MCID: ATY005
MIFTS: 57

Atypical Teratoid Rhabdoid Tumor

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Atypical Teratoid Rhabdoid Tumor

MalaCards integrated aliases for Atypical Teratoid Rhabdoid Tumor:

Name: Atypical Teratoid Rhabdoid Tumor 12 14
Rhabdoid Tumor Predisposition Syndrome 12 23 69
Atypical Teratoid/rhabdoid Tumor 12 69
Rhabdoid Predisposition Syndrome 23 36
Rhabdoid Tumor of the Cns 12
at/rt 12
Rtps 23

Characteristics:

GeneReviews:

23
Penetrance Smarca4. most individuals with smarca4-related rtps have inherited the pathogenic variant from an unaffected, healthy parent. in smarca4-related rtps the penetrance for rhabdoid tumor in the preceding generation of seven informative families was zero. however, in one family, two sibs with a smarca4 pathogenic variant were both affected [schneppenheim et al 2010, hasselblatt et al 2014]...

Classifications:



Summaries for Atypical Teratoid Rhabdoid Tumor

Disease Ontology : 12 A brain cancer that is usually located in the brain, but can occur anywhere in the central nervous system.

MalaCards based summary : Atypical Teratoid Rhabdoid Tumor, also known as rhabdoid tumor predisposition syndrome, is related to rhabdoid cancer and familial rhabdoid tumor, and has symptoms including hydrocephalus, macrocephaly and irritability. An important gene associated with Atypical Teratoid Rhabdoid Tumor is SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1), and among its related pathways/superpathways are PI3K-Akt signaling pathway and Focal Adhesion. The drugs Carboplatin and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include brain, kidney and spinal cord, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

GeneReviews: NBK469816

Related Diseases for Atypical Teratoid Rhabdoid Tumor

Diseases related to Atypical Teratoid Rhabdoid Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 121)
# Related Disease Score Top Affiliating Genes
1 rhabdoid cancer 32.3 IGF1R SMARCA4 SMARCB1 SPP1 SYP
2 familial rhabdoid tumor 32.1 SMARCA4 SMARCB1
3 medulloblastoma 31.4 CTNNB1 GFAP IGF1R SMARCA4 SMARCB1 SYP
4 pineal gland cancer 31.0 GFAP SYP
5 ganglioglioma 30.8 GFAP SMARCB1 SYP
6 choroid plexus cancer 30.7 GFAP SMARCB1
7 pleomorphic xanthoastrocytoma 30.7 GFAP SMARCB1 SYP
8 dysembryoplastic neuroepithelial tumor 30.6 GFAP SYP
9 tumor predisposition syndrome 30.5 SMARCA4 SMARCB1
10 meningioma, familial 30.3 GFAP SMARCB1 SYP
11 nodular medulloblastoma 30.1 CTNNB1 GFAP SMARCB1
12 ewing sarcoma 29.9 CCND1 GFAP IGF1R SYP
13 ependymoma 29.8 GFAP SYP
14 rhabdoid tumor predisposition syndrome 1 12.0
15 rhabdoid tumor predisposition syndrome 2 11.4
16 melanotic medulloblastoma 10.5 GFAP SMARCB1
17 extraosseous chondrosarcoma 10.5 SMARCB1 SYP
18 desmoplastic infantile ganglioglioma 10.5 GFAP SYP
19 extraventricular neurocytoma 10.4 GFAP SYP
20 lung combined type small cell carcinoma 10.4 GFAP SYP
21 cellular ependymoma 10.4 GFAP SYP
22 gangliocytoma 10.4 GFAP SYP
23 central nervous system sarcoma 10.4 SMARCA4 SMARCB1
24 pilomyxoid astrocytoma 10.4 GFAP SYP
25 chordoid meningioma 10.4 GFAP SYP
26 central nervous system primitive neuroectodermal neoplasm 10.4 GFAP SYP
27 subependymal glioma 10.4 GFAP SYP
28 chiari malformation 10.4 GFAP SYP
29 cerebral neuroblastoma 10.4 GFAP SYP
30 benign ependymoma 10.4 GFAP SYP
31 astroblastoma 10.4 GFAP SYP
32 melanotic neuroectodermal tumor 10.4 GFAP SYP
33 ovarian small cell carcinoma 10.4 SMARCA4 SYP
34 anaplastic ependymoma 10.4 GFAP SYP
35 subependymoma 10.4 GFAP SYP
36 angiocentric glioma 10.4 GFAP SYP
37 obstructive hydrocephalus 10.4 GFAP SYP
38 medulloepithelioma 10.4 GFAP SYP
39 pineocytoma 10.4 GFAP SYP
40 pineoblastoma 10.4 GFAP SYP
41 childhood oligodendroglioma 10.4 GFAP SYP
42 papillary ependymoma 10.4 GFAP SYP
43 papilloma of choroid plexus 10.4 GFAP SYP
44 subependymal giant cell astrocytoma 10.3 GFAP SYP
45 central neurocytoma 10.3 GFAP SYP
46 binswanger's disease 10.3 GFAP SYP
47 cystic teratoma 10.3 GFAP SYP
48 choroiditis 10.3
49 cervicitis 10.3
50 cerebritis 10.3

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



Diseases related to Atypical Teratoid Rhabdoid Tumor

Symptoms & Phenotypes for Atypical Teratoid Rhabdoid Tumor

Human phenotypes related to Atypical Teratoid Rhabdoid Tumor:

31 (show all 16)
# Description HPO Frequency HPO Source Accession
1 hydrocephalus 31 frequent (33%) HP:0000238
2 macrocephaly 31 frequent (33%) HP:0000256
3 irritability 31 hallmark (90%) HP:0000737
4 apathy 31 hallmark (90%) HP:0000741
5 seizures 31 frequent (33%) HP:0001250
6 ataxia 31 frequent (33%) HP:0001251
7 muscle weakness 31 frequent (33%) HP:0001324
8 limitation of joint mobility 31 frequent (33%) HP:0001376
9 nausea and vomiting 31 hallmark (90%) HP:0002017
10 migraine 31 frequent (33%) HP:0002076
11 cerebral calcification 31 occasional (7.5%) HP:0002514
12 reduced consciousness/confusion 31 frequent (33%) HP:0004372
13 hemiplegia/hemiparesis 31 frequent (33%) HP:0004374
14 cranial nerve paralysis 31 occasional (7.5%) HP:0006824
15 cerebral palsy 31 occasional (7.5%) HP:0100021
16 malignant neoplasm of the central nervous system 31 hallmark (90%) HP:0100836

GenomeRNAi Phenotypes related to Atypical Teratoid Rhabdoid Tumor according to GeneCards Suite gene sharing:

25 (show all 28)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-102 9.88 SMARCA4
2 Increased shRNA abundance (Z-score > 2) GR00366-A-11 9.88 SMARCA4 CTNNB1
3 Increased shRNA abundance (Z-score > 2) GR00366-A-113 9.88 CTNNB1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-114 9.88 IGF1R
5 Increased shRNA abundance (Z-score > 2) GR00366-A-126 9.88 CCND1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-127 9.88 IGF1R
7 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.88 CCND1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-16 9.88 CCND1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-163 9.88 CCND1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.88 IGF1R
11 Increased shRNA abundance (Z-score > 2) GR00366-A-172 9.88 SMARCA4
12 Increased shRNA abundance (Z-score > 2) GR00366-A-177 9.88 CCND1 SMARCA4 CTNNB1 IGF1R
13 Increased shRNA abundance (Z-score > 2) GR00366-A-197 9.88 CTNNB1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-199 9.88 IGF1R CCND1
15 Increased shRNA abundance (Z-score > 2) GR00366-A-21 9.88 CCND1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-210 9.88 CCND1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.88 IGF1R
18 Increased shRNA abundance (Z-score > 2) GR00366-A-25 9.88 CCND1
19 Increased shRNA abundance (Z-score > 2) GR00366-A-36 9.88 CCND1
20 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.88 IGF1R
21 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.88 IGF1R CTNNB1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-49 9.88 CTNNB1
23 Increased shRNA abundance (Z-score > 2) GR00366-A-67 9.88 CTNNB1
24 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.88 CTNNB1
25 Increased shRNA abundance (Z-score > 2) GR00366-A-74 9.88 IGF1R CTNNB1
26 Increased shRNA abundance (Z-score > 2) GR00366-A-79 9.88 CTNNB1
27 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.88 SMARCA4 IGF1R
28 Increased shRNA abundance (Z-score > 2) GR00366-A-93 9.88 SMARCA4

MGI Mouse Phenotypes related to Atypical Teratoid Rhabdoid Tumor:

43 (show all 14)
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.11 SMARCA4 SMARCB1 SPP1 AURKA CCND1 CTNNB1
2 behavior/neurological MP:0005386 10.08 CCND1 CTNNB1 GFAP IGF1R SMARCB1 SPP1
3 cardiovascular system MP:0005385 10.07 CCND1 CTNNB1 GFAP IGF1R SMARCA4 SMARCB1
4 growth/size/body region MP:0005378 10.06 AURKA CCND1 CTNNB1 GFAP IGF1R SMARCA4
5 hematopoietic system MP:0005397 9.98 SMARCA4 SMARCB1 SPP1 CCND1 CTNNB1 IGF1R
6 digestive/alimentary MP:0005381 9.95 CCND1 CTNNB1 GFAP IGF1R SMARCA4 SMARCB1
7 immune system MP:0005387 9.95 SMARCB1 SPP1 CCND1 CTNNB1 GFAP IGF1R
8 mortality/aging MP:0010768 9.92 AURKA CCND1 CTNNB1 GFAP IGF1R SMARCA4
9 craniofacial MP:0005382 9.89 SPP1 CCND1 CTNNB1 IGF1R SMARCA4
10 liver/biliary system MP:0005370 9.77 CTNNB1 IGF1R SMARCA4 SMARCB1 SPP1
11 muscle MP:0005369 9.73 CTNNB1 GFAP IGF1R SMARCA4 SMARCB1 SPP1
12 neoplasm MP:0002006 9.7 AURKA CCND1 CTNNB1 IGF1R SMARCA4 SMARCB1
13 nervous system MP:0003631 9.56 CCND1 CTNNB1 GFAP IGF1R SMARCA4 SMARCB1
14 respiratory system MP:0005388 9.02 CCND1 CTNNB1 IGF1R SMARCA4 SPP1

Drugs & Therapeutics for Atypical Teratoid Rhabdoid Tumor

Drugs for Atypical Teratoid Rhabdoid Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 154)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Carboplatin Approved Phase 2, Phase 3, Phase 1 41575-94-4 10339178 38904 498142
2
Cyclophosphamide Approved, Investigational Phase 2, Phase 3, Phase 1 50-18-0, 6055-19-2 2907
3
Etoposide Approved Phase 2, Phase 3, Phase 1, Early Phase 1 33419-42-0 36462
4
Melphalan Approved Phase 2, Phase 3,Phase 1 148-82-3 460612 4053
5 Thiotepa Approved, Investigational Phase 2, Phase 3, Phase 1 52-24-4 5453
6
Cisplatin Approved Phase 3,Phase 2,Phase 1 15663-27-1 2767 441203 84093
7
Lenograstim Approved, Investigational Phase 3,Phase 2,Phase 1 135968-09-1
8
Methotrexate Approved Phase 3,Phase 2,Phase 1,Early Phase 1 1959-05-2, 59-05-2 126941
9
Temozolomide Approved, Investigational Phase 3,Phase 2,Phase 1 85622-93-1 5394
10
Vincristine Approved, Investigational Phase 3,Phase 2,Phase 1 2068-78-2, 57-22-7 5978
11
Dactinomycin Approved, Investigational Phase 3,Phase 2 50-76-0 457193 2019
12
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
13
Sulfamethoxazole Approved Phase 3 723-46-6 5329
14
Trimethoprim Approved, Vet_approved Phase 3 738-70-5 5578
15
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
16
Levoleucovorin Approved, Investigational Phase 3,Phase 2,Early Phase 1 68538-85-2
17
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Phase 2,Phase 1,Early Phase 1 59-30-3 6037
18
leucovorin Approved, Nutraceutical Phase 3,Phase 2,Phase 1,Early Phase 1 58-05-9 143 6006
19
Doxil Approved June 1999 Phase 3,Phase 2 31703
20 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
21 Etoposide phosphate Phase 2, Phase 3, Phase 1, Early Phase 1
22 Alkylating Agents Phase 3,Phase 2,Phase 1
23 Antimetabolites Phase 3,Phase 2,Phase 1,Early Phase 1
24 Antimetabolites, Antineoplastic Phase 3,Phase 2,Phase 1,Early Phase 1
25 Antimitotic Agents Phase 3,Phase 2,Phase 1
26 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Phase 1,Early Phase 1
27 Antirheumatic Agents Phase 3,Phase 2,Phase 1,Early Phase 1
28 Dermatologic Agents Phase 3,Phase 2,Phase 1,Early Phase 1
29 Folic Acid Antagonists Phase 3,Phase 2,Phase 1,Early Phase 1
30 Immunosuppressive Agents Phase 3,Phase 2,Phase 1,Early Phase 1
31 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2,Phase 1,Early Phase 1
32 Topoisomerase Inhibitors Phase 3,Phase 2,Phase 1,Early Phase 1
33 Vitamin B Complex Phase 3,Phase 2,Phase 1,Early Phase 1
34 Adjuvants, Immunologic Phase 3,Phase 2,Phase 1
35 Liver Extracts Phase 3,Phase 1,Phase 2
36 Protective Agents Phase 3,Phase 2,Phase 1
37 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
38 Antibiotics, Antitubercular Phase 3,Phase 2,Phase 1
39 Anti-Infective Agents Phase 3,Phase 2,Phase 1
40 Adjuvants, Anesthesia Phase 3
41 Analgesics Phase 3
42 Analgesics, Opioid Phase 3
43 Anesthetics Phase 3
44 Anesthetics, General Phase 3
45 Anesthetics, Intravenous Phase 3
46 Central Nervous System Depressants Phase 3
47 Narcotics Phase 3
48 Peripheral Nervous System Agents Phase 3,Phase 1
49 Antidotes Phase 3,Phase 2,Phase 1
50 Calcium, Dietary Phase 3,Phase 2

Interventional clinical trials:

(show top 50) (show all 85)

# Name Status NCT ID Phase Drugs
1 Stem Cell Transplant for High Risk Central Nervous System (CNS) Tumors Unknown status NCT00179803 Phase 2, Phase 3
2 Combination Chemotherapy With or Without Etoposide Followed By an Autologous Stem Cell Transplant in Treating Young Patients With Previously Untreated Malignant Brain Tumors Unknown status NCT00392886 Phase 3 carboplatin;cisplatin;cyclophosphamide;etoposide;methotrexate;temozolomide;thiotepa;vincristine sulfate
3 Chemotherapy With or Without Surgery, Radiation Therapy, or Stem Cell Transplantation in Treating Young Patients With Kidney Tumors Completed NCT00002610 Phase 3 carboplatin;cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
4 Combination Chemotherapy Followed by Second-Look Surgery and Radiation Therapy in Treating Children With Nonmetastatic Medulloblastoma or Primitive Neuroectodermal Tumor Completed NCT00006461 Phase 3 cisplatin;cyclophosphamide;vincristine sulfate;etoposide
5 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
6 Combination Chemotherapy Alone or With Radiation Therapy in Treating Children With Kidney Cancer Completed NCT00002611 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
7 Treatment of Patients With Newly Diagnosed Medulloblastoma, Supratentorial Primitive Neuroectodermal Tumor, or Atypical Teratoid Rhabdoid Tumor Active, not recruiting NCT00085202 Phase 3 cisplatin;cyclophosphamide;vincristine
8 Combination Chemotherapy, Radiation Therapy, and an Autologous Peripheral Blood Stem Cell Transplant in Treating Young Patients With Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System Active, not recruiting NCT00653068 Phase 3 methotrexate;leucovorin calcium;etoposide;cyclophosphamide;cisplatin;carboplatin;thiotepa;vincristine sulfate
9 Oxaliplatin in Treating Children With Recurrent or Refractory Medulloblastoma, Supratentorial Primitive Neuroectodermal Tumor, or Atypical Teratoid Rhabdoid Tumor Completed NCT00047177 Phase 2 Oxaliplatin
10 Chemotherapy Combined With Radiation Therapy for Newly Diagnosed CNS AT/RT Completed NCT00084838 Phase 2 cisplatin;cyclophosphamide;cytarabine;dexrazoxane hydrochloride;doxorubicin hydrochloride;etoposide;leucovorin calcium;methotrexate;temozolomide;therapeutic hydrocortisone;vincristine sulfate;Dactinomycin
11 Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors Completed NCT00335556 Phase 2 Doxorubicin Hydrochloride;Irinotecan Hydrochloride;Cyclophosphamide;Etoposide;Carboplatin;Vincristine Sulfate
12 Chemotherapy in Treating Patients With Solid Tumors Completed NCT00003103 Phase 1, Phase 2 docetaxel
13 Phase 2 Study of Alisertib Therapy for Rhabdoid Tumors Recruiting NCT02114229 Phase 2 alisertib;methotrexate;cisplatin;carboplatin;cyclophosphamide;etoposide;topotecan;vincristine
14 A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma Recruiting NCT02601950 Phase 2 Tazemetostat
15 Phase II Pediatric Study With Dabrafenib in Combination With Trametinib in Patients With HGG Recruiting NCT02684058 Phase 2 dabrafenib;trametinib
16 Stereotactic Body Radiotherapy (SBRT) for Pulmonary Metastases in Ewing Sarcoma, Rhabdomyosarcoma, and Wilms Tumors Recruiting NCT02581384 Phase 1, Phase 2
17 Tazemetostat Rollover Study (TRuST): An Open-Label Rollover Study Recruiting NCT02875548 Phase 2 Tazemetostat
18 Pediatric MATCH: Tazemetostat in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With EZH2, SMARCB1, or SMARCA4 Gene Mutations Recruiting NCT03213665 Phase 2 Tazemetostat
19 Phase II Study of Intraventricular Methotrexate in Children With Recurrent or Progressive Malignant Brain Tumors Recruiting NCT02684071 Phase 2 Intra thecal methotrexate;topotecan;cyclophosphamide
20 Melphalan, Carboplatin, Mannitol, and Sodium Thiosulfate in Treating Patients With Recurrent or Progressive CNS Embryonal or Germ Cell Tumors Recruiting NCT00983398 Phase 1, Phase 2 Carboplatin;Mannitol;Melphalan;Sodium Thiosulfate
21 Pediatric MATCH: Olaparib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Defects in DNA Damage Repair Genes Recruiting NCT03233204 Phase 2 Olaparib
22 Pediatric MATCH: Vemurafenib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With BRAF V600 Mutations Recruiting NCT03220035 Phase 2 Vemurafenib
23 Pediatric MATCH: Trk Inhibitor LOXO-101 in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With NTRK Fusions Recruiting NCT03213704 Phase 2 Larotrectinib
24 Pediatric MATCH: PI3K/mTOR Inhibitor LY3023414 in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With TSC or PI3K/MTOR Mutations Recruiting NCT03213678 Phase 2 PI3K/mTOR Inhibitor LY3023414
25 Pediatric MATCH: Erdafitinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With FGFR Mutations Recruiting NCT03210714 Phase 2 Erdafitinib
26 Pediatric MATCH: Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders Recruiting NCT03155620 Phase 2 Ensartinib;Erdafitinib;Larotrectinib;Olaparib;PI3K/mTOR Inhibitor LY3023414;Selumetinib Sulfate;Tazemetostat;Vemurafenib
27 Dasatinib, Ifosfamide, Carboplatin, and Etoposide in Treating Young Patients With Metastatic or Recurrent Malignant Solid Tumors Active, not recruiting NCT00788125 Phase 1, Phase 2 carboplatin;dasatinib;etoposide phosphate;ifosfamide
28 A Study to Evaluate the Safety, Tolerability, Pharmacokinetics, Immunogenicity, and Preliminary Efficacy of Atezolizumab (Anti-Programmed Death-Ligand 1 [PD-L1] Antibody) in Pediatric and Young Adult Participants With Solid Tumors Active, not recruiting NCT02541604 Phase 1, Phase 2 Atezolizumab
29 Risk-Adapted Therapy for Young Children With Embryonal Brain Tumors, Choroid Plexus Carcinoma, High Grade Glioma or Ependymoma Active, not recruiting NCT00602667 Phase 2 Induction Chemotherapy;Low-Risk Therapy;High-Risk Therapy;Intermediate-Risk Therapy
30 Crizotinib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Anaplastic Large Cell Lymphoma Active, not recruiting NCT00939770 Phase 1, Phase 2 crizotinib
31 Iodine I 131 Monoclonal Antibody 3F8 in Treating Patients With Central Nervous System Cancer or Leptomeningeal Cancer Active, not recruiting NCT00445965 Phase 2
32 Antineoplaston Therapy in Treating Children With Rhabdoid Tumor of the Central Nervous System Terminated NCT00003469 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
33 Peripheral Stem Cell Transplantation Plus Chemotherapy in Treating Patients With Malignant Solid Tumors Unknown status NCT00007813 Phase 1 carboplatin;cyclophosphamide;etoposide
34 Study of Safety and Efficacy in Patients With Malignant Rhabdoid Tumors (MRT) and Neuroblastoma Completed NCT01747876 Phase 1 LEE011
35 Vorinostat and Temozolomide in Treating Young Patients With Relapsed or Refractory Primary Brain Tumors or Spinal Cord Tumors Completed NCT01076530 Phase 1 vorinostat;temozolomide
36 ABT-888 and Temozolomide in Treating Young Patients With Recurrent or Refractory CNS Tumors Completed NCT00946335 Phase 1 veliparib;temozolomide
37 AZD2171 in Treating Young Patients With Recurrent, Progressive, or Refractory Primary CNS Tumors Completed NCT00326664 Phase 1 Cediranib Maleate
38 Lenalidomide in Treating Young Patients With Recurrent, Progressive, or Refractory CNS Tumors Completed NCT00100880 Phase 1 lenalidomide
39 Vorinostat With or Without Isotretinoin in Treating Young Patients With Recurrent or Refractory Solid Tumors, Lymphoma, or Leukemia Completed NCT00217412 Phase 1 vorinostat;isotretinoin
40 Thalidomide and Docetaxel in Treating Patients With Advanced Cancer Completed NCT00049296 Phase 1 docetaxel;thalidomide
41 Talabostat Combined With Temozolomide or Carboplatin in Treating Young Patients With Relapsed or Refractory Brain Tumors or Other Solid Tumors Completed NCT00303940 Phase 1 carboplatin;talabostat mesylate;temozolomide
42 Temozolomide, Vincristine, and Irinotecan in Treating Young Patients With Refractory Solid Tumors Completed NCT00138216 Phase 1 irinotecan hydrochloride;temozolomide;vincristine sulfate
43 Chemotherapy Plus Peripheral Stem Cell Transplantation in Treating Infants With Malignant Brain or Spinal Cord Tumors Completed NCT00003141 Phase 1 carboplatin;cisplatin;cyclophosphamide;etoposide;thiotepa;vincristine sulfate
44 Chemotherapy and Stem Cell Transplantation in Treating Children With Central Nervous System Cancer Completed NCT00053118 Phase 1 carboplatin;etoposide
45 SCH 66336 in Treating Children With Recurrent or Progressive Brain Tumors Completed NCT00015899 Phase 1 lonafarnib
46 p28 in Treating Younger Patients With Recurrent or Progressive Central Nervous System Tumors Completed NCT01975116 Phase 1 azurin-derived cell-penetrating peptide p28
47 Modified Measles Virus (MV-NIS) for Children and Young Adults With Recurrent Medulloblastoma or Recurrent ATRT Recruiting NCT02962167 Phase 1
48 A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma Recruiting NCT02601937 Phase 1 Tazemetostat
49 Methotrexate Infusion Into Fourth Ventricle in Children With Recurrent Malignant Fourth Ventricular Brain Tumors Recruiting NCT02458339 Phase 1 Methotrexate
50 Molecularly-Driven Doublet Therapy for Recurrent CNS Malignant Neoplasms Recruiting NCT03434262 Phase 1 Gemcitabine;ribociclib;sonidegib;trametinib

Search NIH Clinical Center for Atypical Teratoid Rhabdoid Tumor

Genetic Tests for Atypical Teratoid Rhabdoid Tumor

Anatomical Context for Atypical Teratoid Rhabdoid Tumor

MalaCards organs/tissues related to Atypical Teratoid Rhabdoid Tumor:

38
Brain, Kidney, Spinal Cord, Myeloid, T Cells, Testes, Liver

Publications for Atypical Teratoid Rhabdoid Tumor

Articles related to Atypical Teratoid Rhabdoid Tumor:

(show top 50) (show all 253)
# Title Authors Year
1
Atypical Teratoid Rhabdoid Tumor of the Cauda Equina in a Child: Report of a Very Unusual Case. ( 29346182 )
2018
2
Atypical teratoid/rhabdoid tumor of the sellar region in adult women: Is it a sex-related disease? ( 29248378 )
2018
3
Atypical teratoid/rhabdoid tumor after in-vitro fertilization: illustrative case report and systematic literature review. ( 29452330 )
2018
4
Dumbbell-shaped atypical teratoid rhabdoid tumor in the cervical spine mimicking schwannoma. ( 29018929 )
2018
5
Primary diffuse leptomeningeal atypical teratoid/rhabdoid tumor diagnosed by cerebrospinal fluid cytology: Case report with molecular genetic analysis. ( 29339179 )
2018
6
Atypical Teratoid/ Rhabdoid Tumor of Brain: a Clinicopathologic Study of Eleven Patients and Review of Literature ( 28545192 )
2017
7
Cytologic diagnosis of atypical teratoid rhabdoid tumor based on touch imprint study: Report of a case with review of literature. ( 28616426 )
2017
8
Atypical teratoid/rhabdoid tumor of lumbar spine in a toddler child. ( 28503314 )
2017
9
Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology and Treatment. ( 28676785 )
2017
10
LIN28A, a sensitive immunohistochemical marker for Embryonal Tumor with Multilayered Rosettes (ETMR), is also positive in a subset of Atypical Teratoid/Rhabdoid Tumor (AT/RT). ( 28744687 )
2017
11
Loss of CDKN1C in a Recurrent Atypical Teratoid/Rhabdoid Tumor. ( 28731921 )
2017
12
Repositioning disulfiram as a radiosensitizer against atypical teratoid/rhabdoid tumor. ( 28340172 )
2017
13
Correction: Frequent Overexpression of HMGA2 in Human Atypical Teratoid/Rhabdoid Tumor and Its Correlation with let-7a3/let-7b miRNA. ( 28620002 )
2017
14
Multimodal treatments combined with gamma knife surgery for primary atypical teratoid/rhabdoid tumor of the central nervous system: a single-institute experience of 18 patients. ( 29218405 )
2017
15
Multifocal atypical teratoid rhabdoid tumor of the central nervous system in an adult patient. ( 29150125 )
2017
16
Non-Malignant Cerebrospinal Fluid Ascites in a Patient with Atypical Teratoid Rhabdoid Tumor. ( 28365696 )
2017
17
Atypical teratoid rhabdoid tumor of the central nervous system: Case series from a regional Tertiary Care Cancer Centre in South India. ( 29237968 )
2017
18
Targeting Polo-like kinase 1 in SMARCB1 deleted atypical teratoid rhabdoid tumor. ( 29228610 )
2017
19
Pituitary atypical teratoid rhabdoid tumor in a patient with prolactinoma: A unique description. ( 29110337 )
2017
20
Atypical teratoid rhabdoid tumor arising in a pleomorphic xanthoastrocytoma: a rare entity. ( 28502320 )
2017
21
Ewing Sarcoma and Atypical Teratoid Rhabdoid Tumor. ( 28382842 )
2017
22
Synchronous Central Nervous System Atypical Teratoid/ Rhabdoid Tumor and Malignant Rhabdoid Tumor of the Kidney: Case report of a Long-Term Survivor and Review of the Literature. ( 29223518 )
2017
23
Development of an Atypical Teratoid Rhabdoid Tumor in a Meningioma. ( 28459167 )
2017
24
Sustained Complete Response to Metronomic Chemotherapy in a Child with Refractory Atypical Teratoid Rhabdoid Tumor: A Case Report. ( 29163174 )
2017
25
A Diagnostic Pitfall: Atypical Teratoid Rhabdoid Tumor Versus Dedifferentiated/Poorly Differentiated Chordoma: Analysis of a Mono-institutional Series. ( 28777153 )
2017
26
Description of a new oncogenic mechanism for atypical teratoid rhabdoid tumors in patients with ring chromosome 22. ( 27734605 )
2017
27
Molecular Transition of an Adult Low-Grade Brain Tumor to an Atypical Teratoid/Rhabdoid Tumor Over a Time-Course of 14 Years. ( 28789476 )
2017
28
Suboccipital Extraspinal Extracranial Atypical Teratoid Rhabdoid Tumor. ( 28694650 )
2017
29
Atypical teratoid/rhabdoid tumor with retained INI1 (SMARCB1) expression and loss of BRG1 (SMARCA4). ( 29271065 )
2017
30
OTX2 Defines a Subgroup of Atypical Teratoid Rhabdoid Tumors With Close Relationship to Choroid Plexus Tumors. ( 28025236 )
2017
31
Primary atypical teratoid rhabdoid tumor in the adult spine. ( 28458948 )
2017
32
Suprasellar atypical teratoid/rhabdoid tumor. ( 28454722 )
2017
33
Sellar Atypical Teratoid/Rhabdoid Tumor (AT/RT): A Clinicopathologically and Genetically Distinct Variant of AT/RT. ( 28338502 )
2017
34
Long-term survival of an infant with an atypical teratoid/rhabdoid tumor following subtotal resection and low-cumulative dose chemotherapy: a case report. ( 26747622 )
2016
35
Pituitary apoplexy as presentation of atypical teratoid/rhabdoid tumor in an adult. ( 27349334 )
2016
36
A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child. ( 27606020 )
2016
37
Adult-onset atypical teratoid/rhabdoid tumor featuring long spindle cells with nuclear palisading and perivascular pseudorosettes. ( 27297614 )
2016
38
Atypical teratoid/rhabdoid tumor arising from the trigeminal nerve in an adult. ( 27335108 )
2016
39
Multimodal therapy in children and adolescents with newly diagnosed atypical teratoid rhabdoid tumor: individual pooled data analysis and review of the literature. ( 26608522 )
2016
40
Multifocal atypical teratoid/rhabdoid tumor: a rare entity. ( 27757569 )
2016
41
The Expression of Cyclin D1, VEGF, EZH2, and H3K27me3 in Atypical Teratoid/Rhabdoid Tumors of the CNS: A Possible Role in Targeted Therapy. ( 26469332 )
2016
42
Overexpression of TEAD4 in atypical teratoid/rhabdoid tumor: New insight to the pathophysiology of an aggressive brain tumor. ( 27966820 )
2016
43
Ewing Sarcoma and Atypical Teratoid Rhabdoid Tumor: A FISH and Immunohistochemical Comparison. ( 27467095 )
2016
44
Atypical teratoid/rhabdoid tumor arising in a malignant glioma. ( 27472468 )
2016
45
Primary atypical teratoid/rhabdoid tumor of the spine in an adult patient. ( 27069744 )
2016
46
Atypical Teratoid Rhabdoid Tumor Diagnosis after Partial Resection of Dysembryoplastic Neuroepithelial Tumor: Case Report and Review of the Literature. ( 26978682 )
2016
47
Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation for Atypical Teratoid/Rhabdoid Tumor. ( 27034140 )
2016
48
Atypical Teratoid/Rhabdoid Tumor of the Pineal Region in a Young Adult Male Patient: Case Report and Review of the Literature. ( 27144539 )
2016
49
Atypical teratoid/rhabdoid tumor in the sella turcica of an elderly female with a distinct vascular pattern and genetic alterations. ( 27595781 )
2016
50
Molecular analyses reveal close similarities between small cell carcinoma of the ovary, hypercalcemic type and atypical teratoid/rhabdoid tumor. ( 26646792 )
2016

Variations for Atypical Teratoid Rhabdoid Tumor

Expression for Atypical Teratoid Rhabdoid Tumor

Search GEO for disease gene expression data for Atypical Teratoid Rhabdoid Tumor.

Pathways for Atypical Teratoid Rhabdoid Tumor

Pathways related to Atypical Teratoid Rhabdoid Tumor according to GeneCards Suite gene sharing:

(show all 14)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.63 CCND1 CTNNB1 IGF1R SPP1
2
Show member pathways
12.52 CCND1 CTNNB1 IGF1R SPP1
3
Show member pathways
12.02 CCND1 CTNNB1 IGF1R SMARCA4 SMARCB1
4 11.96 CCND1 CTNNB1 IGF1R
5
Show member pathways
11.74 CCND1 CTNNB1 IGF1R
6 11.59 CCND1 CTNNB1 IGF1R
7 11.4 GFAP SMARCA4 SYP
8 11.33 CCND1 CTNNB1 SMARCA4
9
Show member pathways
11.18 CCND1 SMARCA4 SMARCB1
10 11.14 CTNNB1 IGF1R
11 11.12 CCND1 SPP1
12 11.11 AURKA CCND1 CTNNB1 SMARCA4
13 11.01 CCND1 CTNNB1
14 10.81 CCND1 CTNNB1

GO Terms for Atypical Teratoid Rhabdoid Tumor

Cellular components related to Atypical Teratoid Rhabdoid Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 SWI/SNF complex GO:0016514 9.16 SMARCA4 SMARCB1
2 nBAF complex GO:0071565 8.96 SMARCA4 SMARCB1
3 npBAF complex GO:0071564 8.62 SMARCA4 SMARCB1

Biological processes related to Atypical Teratoid Rhabdoid Tumor according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 cell cycle GO:0007049 9.83 AURKA CCND1 HMGA2 SMARCB1
2 positive regulation of transcription, DNA-templated GO:0045893 9.76 CTNNB1 HMGA2 SMARCA4 SPP1
3 epithelial to mesenchymal transition GO:0001837 9.54 CTNNB1 HMGA2
4 cellular response to organic substance GO:0071310 9.51 CCND1 SYP
5 response to steroid hormone GO:0048545 9.49 CCND1 SPP1
6 liver regeneration GO:0097421 9.48 AURKA CCND1
7 ATP-dependent chromatin remodeling GO:0043044 9.43 SMARCA4 SMARCB1
8 neuron projection extension GO:1990138 9.4 AURKA CTNNB1
9 nucleosome disassembly GO:0006337 9.37 SMARCA4 SMARCB1
10 anterior/posterior axis specification GO:0009948 9.32 AURKA CTNNB1
11 positive regulation by host of viral transcription GO:0043923 9.26 SMARCA4 SMARCB1
12 positive regulation of transcription of nucleolar large rRNA by RNA polymerase I GO:1901838 9.16 SMARCA4 SMARCB1
13 positive regulation of glucose mediated signaling pathway GO:1902661 8.96 SMARCA4 SMARCB1
14 positive regulation of DNA binding transcription factor activity GO:0051091 8.92 CTNNB1 HMGA2 SMARCA4 SMARCB1

Molecular functions related to Atypical Teratoid Rhabdoid Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transcription factor binding GO:0008134 9.46 CCND1 CTNNB1 HMGA2 SMARCA4
2 Tat protein binding GO:0030957 9.26 SMARCA4 SMARCB1
3 RNA polymerase I CORE element sequence-specific DNA binding GO:0001164 8.96 SMARCA4 SMARCB1
4 nucleosomal DNA binding GO:0031492 8.8 HMGA2 SMARCA4 SMARCB1

Sources for Atypical Teratoid Rhabdoid Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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