MCID: RHB017
MIFTS: 50

Rhabdoid Tumor malady

Rare diseases, Cancer diseases categories

Summaries for Rhabdoid Tumor

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66Wikipedia, 34MalaCards
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Wikipedia:66 Malignant rhabdoid tumour (MRT) is a very aggressive form of tumour originally described as a variant of... more...

MalaCards: Rhabdoid Tumor, also known as rhabdoid sarcoma, is related to epithelioid sarcoma and medulloblastoma. An important gene associated with Rhabdoid Tumor is SMARCB1 (SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1), and among its related pathways are BRCA1 Pathway and Transcription Ligand dependent activation of the ESR1 SP pathway. The compounds eosin and orcein have been mentioned in the context of this disorder. Affiliated tissues include kidney, brain and liver, and related mouse phenotypes are digestive/alimentary and tumorigenesis.

Aliases & Classifications for Rhabdoid Tumor

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44NIH Rare Diseases, 46Novoseek, 63UMLS
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Classifications:

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


Aliases & Descriptions:

rhabdoid tumor 44 46 63
rhabdoid sarcoma 44


Related Diseases for Rhabdoid Tumor

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18GeneCards, 19GeneDecks
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Diseases related to Rhabdoid Tumor via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 140)
idRelated DiseaseScoreTop Affiliating Genes
1epithelioid sarcoma30.9SMARCB1, VIM, MUC1
2medulloblastoma30.8SYP, ACTC1, SMARCB1, IGF2, DES
3sarcoma30.7DES, IGF2, SMARCB1, VIM, MUC1, ACTC1
4wilms tumor30.6DES, IGF2, SMARCB1, VIM, ACTC1
5ganglioglioma30.6SYP
6choroid plexus carcinoma30.5SMARCB1
7hemangioma30.4ACTC1
8rhabdomyosarcoma30.3DES, IGF2, SMARCB1, VIM, ACTC1
9fibrous histiocytoma30.3DES, VIM, ACTC1
10chondrosarcoma30.1MUC1, VIM, DES
11adenocarcinoma30.0SPP1, SMARCA4, VIM, MUC1, ACTC1, SYP
12adenoma30.0SYP, ACTC1, MUC1, SMARCA4, IGF2
13pleomorphic adenoma29.9DES, IGF2, VIM, MUC1, ACTC1
14meningioma29.9IGF2, SMARCB1, VIM, MUC1, SYP
15atypical teratoid rhabdoid tumor11.1
16rhabdoid tumors, somatic10.6
17primitive neuroectodermal tumor10.6
18rhabdoid tumor predisposition syndrome 210.5
19cerebritis10.4
20cervicitis10.4
21tumor predisposition syndrome10.4
22rhabdoid tumor predisposition syndrome 110.3
23choroiditis10.3
24hepatoblastoma10.3
25thyroiditis10.3
26hypercalcemia10.2
27kidney rhabdoid cancer10.2
28histiocytoma10.2
29meningitis10.2
30neuroblastoma10.2
31congenital mesoblastic nephroma10.1SMARCB1
32retroperitoneal leiomyosarcoma10.1SMARCB1
33infantile myofibromatosis10.1DES
34neurilemmoma10.1SMARCB1
35fasciitis10.1ACTC1
36syringoma10.1MUC1
37melanotic neuroectodermal tumor10.1SYP
38rhabdoid cancer10.1
39fibrous meningioma10.1MUC1
40subependymal giant cell astrocytoma10.1SYP
41hidradenoma10.1MUC1
42adenomatoid tumor10.1VIM
43cystadenocarcinoma10.1MUC1
44osteofibrous dysplasia10.1SPP1
45desmoid tumor10.1ACTC1, DES
46cutaneous fibrous histiocytoma10.1DES, ACTC1
47adenosarcoma10.1ACTC1, DES
48teratoma10.1ACTC1, SMARCB1
49desmoplastic small round cell tumor10.1DES, MUC1
50thymus cancer10.0MUC1, SYP

Graphical network of the top 20 diseases related to Rhabdoid Tumor:



Diseases related to rhabdoid tumor

Symptoms for Rhabdoid Tumor

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Drugs & Therapeutics for Rhabdoid Tumor

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

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Drug clinical trials:

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Search CenterWatch for Rhabdoid Tumor

Genetic Tests for Rhabdoid Tumor

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Anatomical Context for Rhabdoid Tumor

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34MalaCards
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MalaCards organs/tissues related to Rhabdoid Tumor:

34
Kidney, Brain, Liver, Pineal, Lung, Uterus, Skin, Thyroid, Cerebellum, Ovary, Colon, Tongue, Thalamus, Occipital lobe, Prostate, Adipocyte, Spinal cord, Breast, Adrenal gland, Heart, Smooth muscle, Lymph node

Animal Models for Rhabdoid Tumor or affiliated genes

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38MGI
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Publications for Rhabdoid Tumor

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53PubMed
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Articles related to Rhabdoid Tumor:

(show top 50)    (show all 493)
idTitleAuthorsYear
1
Large congenital melanocytic nevi with atypical teratoid/rhabdoid tumor. (23519929)
2013
2
CRX/OTX3: a useful marker in the differential diagnosis of tumors of the pineal region and indicator of photoreceptor differentiation in medulloblastomas and atypical teratoid rhabdoid tumors. (23235340)
2013
3
Primary rhabdoid tumor with low grade glioma component of the central nervous system in a young adult. (22765644)
2013
4
The histone deacetylase inhibitor SAHA acts in synergism with fenretinide and doxorubicin to control growth of rhabdoid tumor cells. (23764045)
2013
5
Absence of oncogenic canonical pathway mutations in aggressive pediatric rhabdoid tumors. (22997201)
2012
6
Treatment-related morbidity in atypical teratoid/rhabdoid tumor: multifocal necrotizing leukoencephalopathy. (21613772)
2011
7
Supratentorial atypical teratoid rhabdoid tumor: An uncommon childhood tumor. (21977104)
2011
8
Nonsense mutation and inactivation of SMARCA4 (BRG1) in an atypical teratoid/rhabdoid tumor showing retained SMARCB1 (INI1) expression. (21566516)
2011
9
Systematic analysis of the antiproliferative effects of novel and standard anticancer agents in rhabdoid tumor cell lines. (20147838)
2010
10
Adult variant of atypical teratoid/rhabdoid tumor: immunohistochemical and ultrastructural confirmation of a rare tumor in the sella tursica. (20705400)
2010
11
Fetal and neonatal rhabdoid tumor. (20223330)
2010
12
Clinical and molecular features in patients with atypical teratoid rhabdoid tumor or malignant rhabdoid tumor. (19902524)
2010
13
Imprinted CDKN1C is a tumor suppressor in rhabdoid tumor and activated by restoration of SMARCB1 and histone deacetylase inhibitors. (19221586)
2009
14
Atypical teratoid/rhabdoid tumor of the spine in a 4-year-old girl. (19073850)
2008
15
Atypical teratoid/rhabdoid tumor of the central nervous system associated with congenital cataract. (18723966)
2008
16
Mutation of the INI1 gene in composite rhabdoid tumor of the endometrium. (17376508)
2007
17
Atypical teratoid/rhabdoid tumor of the spinal canal. (16411024)
2006
18
Absence of expression of SMARCB1/INI1 in malignant rhabdoid tumors of the central nervous system, kidneys and soft tissue: an immunohistochemical study with implications for diagnosis. (16528370)
2006
19
Treatment of stage IV malignant rhabdoid tumor of the kidney (MRTK) with ICE and VDCy: a case report. (16772877)
2006
20
Atypical teratoid rhabdoid tumor in a child with neurofibromatosis 1. (16086409)
2006
21
Cutaneous extrarenal rhabdoid tumor with myogenic differentiation. (16293182)
2005
22
Rhabdoid tumor of the thyroid gland: a variant of anaplastic carcinoma. (15737050)
2005
23
Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. (15803379)
2005
24
E, N-cadherins and beta-catenin expression in medulloblastoma and atypical teratoid/rhabdoid tumor. (15508347)
2004
25
Cytologic profile of rhabdoid tumor of the kidney. A report of 3 cases. (14674079)
2003
26
The role of INI1 and the SWI/SNF complex in the development of rhabdoid tumors: meeting summary from the workshop on childhood atypical teratoid/rhabdoid tumors. (11782395)
2002
27
Primary intracranial atypical teratoid/rhabdoid tumor in a child: a case report. (12378033)
2002
28
Subcellular distribution of cytokeratin and vimentin in malignant rhabdoid tumor: three-dimensional imaging with confocal laser scanning microscopy and double immunofluorescence. (11557780)
2001
29
Cytokeratin subunits of inclusion bodies in rhabdoid cells: immunohistochemical and clinicopathological study of malignant rhabdoid tumor and epithelioid sarcoma. (11469343)
2001
30
Atypical teratoid/rhabdoid tumor or medulloblastoma? (11344497)
2001
31
Abdominal rhabdoid tumor presenting as fetal hydrops: a case report. (11846311)
2001
32
CSF cytology of atypical teratoid/rhabdoid tumor of the brain in a two-year-old girl: a case report. (11074628)
2000
33
Malignant rhabdoid tumor: A phenotype? An entity?--A controversy revisited. (10809224)
2000
34
Congenital disseminated malignant rhabdoid tumor: a distinct clinicopathologic entity demonstrating abnormalities of chromosome 22q11. (10078913)
1999
35
Congenital malignant rhabdoid tumor of the orbit. (10532580)
1999
36
Rhabdoid tumor of the kidney arising in a newborn infant: description of a case with ultrastructural observations]. (10536466)
1999
37
Atypical teratoid/rhabdoid tumor--a stereogram unveiled. (10331072)
1999
38
Simultaneous presentation of atypical teratoid/rhabdoid tumor in siblings. (10448873)
1999
39
Pathologic quiz case. Extrarenal rhabdoid tumor. (10458840)
1999
40
Rhabdoid tumor of the kidney associated with a tumor of the posterior fossa. (9477277)
1998
41
Congenital malignant rhabdoid tumor presenting as a cutaneous nodule: report of 2 cases with review of the literature. (9870859)
1998
42
Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. (9206002)
1997
43
Narrowing the critical region for a rhabdoid tumor locus in 22q11. (8818656)
1996
44
Carcinosarcoma (malignant mixed mA1llerian tumor) of the uterus with a rhabdoid tumor component. An immunohistochemical, ultrastructural, and immunoelectron microscopic case study. (7856569)
1995
45
Malignant rhabdoid tumor of the brain and kidney in a child: clinical and pathologic features. (7575853)
1995
46
Successful treatment of disseminated central nervous system malignant rhabdoid tumor. (7743242)
1995
47
Primary malignant rhabdoid tumor of the brain: clinical, imaging, and pathologic findings. (8427070)
1993
48
Clinical and imaging features of rhabdoid tumor of the kidney. (1897075)
1991
49
Poorly differentiated primary orbital sarcoma (presumed malignant rhabdoid tumor). Radiologic and histopathologic correlation. (1656924)
1991
50
Malignant rhabdoid tumor of the vulva: is distinction from epithelioid sarcoma possible? A pathologic and immunohistochemical study. (2476944)
1989

Variations for Rhabdoid Tumor

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Expression for genes affiliated with Rhabdoid Tumor

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Rhabdoid Tumor

Search GEO for disease gene expression data for Rhabdoid Tumor.

Pathways for genes affiliated with Rhabdoid Tumor

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51PathCards, 54QIAGEN, 61Thomson Reuters, 39NCBI BioSystems Database, 56Reactome, 5Cell Signaling Technology
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Pathways related to Rhabdoid Tumor according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.8SMARCA4, SMARCB1
2
Show member pathways
Development Ligand dependent activation of the ESR1 AP 1 pathway61
9.8SMARCA4, SMARCB1
39.8SMARCB1, SMARCA4
49.6ACTC1, IGF2
5
Show member pathways
Signaling by Aurora kinases39
Aurora C signaling39
9.4DES, VIM
69.1IGF2, SPP1
7
Show member pathways
9.1ACTC1, VIM, DES
89.1ACTC1, VIM, DES
9
Show member pathways
7.6SPP1, DES, IGF2, VIM, MUC1

Compounds for genes affiliated with Rhabdoid Tumor

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46Novoseek, 3BitterDB, 52PharmGKB, 12DrugBank, 25HMDB, 30IUPHAR, 62Tocris Bioscience
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Compounds related to Rhabdoid Tumor according to GeneCards/GeneDecks:

(show top 50)    (show all 90)
idCompoundScoreTop Affiliating Genes
1eosin469.5MUC1, VIM
2orcein469.5ACTC1, VIM, DES
32-methoxyethanol469.3IGF2, VIM, ACTC1
4ganglioside469.3SYP, VIM, DES
5peg-10469.2IGF2, SPP1
6dbc-amp469.1SYP, VIM, IGF2
7azathioprine46 3 52 1212.1DES, IGF2, VIM
8biotin46 25 1210.9ACTC1, MUC1, VIM, DES
9polyethylene glycol468.8MUC1, IGF2, SPP1
10progestin468.8MUC1, VIM, IGF2, DES
11neomycin468.8SPP1, IGF2, VIM
12dihydrotestosterone46 30 25 1211.8DES, IGF2, VIM, MUC1
13hydrocortisone46 3 62 1211.7IGF2, VIM, MUC1
14steroid468.7SYP, ACTC1, SMARCA4, IGF2, DES
15procollagen468.6SPP1, DES, VIM, ACTC1
16adriamycin468.6DES, VIM, MUC1
17forskolin46 52 1210.6IGF2, VIM, MUC1, SYP
18ribonucleic acid468.6ACTC1, MUC1, IGF2, SPP1
19s 100468.5SYP, ACTC1, MUC1, VIM, DES
20reticulin468.5DES, VIM, MUC1, ACTC1, SYP
21mucicarmine468.5DES, VIM, MUC1, ACTC1, SYP
22b 723468.5DES, VIM, MUC1, ACTC1, SYP
23hematoxylin468.5DES, VIM, MUC1, ACTC1, SYP
24formaldehyde46 259.5DES, VIM, MUC1, ACTC1, SYP
25cyclic amp46 259.5DES, SMARCA4, VIM, MUC1, SYP
26matrigel468.5SPP1, VIM, MUC1, ACTC1
27hyaluronic acid46 259.5MUC1, VIM, DES, SPP1
28estradiol46 25 1210.3DES, IGF2, VIM, MUC1, SYP
29butyrate468.3SPP1, IGF2, VIM, MUC1
30lactate468.3VIM, IGF2, DES, SPP1
31cisplatin46 52 62 1211.3SPP1, IGF2, MUC1, SYP
32heparin46 30 25 1211.1SPP1, DES, IGF2, VIM
33arginine468.1SPP1, DES, IGF2, SMARCA4, VIM
34agar468.0SPP1, IGF2, SMARCA4, VIM, MUC1
35glycogen46 259.0DES, IGF2, VIM, MUC1, ACTC1, SYP
36h2o2468.0SPP1, DES, IGF2, VIM, SYP
37actinomycin d468.0MUC1, VIM, IGF2, DES, SPP1
38glucose467.8SPP1, DES, IGF2, SMARCA4, MUC1, ACTC1
39lipid467.7SYP, MUC1, VIM, SMARCA4, SPP1
40dexamethasone46 52 30 1210.6SPP1, IGF2, VIM, MUC1, ACTC1, SYP
41serine467.4SPP1, DES, IGF2, VIM, MUC1, SYP
42tyrosine467.4SPP1, DES, IGF2, SMARCA4, MUC1, ACTC1
43cysteine467.2SPP1, DES, IGF2, SMARCA4, VIM, MUC1
44paraffin467.2SPP1, DES, IGF2, VIM, MUC1, ACTC1
45creatinine467.2SPP1, DES, IGF2, VIM, MUC1, ACTC1
46vegf467.2SPP1, DES, IGF2, VIM, MUC1, ACTC1
47retinoic acid46 258.2SPP1, DES, IGF2, VIM, MUC1, ACTC1
48progesterone46 30 62 25 1210.8SPP1, DES, IGF2, SMARCA4, VIM, MUC1
49testosterone46 62 25 129.8SPP1, DES, IGF2, SMARCA4, VIM, MUC1
50estrogen466.8SPP1, DES, IGF2, SMARCA4, VIM, MUC1

GO Terms for genes affiliated with Rhabdoid Tumor

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17Gene Ontology
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Cellular components related to Rhabdoid Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1npBAF complexGO:0715649.9SMARCA4, SMARCB1
2nBAF complexGO:0715659.8SMARCA4, SMARCB1
3SWI/SNF complexGO:0165149.7SMARCA4, SMARCB1
4cell projectionGO:0429959.0VIM, SPP1
5extracellular spaceGO:0056158.1SPP1, IGF2, SMARCA4, ACTC1

Biological processes related to Rhabdoid Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1blastocyst hatchingGO:0018359.9SMARCA4, SMARCB1
2positive regulation by host of viral transcriptionGO:0439239.8SMARCB1, SMARCA4
3chromatin remodelingGO:0063389.8SMARCA4, SMARCB1
4nucleosome disassemblyGO:0063379.7SMARCA4, SMARCB1
5muscle filament slidingGO:0300499.2ACTC1, VIM, DES
6osteoblast differentiationGO:0016498.8IGF2, SPP1

Molecular functions related to Rhabdoid Tumor according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1Tat protein bindingGO:0309579.8SMARCA4, SMARCB1
2p53 bindingGO:0020399.0MUC1, SMARCA4, SMARCB1

Products for genes affiliated with Rhabdoid Tumor

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Sources for Rhabdoid Tumor

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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