DIG
MCID: DSM006
MIFTS: 22

Desmoplastic Infantile Ganglioglioma (DIG)

Categories: Rare diseases

Aliases & Classifications for Desmoplastic Infantile Ganglioglioma

MalaCards integrated aliases for Desmoplastic Infantile Ganglioglioma:

Name: Desmoplastic Infantile Ganglioglioma 20 70
Dig 20

Classifications:



External Ids:

UMLS 70 C1321878

Summaries for Desmoplastic Infantile Ganglioglioma

GARD : 20 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).

MalaCards based summary : Desmoplastic Infantile Ganglioglioma, also known as dig, is related to ganglioglioma and malignant astrocytoma, and has symptoms including seizures An important gene associated with Desmoplastic Infantile Ganglioglioma is BRAF (B-Raf Proto-Oncogene, Serine/Threonine Kinase), and among its related pathways/superpathways are Neuroscience and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. Affiliated tissues include brain, cerebellum and spinal cord, and related phenotype is normal.

Related Diseases for Desmoplastic Infantile Ganglioglioma

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

(show top 50) (show all 118)
# Related Disease Score Top Affiliating Genes
1 ganglioglioma 30.4 SYP MYCN GFAP BRAF
2 malignant astrocytoma 30.2 GFAP BRAF
3 gangliocytoma 30.1 SYP GFAP
4 pleomorphic xanthoastrocytoma 29.8 SYP GFAP BRAF
5 glioblastoma 29.7 MYCN GFAP BRAF
6 pilocytic astrocytoma 29.7 SYP GFAP BRAF
7 rhabdomyosarcoma 29.3 SYP MYCN GFAP
8 brain cancer 29.1 SYP MYCN GFAP BRAF
9 rare tumor 10.6
10 glioma susceptibility 1 10.6
11 cutis marmorata telangiectatica congenita 10.1
12 low grade glioma 10.1
13 rapidly involuting congenital hemangioma 10.1
14 subdural empyema 10.1
15 anaplastic astrocytoma 10.1
16 pleural empyema 10.1
17 intracranial hypertension 10.1
18 desmoplastic infantile astrocytoma 10.1
19 aneurysm 10.1
20 cerebral aneurysms 10.1
21 seizure disorder 10.1
22 papillary tumor of the pineal region 10.1 SYP BRAF
23 extraventricular neurocytoma 10.0 SYP GFAP
24 mature teratoma 10.0 SYP BRAF
25 cerebral neuroblastoma 10.0 SYP GFAP
26 lung combined type small cell carcinoma 10.0 SYP GFAP
27 cerebellopontine angle primitive neuroectodermal tumor 10.0 SYP GFAP
28 papillary ependymoma 10.0 SYP GFAP
29 angiocentric glioma 10.0 SYP GFAP
30 subependymoma 10.0 SYP GFAP
31 pineocytoma 10.0 SYP GFAP
32 chiari malformation 10.0 SYP GFAP
33 tanycytic ependymoma 10.0 SYP GFAP
34 medullomyoblastoma 10.0 SYP GFAP
35 spinal cord oligodendroglioma 10.0 SYP GFAP
36 oligodendroglioma 10.0 SYP GFAP
37 endobronchial leiomyoma 10.0 SYP GFAP
38 syringocystadenoma papilliferum 10.0 GFAP BRAF
39 cauda equina syndrome 10.0 SYP GFAP
40 binswanger's disease 10.0 SYP GFAP
41 chordoid meningioma 10.0 SYP GFAP
42 anaplastic ependymoma 10.0 SYP GFAP
43 cerebrovascular benign neoplasm 10.0 SYP GFAP
44 olfactory nerve neoplasm 10.0 SYP GFAP
45 cranial nerve malignant neoplasm 10.0 SYP GFAP
46 cerebellar medulloblastoma 10.0 SYP GFAP
47 clear cell ependymoma 10.0 SYP GFAP
48 pineal gland cancer 10.0 SYP GFAP
49 chordoid glioma 10.0 SYP GFAP
50 cranial nerve neoplasm 10.0 SYP GFAP

Graphical network of the top 20 diseases related to Desmoplastic Infantile Ganglioglioma:



Diseases related to Desmoplastic Infantile Ganglioglioma

Symptoms & Phenotypes for Desmoplastic Infantile Ganglioglioma

UMLS symptoms related to Desmoplastic Infantile Ganglioglioma:


seizures

MGI Mouse Phenotypes related to Desmoplastic Infantile Ganglioglioma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 normal MP:0002873 8.92 BRAF GFAP MYCN SYP

Drugs & Therapeutics for Desmoplastic Infantile Ganglioglioma

Search Clinical Trials , NIH Clinical Center for Desmoplastic Infantile Ganglioglioma

Genetic Tests for Desmoplastic Infantile Ganglioglioma

Anatomical Context for Desmoplastic Infantile Ganglioglioma

MalaCards organs/tissues related to Desmoplastic Infantile Ganglioglioma:

40
Brain, Cerebellum, Spinal Cord

Publications for Desmoplastic Infantile Ganglioglioma

Articles related to Desmoplastic Infantile Ganglioglioma:

(show top 50) (show all 113)
# Title Authors PMID Year
1
Conditional reprograming culture conditions facilitate growth of lower grade glioma models. 61
33258947 2020
2
Molecular and clinicopathologic features of gliomas harboring NTRK fusions. 61
32665022 2020
3
Desmoplastic Infantile Ganglioglioma: A MAPK Pathway-Driven and Microglia/Macrophage-Rich Neuroepithelial Tumor. 61
31562743 2019
4
Mixed Solid and Cystic Mass in an Infant. 61
31582387 2019
5
Desmoplastic Infantile Ganglioglioma Masquerading as an Arachnoid Cyst. 61
31555425 2019
6
The great neurosurgical masquerader: 3 cases of desmoplastic infantile ganglioglioma. 61
31277058 2019
7
Desmoplastic infantile ganglioglioma. 61
31223403 2019
8
The Role of 5-ALA in Low-Grade Gliomas and the Influence of Antiepileptic Drugs on Intraoperative Fluorescence. 61
31192128 2019
9
Novel BRAF alteration in desmoplastic infantile ganglioglioma with response to targeted therapy. 61
30396366 2018
10
Pipeline-assisted coil embolization of a large middle cerebral artery pseudoaneurysm in a 9-month-old infant: experience from the youngest flow diversion case. 61
30141750 2018
11
Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIG/DIA) Are Distinct Entities with Frequent BRAFV600 Mutations. 61
30006355 2018
12
Magnetic resonance imaging findings of mixed neuronal-glial tumors with pathologic correlation: a review. 61
29987554 2018
13
A Rare Tumor in Childhood Desmoplastic Infantile Astrocytoma: Two Case Reports. 61
32595403 2018
14
Progressive Disordered Movements in an Infant Leads to Rare Diagnosis. 61
29849427 2017
15
Unusual Occurrence of Multifocal Desmoplastic Infantile Astrocytoma: A Case Report and Review of the Literature. 61
28222441 2017
16
Desmoplastic infantile astrocytoma and ganglioglioma: case report and review of the literature. 61
27668845 2017
17
Supratentorial tumors typical of the infantile age: desmoplastic infantile ganglioglioma (DIG) and astrocytoma (DIA). A review. 61
27659826 2016
18
Desmoplastic infantile ganglioglioma with focal cortical dysplasia: A rare double pathology in an infant with history of seizures. 61
26991995 2016
19
Diffusion, Perfusion, and Histopathologic Characteristics of Desmoplastic Infantile Ganglioglioma. 61
27761184 2016
20
Desmoplastic infantile ganglioglioma with associated giant aneurysm—case report. 61
25922053 2015
21
Non-infantile variant of desmoplastic ganglioglioma: Conventional and advanced MR imaging characteristics. 61
26246092 2015
22
A case of giant intracranial tuberculoma in an infant: clinical and radiologic pitfalls. 61
24832400 2015
23
Malignant transformation of a desmoplastic infantile ganglioglioma in an infant carrier of a nonsynonymous TP53 mutation. 61
24768217 2014
24
Desmoplastic infantile ganglioglioma: Report of an unusual case with a cranial defect. 61
24891905 2014
25
Desmoplastic infantile ganglioglioma with late presentation. A clinical, radiological and histopathological analysis. 61
24355183 2013
26
Genome-wide DNA copy number analysis of desmoplastic infantile astrocytomas and desmoplastic infantile gangliogliomas. 61
23965740 2013
27
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. 61
22899016 2012
28
Clinical heterogeneity of desmoplastic infantile ganglioglioma: a case series and literature review. 61
22735886 2012
29
Cyst with a mural nodule tumor of the brain. 61
22935908 2012
30
Surgical treatment of brain tumors in infants younger than six months of age and review of the literature. 61
22120270 2012
31
Pathological spectrum of neuronal/glioneuronal tumors from a tertiary referral neurological Institute. 61
21410777 2012
32
Desmoplastic infantile astrocytoma: recurrence with malignant transformation into glioblastoma: a case report. 61
21947035 2011
33
Malignant transformation of a desmoplastic infantile ganglioglioma. 61
21763958 2011
34
Desmoplastic non-infantile ganglioglioma in late adulthood. 61
20857318 2011
35
Activating mutations in BRAF characterize a spectrum of pediatric low-grade gliomas. 61
20156809 2010
36
Desmoplastic infantile astrocytoma with benign histological phenotype and multiple intracranial localizations at presentation. 61
20012157 2010
37
Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature. 61
21246390 2010
38
Desmoplastic infantile ganglioglioma: Report of a case and review of the literature. 61
21042508 2010
39
A case of melanotic desmoplastic ganglioglioma. 61
19622111 2009
40
Imaging of desmoplastic infantile ganglioglioma: a spectroscopic viewpoint. 61
19139903 2009
41
Desmoplastic infantile ganglioglioma: case report. 61
19102059 2008
42
Gliomas in neurofibromatosis type 1: a clinicopathologic study of 100 patients. 61
18344915 2008
43
Desmoplastic infantile ganglioglioma with a malignant course. 61
18352812 2008
44
Desmoplastic infantile ganglioglioma: a report of 2 cases and a review of the literature. 61
18703892 2008
45
Desmoplastic infantile ganglioglioma: novel aspects in clinical presentation and genetics. 61
17719974 2007
46
Desmoplastic infantile ganglioglioma. 61
17450367 2007
47
Desmoplastic infantile ganglioglioma/astrocytoma with cerebrospinal metastasis. 61
17386372 2007
48
Desmoplastic noninfantile ganglioglioma: report of a case. 61
17163789 2006
49
Desmoplastic infantile ganglioglioma with calcification. 61
16961068 2006
50
Imaging surgical epilepsy in children. 61
16838193 2006

Variations for Desmoplastic Infantile Ganglioglioma

Expression for Desmoplastic Infantile Ganglioglioma

Search GEO for disease gene expression data for Desmoplastic Infantile Ganglioglioma.

Pathways for Desmoplastic Infantile Ganglioglioma

Pathways related to Desmoplastic Infantile Ganglioglioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.33 SYP MYCN GFAP
2 10.85 SYP GFAP

GO Terms for Desmoplastic Infantile Ganglioglioma

Cellular components related to Desmoplastic Infantile Ganglioglioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell body GO:0044297 8.62 GFAP BRAF

Biological processes related to Desmoplastic Infantile Ganglioglioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 long-term synaptic potentiation GO:0060291 8.62 GFAP BRAF

Molecular functions related to Desmoplastic Infantile Ganglioglioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 kinase binding GO:0019900 8.62 MYCN GFAP

Sources for Desmoplastic Infantile Ganglioglioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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