Name: Anaplastic Ganglioglioma 54 60 74

Classifications:

Orphanet: 60  
Rare neurological diseases


External Ids:

UMLS via Orphanet 75 C0431112
Orphanet 60 ORPHA251957
UMLS 74 C0431112

Summaries for Anaplastic Ganglioglioma

NIH Rare Diseases : 54 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.

MalaCards based summary : Anaplastic Ganglioglioma is related to ganglioglioma and gliosarcoma. An important gene associated with Anaplastic Ganglioglioma is GFAP (Glial Fibrillary Acidic Protein), and among its related pathways/superpathways are Cytoskeleton remodeling Neurofilaments and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Dabrafenib and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include brain, spinal cord and colon, 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 57)
# Related Disease Score Top Affiliating Genes
1 ganglioglioma 31.2 GFAP NES
2 gliosarcoma 29.3 ACTC1 GFAP NES
3 central nervous system germ cell tumor 10.0 GFAP NES
4 central nervous system primitive neuroectodermal neoplasm 10.0 GFAP NES
5 dysembryoplastic neuroepithelial tumor 10.0 GFAP NES
6 ependymoblastoma 10.0 GFAP NES
7 astroblastoma 10.0 GFAP NES
8 medulloepithelioma 10.0 GFAP NES
9 subependymoma 10.0 GFAP NES
10 ependymoma 10.0 GFAP NES
11 tanycytic ependymoma 10.0 GFAP NES
12 papillary ependymoma 10.0 GFAP NES
13 gliomatosis cerebri 10.0 GFAP NES
14 brain ischemia 10.0 GFAP NES
15 central neurocytoma 10.0 GFAP NES
16 neurofibromatosis, type i 10.0
17 neurofibromatosis, type iv, of riccardi 10.0
18 neuroblastoma 10.0
19 leukemia, acute myeloid 10.0
20 leukemia 10.0
21 tuberous sclerosis 10.0
22 adenocarcinoma 10.0
23 ganglioneuroblastoma 10.0
24 pleomorphic xanthoastrocytoma 10.0
25 myeloid leukemia 10.0
26 neurofibroma 10.0
27 anaplastic pleomorphic xanthoastrocytoma 10.0
28 glioblastoma 10.0
29 seizure disorder 10.0
30 extraventricular neurocytoma 10.0
31 subependymal giant cell astrocytoma 10.0 GFAP NES
32 retinitis pigmentosa 42 10.0 GFAP NES
33 brain glioma 10.0 GFAP NES
34 optic nerve glioma 10.0 GFAP NES
35 infratentorial cancer 10.0 GFAP NES
36 neurilemmoma 9.9 GFAP NES
37 supratentorial cancer 9.9 GFAP NES
38 cerebrum cancer 9.9 GFAP NES
39 central nervous system cancer 9.9 GFAP NES
40 nervous system cancer 9.9 GFAP NES
41 nervous system disease 9.9 GFAP NES
42 cutaneous leiomyosarcoma 9.9 ACTC1 GFAP
43 parachordoma 9.9 ACTC1 GFAP
44 syringocystadenoma papilliferum 9.9 ACTC1 GFAP
45 angiocentric glioma 9.8 ACTC1 GFAP
46 hydrocephalus 9.8 GFAP NES
47 binswanger's disease 9.8 ACTC1 GFAP
48 necrotizing sialometaplasia 9.8 ACTC1 GFAP
49 glioblastoma multiforme 9.8 GFAP NES
50 myoepithelioma 9.8 ACTC1 GFAP

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:

27
# 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):

(show all 16)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dabrafenib Approved, Investigational Phase 2 1195765-45-7 44462760 44516822
2
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
3
Carboplatin Approved Phase 2 41575-94-4 38904 10339178 498142
4
Trametinib Approved Phase 2,Phase 1 871700-17-3 11707110
5 Protein Kinase Inhibitors Phase 2,Phase 1
6 Antimitotic Agents Phase 2
7 Antineoplastic Agents, Phytogenic Phase 2
8
Gemcitabine Approved Phase 1 95058-81-4 60750
9
Lenograstim Approved, Investigational Phase 1 135968-09-1
10 Antimetabolites, Antineoplastic Phase 1
11 Immunologic Factors Phase 1
12 Antimetabolites Phase 1
13 Anti-Infective Agents Phase 1
14 Adjuvants, Immunologic Phase 1
15 Immunosuppressive Agents Phase 1
16 Antiviral Agents Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Pediatric Study With Dabrafenib in Combination With Trametinib in Patients With HGG and LGG Recruiting NCT02684058 Phase 2 dabrafenib;trametinib;Carboplatin with vincristine
2 SJDAWN: St. Jude Children's Research Hospital Phase 1 Study Evaluating Molecularly-Driven Doublet Therapies for Children and Young Adults With Recurrent Brain Tumors 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:

42
Brain, Spinal Cord, Colon, Myeloid

Publications for Anaplastic Ganglioglioma

Articles related to Anaplastic Ganglioglioma:

(show all 37)
# Title Authors Year
1
Impact of surgery, adjuvant treatment, and other prognostic factors in the management of anaplastic ganglioglioma. ( 29594461 )
2018
2
Response to the BRAF/MEK inhibitors dabrafenib/trametinib in an adolescent with a BRAF V600E mutated anaplastic ganglioglioma intolerant to vemurafenib. ( 29380516 )
2018
3
Unprecedented response to combination BRAF and MEK inhibitors in adult anaplastic ganglioglioma. ( 29335912 )
2018
4
Anaplastic ganglioglioma with epithelioid cell components. ( 29869356 )
2018
5
Subtotal Resection of an Anaplastic Ganglioglioma in Pregnancy. ( 29862102 )
2018
6
Cerebellopontine angle anaplastic ganglioglioma masquerading as vestibular schwannoma: an unusual entity. ( 29929035 )
2018
7
Anaplastic Ganglioglioma Involving the Entire Length of the Spinal Cord. ( 29502115 )
2018
8
Pediatric primary anaplastic ganglioglioma with malignant neuronal component. ( 30102489 )
2018
9
Ganglioglioma progression to combined anaplastic ganglioglioma and anaplastic pleomorphic xanthoastrocytoma. Case report and literature review. ( 28939541 )
2017
10
Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma. ( 27539475 )
2016
11
Progression of Atypical Extraventricular Neurocytoma to Anaplastic Ganglioglioma. ( 27597523 )
2016
12
Pediatric intracranial primary anaplastic ganglioglioma. ( 27921213 )
2016
13
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
14
Anaplastic ganglioglioma: a report of three cases and review of the literature. ( 25862009 )
2015
15
Anaplastic ganglioglioma of the brainstem in an adult. ( 24028985 )
2012
16
Pediatric primary anaplastic ganglioglioma: a case report and review of the literature. ( 22922381 )
2012
17
Anaplastic ganglioglioma in the spinal cord: case report and literature review. ( 23689037 )
2012
18
Anaplastic ganglioglioma: a very rare intramedullary spinal cord tumor. ( 22922809 )
2012
19
Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database. ( 21626070 )
2011
20
Anaplastic ganglioglioma with malignant features in both neuronal and glial components--case report. ( 20339274 )
2010
21
Anaplastic ganglioglioma in children. ( 19043777 )
2009
22
Anaplastic ganglioglioma with gliosarcoma component. ( 19900112 )
2009
23
Primary anaplastic ganglioglioma with a small-cell glioblastoma component. ( 18402388 )
2008
24
Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion. Case report. ( 18459885 )
2007
25
Anaplastic ganglioglioma in a middle-aged woman: a case report with a review of the literature. ( 17923741 )
2007
26
Primary spinal anaplastic ganglioglioma. ( 17047418 )
2006
27
Anaplastic ganglioglioma of the cerebellopontine angle. Case report. ( 16308520 )
2005
28
Anaplastic ganglioglioma with sarcomatous component: an immunohistochemical study and molecular analysis of p53 tumor suppressor gene. ( 12030414 )
2002
29
Novel cerebral lesions in the Eker rat model of tuberous sclerosis: cortical tuber and anaplastic ganglioglioma. ( 10744057 )
2000
30
Extracranial metastasis of anaplastic ganglioglioma through a ventriculoperitoneal shunt: a case report. ( 10338084 )
1999
31
Anaplastic ganglioglioma: case report and review of the literature. ( 11013696 )
1998
32
Anaplastic ganglioglioma with dissemination to the spinal cord: a case report. ( 9537666 )
1998
33
Recurrent anaplastic ganglioglioma: pathological characterization of tumor cells. Case report. ( 8847572 )
1996
34
Anaplastic ganglioglioma: case report and review of the literature. ( 7984264 )
1994
35
An immunohistochemical study of peripheral neuroblastoma, ganglioneuroblastoma, anaplastic ganglioglioma, schwannoma and neurofibroma in cattle. ( 7962722 )
1994
36
Anaplastic ganglioglioma of the brain stem demonstrating active neurosecretory features of neoplastic neuronal cells. ( 1575013 )
1992
37
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
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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