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 53 59 73

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA251957
UMLS via Orphanet 74 C0431112
UMLS 73 C0431112

Summaries for Anaplastic Ganglioglioma

NIH Rare Diseases : 53 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 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 Dabrafenib and Trametinib 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 56)
# Related Disease Score Top Affiliating Genes
1 ganglioglioma 30.4 GFAP NES
2 gliosarcoma 28.4 ACTC1 GFAP NES
3 neuronitis 10.0
4 central nervous system teratoma 9.8 GFAP NES
5 central nervous system germ cell tumor 9.8 GFAP NES
6 central nervous system primitive neuroectodermal neoplasm 9.8 GFAP NES
7 neurofibromatosis, type i 9.8
8 neurofibromatosis, type iv, of riccardi 9.8
9 neuroblastoma 9.8
10 aging 9.8
11 leukemia, acute myeloid 9.8
12 leukemia 9.8
13 tuberous sclerosis 9.8
14 colon adenocarcinoma 9.8
15 adenocarcinoma 9.8
16 cerebritis 9.8
17 ganglioneuroblastoma 9.8
18 pleomorphic xanthoastrocytoma 9.8
19 myeloid leukemia 9.8
20 neurofibroma 9.8
21 glioblastoma 9.8
22 extraventricular neurocytoma 9.8
23 dermatofibrosarcoma protuberans 9.8 ACTC1 NES
24 dysembryoplastic neuroepithelial tumor 9.8 GFAP NES
25 ependymoblastoma 9.8 GFAP NES
26 astroblastoma 9.8 GFAP NES
27 medulloepithelioma 9.8 GFAP NES
28 subependymoma 9.8 GFAP NES
29 tanycytic ependymoma 9.8 GFAP NES
30 papillary ependymoma 9.8 GFAP NES
31 gliomatosis cerebri 9.8 GFAP NES
32 brain ischemia 9.8 GFAP NES
33 subependymal giant cell astrocytoma 9.8 GFAP NES
34 gastrointestinal stromal tumor 9.8 ACTC1 NES
35 central neurocytoma 9.8 GFAP NES
36 brain glioma 9.7 GFAP NES
37 cutaneous leiomyosarcoma 9.7 ACTC1 GFAP
38 parachordoma 9.7 ACTC1 GFAP
39 retinitis pigmentosa 42 9.7 GFAP NES
40 syringocystadenoma papilliferum 9.7 ACTC1 GFAP
41 optic nerve glioma 9.7 GFAP NES
42 angiocentric glioma 9.7 ACTC1 GFAP
43 infratentorial cancer 9.7 GFAP NES
44 neurilemmoma 9.6 GFAP NES
45 binswanger's disease 9.6 ACTC1 GFAP
46 necrotizing sialometaplasia 9.6 ACTC1 GFAP
47 ependymoma 9.6 GFAP NES
48 myoepithelioma 9.6 ACTC1 GFAP
49 atypical teratoid rhabdoid tumor 9.5 ACTC1 GFAP
50 hydrocephalus 9.5 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:

26
# 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
Dabrafenib Approved, Investigational Phase 2 1195765-45-7 44462760 44516822
2
Trametinib Approved Phase 2 871700-17-3 11707110
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 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:

41
Brain, Spinal Cord, Myeloid, Colon

Publications for Anaplastic Ganglioglioma

Articles related to Anaplastic Ganglioglioma:

(show all 36)
# 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
Ganglioglioma progression to combined anaplastic ganglioglioma and anaplastic pleomorphic xanthoastrocytoma. Case report and literature review. ( 28939541 )
2017
9
Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma. ( 27539475 )
2016
10
Progression of Atypical Extraventricular Neurocytoma to Anaplastic Ganglioglioma. ( 27597523 )
2016
11
Pediatric intracranial primary anaplastic ganglioglioma. ( 27921213 )
2016
12
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
13
Anaplastic ganglioglioma: a report of three cases and review of the literature. ( 25862009 )
2015
14
Anaplastic ganglioglioma of the brainstem in an adult. ( 24028985 )
2012
15
Pediatric primary anaplastic ganglioglioma: a case report and review of the literature. ( 22922381 )
2012
16
Anaplastic ganglioglioma in the spinal cord: case report and literature review. ( 23689037 )
2012
17
Anaplastic ganglioglioma: a very rare intramedullary spinal cord tumor. ( 22922809 )
2012
18
Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database. ( 21626070 )
2011
19
Anaplastic ganglioglioma with malignant features in both neuronal and glial components--case report. ( 20339274 )
2010
20
Anaplastic ganglioglioma in children. ( 19043777 )
2009
21
Anaplastic ganglioglioma with gliosarcoma component. ( 19900112 )
2009
22
Primary anaplastic ganglioglioma with a small-cell glioblastoma component. ( 18402388 )
2008
23
Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion. Case report. ( 18459885 )
2007
24
Anaplastic ganglioglioma in a middle-aged woman: a case report with a review of the literature. ( 17923741 )
2007
25
Primary spinal anaplastic ganglioglioma. ( 17047418 )
2006
26
Anaplastic ganglioglioma of the cerebellopontine angle. Case report. ( 16308520 )
2005
27
Anaplastic ganglioglioma with sarcomatous component: an immunohistochemical study and molecular analysis of p53 tumor suppressor gene. ( 12030414 )
2002
28
Novel cerebral lesions in the Eker rat model of tuberous sclerosis: cortical tuber and anaplastic ganglioglioma. ( 10744057 )
2000
29
Extracranial metastasis of anaplastic ganglioglioma through a ventriculoperitoneal shunt: a case report. ( 10338084 )
1999
30
Anaplastic ganglioglioma: case report and review of the literature. ( 11013696 )
1998
31
Anaplastic ganglioglioma with dissemination to the spinal cord: a case report. ( 9537666 )
1998
32
Recurrent anaplastic ganglioglioma: pathological characterization of tumor cells. Case report. ( 8847572 )
1996
33
Anaplastic ganglioglioma: case report and review of the literature. ( 7984264 )
1994
34
An immunohistochemical study of peripheral neuroblastoma, ganglioneuroblastoma, anaplastic ganglioglioma, schwannoma and neurofibroma in cattle. ( 7962722 )
1994
35
Anaplastic ganglioglioma of the brain stem demonstrating active neurosecretory features of neoplastic neuronal cells. ( 1575013 )
1992
36
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 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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