DIG
MCID: DSM006
MIFTS: 26

Desmoplastic Infantile Ganglioglioma (DIG) malady

Rare diseases category

Summaries for Desmoplastic Infantile Ganglioglioma

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44NIH Rare Diseases, 34MalaCards
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NIH Rare Diseases:44 Desmoplastic infantile gangliomas (digs) are rare brain tumors that are normally located in the frontal or parietal lobes of the brain. they are usually diagnosed before 18 months of age with most infants presenting with a short duration of symptoms. although seizures are not commonly observed, a bulging fontanelle, rapid head growth, vomiting, and a sunset sign are usually noted. the standard treatment for digs is surgical resection (surgical procedure in which the portion of the brain with the tumor is removed). last updated: 7/30/2013

MalaCards: Desmoplastic Infantile Ganglioglioma, also known as DIG, is related to ganglioglioma and astrocytoma. An important gene associated with Desmoplastic Infantile Ganglioglioma is MYCN (v-myc avian myelocytomatosis viral oncogene neuroblastoma derived homolog). The compounds valproic acid and agar have been mentioned in the context of this disorder. Affiliated tissues include brain and parietal lobe, and related mouse phenotypes are vision/eye and liver/biliary system.

Aliases & Classifications for Desmoplastic Infantile Ganglioglioma

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44NIH Rare Diseases, 63UMLS
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases


Aliases & Descriptions:

desmoplastic infantile ganglioglioma 44 63
dig 44


Related Diseases for Desmoplastic Infantile Ganglioglioma

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18GeneCards, 19GeneDecks
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Diseases related to Desmoplastic Infantile Ganglioglioma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 13)
idRelated DiseaseScoreTop Affiliating Genes
1ganglioglioma11.1
2astrocytoma10.5
3cerebritis10.3
4pleomorphic xanthoastrocytoma10.3
5desmoplastic infantile astrocytoma/ganglioglioma10.3
6hepatitis10.0CP, MYCN
7factor v leiden thrombophilia10.0CP, MYCN
8brain cancer10.0MYCN, CP
9non-hodgkin lymphoma10.0MYCN, CP
10burkitt's lymphoma10.0MYCN, CP
11breast cancer10.0MYCN, CP
12colorectal cancer10.0MYCN, CP
13melanoma9.9MYCN, CP

Graphical network of diseases related to Desmoplastic Infantile Ganglioglioma:



Diseases related to desmoplastic infantile ganglioglioma

Symptoms for Desmoplastic Infantile Ganglioglioma

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Drugs & Therapeutics for Desmoplastic Infantile Ganglioglioma

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Desmoplastic Infantile Ganglioglioma

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Anatomical Context for Desmoplastic Infantile Ganglioglioma

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34MalaCards
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MalaCards organs/tissues related to Desmoplastic Infantile Ganglioglioma:

34
Brain, Parietal lobe

Animal Models for Desmoplastic Infantile Ganglioglioma or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Desmoplastic Infantile Ganglioglioma:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053919.1MYCN, CP
2MP:00053708.8MYCN, CP

Publications for Desmoplastic Infantile Ganglioglioma

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53PubMed
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Articles related to Desmoplastic Infantile Ganglioglioma:

(show all 43)
idTitleAuthorsYear
1
Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis*. (24355183)
2013
2
Strong desmin expression in a congenital desmoplastic infantile ganglioglioma mimicking pleomorphic rhadomyosarcoma: a case report including ultrastructural and cytogenetic evaluation and review of the literature. (22899016)
2012
3
Clinical heterogeneity of desmoplastic infantile ganglioglioma: a case series and literature review. (22735886)
2012
4
Malignant transformation of a desmoplastic infantile ganglioglioma. (21763958)
2011
5
Desmoplastic infantile ganglioglioma: Report of a case and review of the literature. (21042508)
2010
6
Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. (19139903)
2009
7
Desmoplastic infantile ganglioglioma with a malignant course. (18352812)
2008
8
Desmoplastic infantile ganglioglioma: a report of 2 cases and a review of the literature. (18703892)
2008
9
Desmoplastic infantile ganglioglioma: case report. (19102059)
2008
10
Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. (17386372)
2007
11
Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. (17719974)
2007
12
Desmoplastic infantile ganglioglioma. (17450367)
2007
13
Desmoplastic infantile ganglioglioma with calcification. (16961068)
2006
14
Desmoplastic infantile ganglioglioma. Morphological, immunohistochemical and genetic features. (16623795)
2006
15
Desmoplastic infantile ganglioglioma with high proliferation index: report of a case. (16519002)
2005
16
Desmoplastic Infantile Ganglioglioma: cytologic findings and differential diagnosis on aspiration material. (15644137)
2005
17
Atypical MRI appearance of desmoplastic infantile ganglioglioma. (15900435)
2005
18
Case of desmoplastic infantile ganglioglioma secreting ceruloplasmin. (16417865)
2005
19
Cervico-medullary desmoplastic infantile ganglioglioma: an unusual case with diffuse leptomeningeal dissemination at diagnosis. (15702481)
2005
20
Desmoplastic infantile ganglioglioma: a questionably benign tumour. (16174188)
2005
21
Desmoplastic infantile ganglioglioma: MRI and histological findings case report. (15551129)
2004
22
Desmoplastic infantile ganglioglioma: a rare tumor with an unusual presentation. (15494934)
2004
23
Desmoplastic infantile ganglioglioma -- a case report. (15472436)
2004
24
Desmoplastic infantile ganglioglioma. (12750935)
2003
25
Therapeutic strategies and management of desmoplastic infantile ganglioglioma: two case reports and literature overview. (12783262)
2003
26
Desmoplastic infantile ganglioglioma: a potentially malignant tumor? (12409729)
2002
27
Chronological changes on magnetic resonance images in a case of desmoplastic infantile ganglioglioma. (11818743)
2002
28
Desmoplastic infantile ganglioglioma--clinicopathological and immunohistochemical study of four cases. (12382166)
2002
29
Desmoplastic infantile ganglioglioma (DIG): cranial ultrasound findings. (11436886)
2001
30
Mixed conventional and desmoplastic infantile ganglioglioma: an autopsied case with 6-year follow-up. (11455006)
2001
31
Cytologic features of desmoplastic infantile ganglioglioma: a report of two cases. (11726100)
2001
32
Image interpretation session: 1999. Desmoplastic infantile ganglioglioma. (10682792)
2000
33
Desmoplastic infantile ganglioglioma. (10508884)
1999
34
High amino acid uptake in a low-grade desmoplastic infantile ganglioglioma in a 14-year-old patient. (9584283)
1998
35
Desmoplastic infantile ganglioglioma. (9408160)
1997
36
Telomere associations in desmoplastic infantile ganglioglioma. (8956861)
1996
37
Desmoplastic infantile ganglioglioma: CT and MRI features. (8545186)
1995
38
Clinical, radiological and histological findings in desmoplastic infantile ganglioglioma. (7842437)
1994
39
Pleomorphic xanthoastrocytoma and desmoplastic infantile ganglioglioma--have these neoplasms a common origin? (7889337)
1994
40
Desmoplastic infantile ganglioglioma--case report. (7692325)
1993
41
Desmoplastic infantile ganglioglioma with extraparenchymatous cyst--case report. (7683126)
1993
42
Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy. (8293187)
1993
43
Desmoplastic infantile ganglioglioma: CT and MR features. (1763751)
1991

Variations for Desmoplastic Infantile Ganglioglioma

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Expression for genes affiliated with Desmoplastic Infantile Ganglioglioma

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Desmoplastic Infantile Ganglioglioma

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Pathways for genes affiliated with Desmoplastic Infantile Ganglioglioma

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Compounds for genes affiliated with Desmoplastic Infantile Ganglioglioma

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46Novoseek, 52PharmGKB, 25HMDB, 12DrugBank, 30IUPHAR
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Compounds related to Desmoplastic Infantile Ganglioglioma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1valproic acid46 52 25 1212.3CP, MYCN
2agar469.3MYCN, CP
3iron46 2510.2CP, MYCN
4lactate469.2CP, MYCN
5dopamine46 30 25 1212.1MYCN, CP
6serine469.1CP, MYCN
7vegf469.0CP, MYCN
8retinoic acid46 259.8CP, MYCN

GO Terms for genes affiliated with Desmoplastic Infantile Ganglioglioma

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Products for genes affiliated with Desmoplastic Infantile Ganglioglioma

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Sources for Desmoplastic Infantile Ganglioglioma

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet