WT1
MCID: WLM013
MIFTS: 68

Wilms Tumor 1 (WT1)

Categories: Blood diseases, Cancer diseases, Fetal diseases, Genetic diseases, Nephrological diseases, Rare diseases
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Aliases & Classifications for Wilms Tumor 1

MalaCards integrated aliases for Wilms Tumor 1:

Name: Wilms Tumor 1 57 73 28 5
Nephroblastoma 57 11 19 58 28 5 14 71
Wilms Tumor 57 58 43 14
Wt1 57 19 73
Bilateral Wilms Tumor 19 71
Wilms' Tumor 11 19
Nonanaplastic Kidney Wilms Tumor 71
Childhood Kidney Wilms Tumor 71
Adult Kidney Wilms Tumor 71
Renal Embryonic Tumor 58
Wilms Tumor, Somatic 57
Adult Nephroblastoma 11
Wilms Tumor, Type 1 57
Wt1 Disorder 24

Characteristics:


Inheritance:

Wilms Tumor 1: Autosomal dominant 57
Nephroblastoma: Autosomal dominant 58

Prevelance:

Nephroblastoma: 1-9/1000000 (Europe, United Kingdom, Austria, Belgium, Croatia, Estonia, Finland, Ireland, Italy, Lithuania, Malta, Norway, Poland, Slovakia, Slovenia, Spain, Switzerland, Netherlands) 1-9/100000 (Europe, Europe) 1-5/10000 (Europe) <1/1000000 (Bulgaria, Czech Republic, Germany, Iceland, Latvia, Portugal) 58

Age Of Onset:

Nephroblastoma: Childhood 58

OMIM®:

57 (Updated 08-Dec-2022)
Miscellaneous:
genetic heterogeneity
majority of wilms tumors are sporadic
onset between 2-5 years
rare adult cases reported
5-10% of all wilms tumor are bilateral
21% of hereditary wilms tumor are bilateral
associated with several congenital malformation syndromes (wagr , abnormal urogenital development syndromes)
two-step mutation hypothesis (germline mutation followed by somatic mutation or two sequential somatic mutations)
loss of tumor suppressor gene
associated with several loci on chromosomes 11p15 (wt2, ), 16 (wt3, ), 17 (wt4, ), and 7 (wt5, ).


GeneReviews:

24
Penetrance The penetrance of wt1 disorder is high. it is age dependent, reaching about 90% by the end of puberty....

Classifications:

Orphanet: 58  
Rare renal diseases


External Ids:

Disease Ontology 11 DOID:2154
OMIM® 57 194070
OMIM Phenotypic Series 57 PS194070
MeSH 43 D009396
SNOMED-CT 68 25081006
MESH via Orphanet 44 D009396
ICD10 via Orphanet 32 C64
UMLS via Orphanet 72 C0027708
Orphanet 58 ORPHA654
MedGen 40 C0027708
UMLS 71 C0027708 C1332219 C1333015 more

Summaries for Wilms Tumor 1

OMIM®: 57 Wilms tumor is the most common renal tumor of childhood, occurring with an incidence of 1 in 10,000 and with a median age of diagnosis between 3 and 4 years of age. Wilms tumors are thought to develop from abnormally persistent embryonal cells within nephrogenic rests. Histologically, Wilms tumor mirrors the development of the normal kidney and classically consists of 3 cell types: blastema, epithelia, and stroma (summary by Slade et al., 2010). (194070) (Updated 08-Dec-2022)

MalaCards based summary: Wilms Tumor 1, also known as nephroblastoma, is related to wilms tumor 5 and nephrotic syndrome, type 4, and has symptoms including abdominal pain An important gene associated with Wilms Tumor 1 is WT1 (WT1 Transcription Factor), and among its related pathways/superpathways are DNA IR-double strand breaks and cellular response via ATM and DNA IR-damage and cellular response via ATR. The drugs Miconazole and Clotrimazole have been mentioned in the context of this disorder. Affiliated tissues include children, kidney and myeloid, and related phenotypes are abdominal pain and nephroblastoma

GARD: 19 A rare malignant renal tumor, typically affecting the pediatric population, characterized by an abnormal proliferation of cells that resemble the kidney cells of an embryo (metanephroma), leading to the term embryonal tumor.

Orphanet: 58 A rare malignant renal tumor, typically affecting the pediatric population, characterized by an abnormal proliferation of cells that resemble the kidney cells of an embryo (metanephroma), leading to the term embryonal tumor.

UniProtKB/Swiss-Prot: 73 Embryonal malignancy of the kidney that affects approximately 1 in 10'000 infants and young children. It occurs both in sporadic and hereditary forms.

Disease Ontology: 11 A kidney cancer that affects the kidneys and typically located in children.

GeneReviews: NBK556455

Related Diseases for Wilms Tumor 1

Diseases in the Hereditary Wilms' Tumor family:

Wilms Tumor 1 Wilms Tumor 2
Wilms Tumor 3 Wilms Tumor 4
Wilms Tumor 5 Wilms Tumor 6
Familial Wilms Tumor 2

Diseases related to Wilms Tumor 1 via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 1178)
# Related Disease Score Top Affiliating Genes
1 wilms tumor 5 32.9 WT1 TRIM28 POU6F2 IGF2 H19 GPC3
2 nephrotic syndrome, type 4 32.8 WT1 LOC107982234
3 hereditary wilms' tumor 32.7 WT1 TRIM28 DIS3L2
4 simpson-golabi-behmel syndrome, type 1 32.5 GPC4 GPC3
5 wilms tumor, aniridia, genitourinary anomalies, and mental retardation syndrome 32.5 WT1 LOC107982234 IGF2
6 beckwith-wiedemann syndrome 32.5 WT1 MEG3 IGF2-AS IGF2 H19 GPC3
7 clear cell sarcoma 32.4 WT1 IGF2 BRAF
8 li-fraumeni syndrome 32.4 IGF2 CHEK2 BRCA2
9 leiomyosarcoma 32.3 WT1 IGF2 CHEK2
10 hepatoblastoma 32.3 WT1 IGF2 H19 GPC3 BRCA2
11 fallopian tube carcinoma 32.2 WT1 CHEK2 BRCA2
12 embryonal rhabdomyosarcoma 32.2 WT1 IGF2 H19
13 hemihyperplasia, isolated 32.1 WT1 IGF2 H19
14 intellectual developmental disorder, x-linked, syndromic, wilson-turner type 32.0 WT1 POU6F2 IGF2 DIS3L2
15 syndromic x-linked intellectual disability 32.0 WT1 POU6F2 IGF2
16 ovarian carcinosarcoma 31.9 WT1 BRCA2
17 endosalpingiosis 31.9 WT1 BRCA2
18 ovarian gonadoblastoma 31.9 WT1 GPC3
19 ovarian clear cell adenocarcinoma 31.9 WT1-AS CHEK2
20 epididymis adenocarcinoma 31.9 WT1 BRAF
21 epididymis cancer 31.9 WT1 BRAF
22 liver leiomyosarcoma 31.9 WT1 GPC3
23 ovarian germ cell cancer 31.8 WT1 GPC3
24 malignant teratoma 31.7 WT1 GPC3
25 adenofibroma 31.7 WT1 BRAF
26 syndromic intellectual disability 31.7 WT1 POU6F2 IGF2
27 osteogenic sarcoma 31.4 MEG3 IGF2 H19 CHEK2
28 rhabdomyosarcoma 31.3 WT1 IGF2 H19 GPC3 CHEK2 BRCA2
29 cholangiocarcinoma 31.0 H19 GPC3 BRCA2 BRAF
30 lynch syndrome 31.0 IGF2 CHEK2 BRCA2 BRAF
31 silver-russell syndrome 1 30.9 MEG3 IGF2 H19
32 sotos syndrome 30.9 IGF2 GPC3 DIS3L2
33 adrenal cortical carcinoma 30.8 IGF2 H19 CHEK2 BRCA2 BRAF
34 ovarian epithelial cancer 30.7 MEG3 H19
35 renal wilms' tumor 30.7 WT1 DIS3L2
36 thyroid cancer, nonmedullary, 1 30.6 MEG3 H19 BRAF
37 wilms tumor predisposition 30.6 TRIM28 REST
38 wilms tumor 2 30.4 KCNQ1DN H19
39 choriocarcinoma 30.3 IGF2 H19 GPC3
40 global developmental delay, lung cysts, overgrowth, and wilms tumor 11.7
41 cervical wilms' tumor 11.6
42 wilms tumor 3 11.5
43 stromal predominant kidney wilms' tumor 11.4
44 mixed cell type kidney wilms' tumor 11.3
45 blastema predominant kidney wilms' tumor 11.3
46 adenomatoid tumor 11.3
47 wilms tumor, aniridia, genitourinary anomalies, mental retardation, and obesity syndrome 11.2
48 metachronous kidney wilms' tumor 11.2
49 mulibrey nanism 11.2
50 mosaic variegated aneuploidy syndrome 1 11.2

Graphical network of the top 20 diseases related to Wilms Tumor 1:



Diseases related to Wilms Tumor 1

Symptoms & Phenotypes for Wilms Tumor 1

Human phenotypes related to Wilms Tumor 1:

58 30 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abdominal pain 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002027
2 nephroblastoma 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002667
3 hypertension 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000822
4 fever 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001945
5 hematuria 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000790
6 weight loss 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001824
7 neoplasm of the lung 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100526
8 aniridia 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0000526
9 neoplasm of the liver 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002896
10 lymphadenopathy 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002716
11 neoplasm 58 Very frequent (99-80%)

Symptoms via clinical synopsis from OMIM®:

57 (Updated 08-Dec-2022)
Genitourinary Kidneys:
nephroblastoma

Neoplasia:
nephroblastoma

Clinical features from OMIM®:

194070 (Updated 08-Dec-2022)

UMLS symptoms related to Wilms Tumor 1:


abdominal pain

MGI Mouse Phenotypes related to Wilms Tumor 1:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.7 AIRN BRAF BRCA2 CHEK2 GPC3 IGF2
2 reproductive system MP:0005389 9.32 AIRN BRAF BRCA2 CHEK2 GPC3 IGF2

Drugs & Therapeutics for Wilms Tumor 1

Drugs for Wilms Tumor 1 (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 152)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
2
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
3
Itraconazole Approved, Investigational Phase 4 84625-61-6 55283
4
Epirubicin Approved Phase 4 56420-45-2 41867
5
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
6
Vincristine Approved, Investigational Phase 4 2068-78-2, 57-22-7 5978
7
Dactinomycin Approved, Investigational Phase 4 50-76-0 2019 457193
8
Oxytetracycline Approved, Investigational, Vet_approved Phase 4 79-57-2 54715139 54675779
9
Doxorubicin Approved, Investigational Phase 4 23214-92-8 31703
10
Pirarubicin Investigational Phase 4 72496-41-4 4844
11 Antifungal Agents Phase 4
12
Hydroxyitraconazole Phase 4 108222
13 Cytochrome P-450 Enzyme Inhibitors Phase 4
14 Cytochrome P-450 CYP3A Inhibitors Phase 4
15 Anti-Bacterial Agents Phase 4
16 Antirheumatic Agents Phase 4
17 Anti-Infective Agents Phase 4
18 Antimitotic Agents Phase 4
19 Tubulin Modulators Phase 4
20 Antibiotics, Antitubercular Phase 4
21 Liposomal doxorubicin Phase 4
22 Immunosuppressive Agents Phase 4
23
Trimethoprim Approved, Vet_approved Phase 3 738-70-5 5578
24
Sulfamethoxazole Approved Phase 3 723-46-6 5329
25
Lenograstim Approved, Investigational Phase 3 135968-09-1
26 Hormones Phase 3
27 Liver Extracts Phase 3
28 Calcium, Dietary Phase 3
29 Cactinomycin Phase 3
30 Immunologic Factors Phase 3
31 Adjuvants, Immunologic Phase 3
32
Calcium Nutraceutical Phase 3 7440-70-2 271
33
Topotecan Approved, Investigational Phase 2 123948-87-8, 119413-54-6 60699 60700
34
Adenosine Approved, Investigational Phase 2 58-61-7 60961
35
Trastuzumab Approved, Investigational Phase 2 180288-69-1
36
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
37
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
38
Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
39
Prednisolone Approved, Vet_approved Phase 2 50-24-8 4894 5755
40
Etanercept Approved, Investigational Phase 2 185243-69-0
41
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5 1875
42
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 4159 6741
43
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239
44
Picropodophyllin Approved, Investigational Phase 2 518-28-5, 477-47-4 10607 72435
45
Daunorubicin Approved Phase 2 20830-81-3 30323
46
Ivosidenib Approved, Investigational Phase 2 1448347-49-6 78140854 71657455
47
Cisplatin Approved Phase 1, Phase 2 15663-27-1 2767 5702198 441203
48
Talazoparib Approved, Investigational Phase 1, Phase 2 1207456-01-6 135565082
49
Ipilimumab Approved Phase 2 477202-00-9
50
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030

Interventional clinical trials:

(show top 50) (show all 122)
# Name Status NCT ID Phase Drugs
1 Clinical Study to Evaluate the Efficacy of Prophylactic Itraconazole in High-Dose Chemotherapy and Autologous Hematopoietic Stem Cell Transplantation for Pediatric Solid Tumors Completed NCT00336531 Phase 4 itraconazole
2 A Multicenter, Randomized, Double-blind, Prospective Study to Evaluate the Efficacy and Safety of Vincristine, Dactinomycin/Cyclophosphamide Combination Therapy Combined With Liposomal Doxorubicin/Doxorubicin/Pharmorubicin/Pirarubicin in 0.5-14 Year Old Children With Nephroblastoma. Not yet recruiting NCT03892330 Phase 4 Vincristine;Oxytetracycline/ Cyclophosphamide;Liposomal doxorubicin;Doxorubicin;Pharmorubicin;Pirarubicin
3 Nephroblastoma (Wilms Tumour) Clinical Trial And Study Unknown status NCT00047138 Phase 3 carboplatin;cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
4 Nephroblastoma Clinical Trial and Study Unknown status NCT00003804 Phase 3 vincristine sulfate
5 National Wilms Tumor Study-5 -- Treatment of Relapsed Patients, A National Wilms Tumor Study Group Phase III Study Completed NCT00002610 Phase 3 carboplatin;cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
6 NATIONAL WILMS TUMOR STUDY-5 -- THERAPEUTIC TRIAL AND BIOLOGY STUDY Completed NCT00002611 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
7 A Randomized Double Blinded Trial of Topical Caphosol to Prevent Oral Mucositis in Children Undergoing Hematopoietic Stem Cell Transplantation Completed NCT01305200 Phase 3 supersaturated calcium phosphate rinse
8 Detection of Minimal Residual Disease in Newly Diagnosed Patients With Leukemia and Those Who Undergo a Bone Marrow Transplant Using the Wilms Tumor Suppressor Gene (WT1) as a Marker By RT-PCR Completed NCT00179829 Phase 2, Phase 3
9 Treatment for Patients With Bilateral, Multicentric, or Bilaterally-Predisposed Unilateral Wilms Tumor Active, not recruiting NCT00945009 Phase 3 Doxorubicin Hydrochloride;Vincristine Sulfate
10 Treatment for Very Low and Standard Risk Favorable Histology Wilms Tumor Active, not recruiting NCT00352534 Phase 3 vincristine sulfate;doxorubicin hydrochloride
11 Treatment of Newly Diagnosed Higher Risk Favorable Histology Wilms Tumors Active, not recruiting NCT00379340 Phase 3 doxorubicin hydrochloride;liposomal vincristine sulfate;cyclophosphamide;etoposide
12 Prospective and Randomized Study of Fixed Versus Flexible Prophylactic Administration of Granulocyte Colony-Stimulating Factor (G-CSF) in Children With Cancer Terminated NCT01987596 Phase 3
13 Protocol For The Treatment Of Relapsed And Refractory Wilms Tumour And Clear Cell Sarcoma Of The Kidney (CCSK) Unknown status NCT00025103 Phase 2 carboplatin;cyclophosphamide;doxorubicin hydrochloride;etoposide;melphalan;vincristine sulfate
14 Therapeutic Efficacy of Wilms Tumor Gene (WT1) mRNA-electroporated Autologous Dendritic Cell Vaccination in Patients With Myeloid Malignancies and Multiple Myeloma: A Phase II Trial Unknown status NCT00965224 Phase 2
15 Therapeutic Efficacy of Wilms' Tumor Gene (WT1) mRNA-electroporated Autologous Dendritic Cell Vaccination in Patients With Solid Tumors: a Phase I/Feasibility Study Unknown status NCT01291420 Phase 1, Phase 2
16 A Phase II Trial of All-Trans-Retinoic Acid in Combination With Interferon-Alpha 2a in Children With Recurrent Neuroblastoma or Wilms' Tumor Completed NCT00001509 Phase 2 IFN-alpha with retinoic acid
17 Treatment of High Risk Renal Tumors: A Groupwide Phase II Study Completed NCT00335556 Phase 2 Doxorubicin Hydrochloride;Irinotecan Hydrochloride;Cyclophosphamide;Etoposide;Carboplatin;Vincristine Sulfate
18 Wilm's Tumor 1 (WT1) Peptide Vaccination for Patients With High Risk Hematological Malignancies Completed NCT00433745 Phase 2 WT1 Peptide Vaccine
19 A Phase II Study of IMC-A12 (Anti-IGF-I Receptor Monoclonal Antibody, NSC #742460) in Children With Relapsed/Refractory Solid Tumors Completed NCT00831844 Phase 2
20 A Pilot Study of Tumor Lysate-pulsed Dendritic Cell Vaccine for Immune Augmentation for High-risk Solid Tumor Patients Following Autologous Stem Cell Transplantation Completed NCT00405327 Phase 2
21 A Phase 2 Study of IMGN901 (Lorvotuzumab Mertansine; NSC#: 783609) in Children With Relapsed or Refractory Wilms Tumor, Rhabdomyosarcoma, Neuroblastoma, Pleuropulmonary Blastoma, Malignant Peripheral Nerve Sheath Tumor (MPNST) and Synovial Sarcoma Completed NCT02452554 Phase 2
22 A Phase II Study of Topotecan in Children With Recurrent Wilms Tumor Completed NCT00187031 Phase 2 Topotecan, Filgrastim (G-CSF), Pegfilgrastim
23 Myeloablative Chemotherapy With Stem Cell Rescue for Rare Poor-Prognosis Cancers Completed NCT00141765 Phase 2
24 Phase II Liposomal Vincristine for Pediatric and Adolescent Patients With Relapsed Malignancies Completed NCT00038207 Phase 2 Liposomal Vincristine
25 Phase II Pharmacokinetic Study to Assess the Age-dependency in the Clearance of Doxorubicin in Paediatric Patients With Solid Tumours and Leukaemia Completed NCT01095926 Phase 2 doxorubicin
26 Phase II Trial of Ixabepilone (BMS-247550), an Epothilone B Analog, in Children and Young Adults With Refractory Solid Tumors Completed NCT00331643 Phase 2 ixabepilone
27 Soluble Tumor Necrosis Factor Receptor: Enbrel® (Etanercept) for the Treatment of Acute Non-Infectious Pulmonary Dysfunction (Idiopathic Pneumonia Syndrome) Following Allogeneic Stem Cell Transplantation Completed NCT00309907 Phase 2 methylprednisolone
28 Phase II Study of Cyclophosphamide, Doxorubicin, Vincristine, Etoposide, and Ifosfamide, Followed by Resection and Radiotherapy in Patients With Peripheral Primitive Neuroectodermal Tumors or Ewing's Sarcoma Completed NCT00002466 Phase 2 cyclophosphamide;doxorubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
29 A Phase II Study of the Raf Kinase and Receptor Tyrosine Kinase Inhibitor Sorafenib in Children and Young Adults With Relapsed/Refractory Rhabdomyosarcoma, Wilms Tumor, Hepatocellular Carcinoma, and Papillary Thyroid Carcinoma Completed NCT01502410 Phase 2 sorafenib tosylate
30 Treatment of Newly Diagnosed Diffuse Anaplastic Wilms Tumors (DAWT) and Relapsed Favorable Histology Wilms Tumors (FHWT) Recruiting NCT04322318 Phase 2 Carboplatin;Cyclophosphamide;Doxorubicin;Etoposide;Ifosfamide;Irinotecan;Topotecan;Vincristine
31 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Tipifarnib in Patients With Tumors Harboring HRAS Genomic Alterations Recruiting NCT04284774 Phase 2 Tipifarnib
32 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of AG-120 (Ivosidenib) in Patients With Tumors Harboring IDH1 Mutations Recruiting NCT04195555 Phase 2 Ivosidenib
33 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-292 in Patients With Tumors Harboring RET Gene Alterations Recruiting NCT04320888 Phase 2 Selpercatinib
34 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of LOXO-101 (Larotrectinib) in Patients With Tumors Harboring Actionable NTRK Fusions Recruiting NCT03213704 Phase 2 Larotrectinib Sulfate
35 A Randomized Phase I/II Study of Talazoparib or Temozolomide in Combination With Onivyde in Children With Recurrent Solid Malignancies and Ewing Sarcoma Recruiting NCT04901702 Phase 1, Phase 2 Onivyde;Talazoparib;Temozolomide
36 Phase II Study Investigating the Efficacy of Neoadjuvant Dual Checkpoint Inhibition and Cryoablation Therapy in Children, Adolescents, and Young Adults With Relapsed/Refractory Solid Tumors Recruiting NCT05302921 Phase 2 Nivolumab;Ipilimumab
37 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) Screening Protocol Recruiting NCT03155620 Phase 2 Ensartinib;Erdafitinib;Larotrectinib Sulfate;Olaparib;Palbociclib;Samotolisib;Selpercatinib;Selumetinib Sulfate;Tazemetostat;Tipifarnib;Ulixertinib;Vemurafenib
38 NCI-COG Pediatric MATCH (Molecular Analysis For Therapy Choice)- Phase 2 Subprotocol of LY3023414 in Patients With Solid Tumors Recruiting NCT03213678 Phase 2 Samotolisib
39 A Phase 1/2 Study of Tegavivint (NSC#826393) in Children, Adolescents, and Young Adults With Recurrent or Refractory Solid Tumors, Including Lymphomas and Desmoid Tumors Recruiting NCT04851119 Phase 1, Phase 2 Tegavivint
40 Multi-Center Trial Sponsored by St. Jude Children's Research Hospital for the Treatment of Newly Diagnosed Patient's With Wilm's Tumor Requiring Abdominal Radiation Delivered With Proton Beam Irradiation Recruiting NCT04968990 Phase 2 DD-4A Chemotherapy Regimen
41 First-line Immunotherapy Using Wilms' Tumor Protein 1 (WT1)-Targeted Dendritic Cell Vaccinations for Malignant Pleural Mesothelioma Recruiting NCT02649829 Phase 1, Phase 2
42 Wilms' Tumor (WT1) Antigen-targeted Dendritic Cell Vaccination to Prevent Relapse in Adult Patients With Acute Myeloid Leukemia: a Multicenter Randomized Phase II Trial Recruiting NCT01686334 Phase 2
43 Phase 2 Trial of XL184 (Cabozantinib) an Oral Small-Molecule Inhibitor of Multiple Kinases, in Children and Young Adults With Refractory Sarcomas, Wilms Tumor, and Other Rare Tumors Active, not recruiting NCT02867592 Phase 2 Cabozantinib;Cabozantinib S-malate
44 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of BVD-523FB (Ulixertinib) in Patients With Tumors Harboring Activating MAPK Pathway Mutations Active, not recruiting NCT03698994 Phase 2 Ulixertinib
45 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Palbociclib in Patients With Tumors Harboring Activating Alterations in Cell Cycle Genes Active, not recruiting NCT03526250 Phase 2 Palbociclib
46 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- A Phase 2 Subprotocol of Olaparib in Patients With Tumors Harboring Defects in DNA Damage Repair Genes Active, not recruiting NCT03233204 Phase 2 Olaparib
47 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice) - Phase 2 Subprotocol of Tazemetostat in Patients With Tumors Harboring Alterations in EZH2 or Members of the SWI/SNF Complex Active, not recruiting NCT03213665 Phase 2 Tazemetostat
48 NCI-COG Pediatric MATCH (Molecular Analysis for Therapy Choice)- Phase 2 Subprotocol of Vemurafenib in Patients With Tumors Harboring BRAF V600 Mutations Active, not recruiting NCT03220035 Phase 2 Vemurafenib
49 A Pilot Study of Lyso-thermosensitive Liposomal Doxorubicin (LTLD, ThermoDox®) and Magnetic Resonance-Guided High Intensity Focused Ultrasound (MR-HIFU) for Treatment of Relapsed or Refractory Solid Tumors Not yet recruiting NCT04791228 Phase 2 Lyso-thermosensitive Liposomal Doxorubicin
50 Phase 2 Trial Evaluating Metronomic Chemotherapy in Patients With a Relapsed or Refractory Wilms Tumor Not yet recruiting NCT05384821 Phase 1, Phase 2 Vincristine;Irinotecan;Temozolomide;Etoposide;Cis-Retinoic acid

Search NIH Clinical Center for Wilms Tumor 1

Cochrane evidence based reviews: wilms tumor

Genetic Tests for Wilms Tumor 1

Genetic tests related to Wilms Tumor 1:

# Genetic test Affiliating Genes
1 Wilms Tumor 1 28 BRCA2 GPC3 GPC4 H19 IGF2 WT1
2 Nephroblastoma 28

Anatomical Context for Wilms Tumor 1

Organs/tissues related to Wilms Tumor 1:

FMA: Children
MalaCards : Kidney, Myeloid, T Cells, Lung, Bone Marrow, Liver, Bone
ODiseA: Kidney

Publications for Wilms Tumor 1

Articles related to Wilms Tumor 1:

(show top 50) (show all 9423)
# Title Authors PMID Year
1
Vertical transmission of a mutation in exon 1 of the WT1 gene: lessons for genetic counseling. 62 57 5
18688870 2008
2
Genotype-phenotype analysis of pediatric patients with WT1 glomerulopathy. 62 24 5
27300205 2017
3
Clinical and molecular characterization of patients with heterozygous mutations in wilms tumor suppressor gene 1. 62 24 5
25818337 2015
4
Biallelic BRCA2 mutations are associated with multiple malignancies in childhood including familial Wilms tumour. 57 5
15689453 2005
5
Somatic glypican 3 (GPC3) mutations in Wilms' tumour. 57 5
12085187 2002
6
Correlation of germ-line mutations and two-hit inactivation of the WT1 gene with Wilms tumors of stromal-predominant histology. 57 5
9108089 1997
7
Donor splice-site mutations in WT1 are responsible for Frasier syndrome. 24 5
9398852 1997
8
TRIM28 congenital predisposition to Wilms' tumor: novel mutations and presentation in a sibling pair. 62 5
32699065 2020
9
Embryonal precursors of Wilms tumor. 62 57
31806814 2019
10
Early recognition of gonadal dysgenesis in congenital nephrotic syndrome
. 62 5
27719739 2016
11
WT1, WTX and CTNNB1 mutation analysis in 43 patients with sporadic Wilms' tumor. 62 5
23117548 2013
12
Frequency of WT1 and 11p15 constitutional aberrations and phenotypic correlation in childhood Wilms tumour patients. 62 5
22796116 2012
13
Analysis of the Wilms' tumor suppressor gene (WT1) in patients 46,XY disorders of sex development. 62 5
21508141 2011
14
Clinical pictures and novel mutations of WT1-associated Denys-Drash syndrome in two Chinese children. 62 5
21851196 2011
15
Wilms tumor cells with WT1 mutations have characteristic features of mesenchymal stem cells and express molecular markers of paraxial mesoderm. 62 57
20106868 2010
16
An X chromosome gene, WTX, is commonly inactivated in Wilms tumor. 62 57
17204608 2007
17
Lack of puberty despite elevated estradiol in a 46,XY phenotypic female with Frasier syndrome. 62 5
16717397 2006
18
Differential expression of a novel ankyrin containing E3 ubiquitin-protein ligase, Hace1, in sporadic Wilms' tumor versus normal kidney. 62 57
15254018 2004
19
Twenty-four new cases of WT1 germline mutations and review of the literature: genotype/phenotype correlations for Wilms tumor development. 62 5
15150775 2004
20
Association of biallelic BRCA2/FANCD1 mutations with spontaneous chromosomal instability and solid tumors of childhood. 62 5
14670928 2004
21
An unusual phenotype of Frasier syndrome due to IVS9 +4C>T mutation in the WT1 gene: predominantly male ambiguous genitalia and absence of gonadal dysgenesis. 62 5
12050205 2002
22
Absence of PPP2R1A mutations in Wilms tumor. 62 57
11360189 2001
23
Wilms' tumor and related abnormalities in the fetus and newborn. 62 57
10475544 1999
24
A zinc finger truncation of murine WT1 results in the characteristic urogenital abnormalities of Denys-Drash syndrome. 62 57
10077614 1999
25
Nephrogenic rests and the pathogenesis of Wilms tumor: developmental and clinical considerations. 62 57
9781906 1998
26
Frasier syndrome is caused by defective alternative splicing of WT1 leading to an altered ratio of WT1 +/-KTS splice isoforms. 62 5
9499425 1998
27
Linkage of familial Wilms' tumor predisposition to chromosome 19 and a two-locus model for the etiology of familial tumors. 62 57
9537236 1998
28
Familial Wilms' tumor associated with a WT1 zinc finger mutation. 62 5
8975729 1996
29
[Hereditary renal tumors: Wilms' tumor--congenital anomalies' syndrome]. 62 57
8538037 1995
30
Fine structure analysis of the WT1 gene in sporadic Wilms tumors. 62 57
8170946 1994
31
WT1-mediated growth suppression of Wilms tumor cells expressing a WT1 splicing variant. 62 57
8266105 1993
32
Altered trans-activational properties of a mutated WT1 gene product in a WAGR-associated Wilms' tumor. 62 57
8402654 1993
33
A dominant mutation in the Wilms tumor gene WT1 cooperates with the viral oncogene E1A in transformation of primary kidney cells. 62 57
1321431 1992
34
Pax-2 is a DNA-binding protein expressed in embryonic kidney and Wilms tumor. 62 57
1311084 1992
35
Familial predisposition to Wilms tumor does not segregate with the WT1 gene. 62 57
1655633 1991
36
Constitutional extra chromosomal element in a family with Wilms' tumor. 62 57
1648545 1991
37
Smallest region of overlap in Wilms tumor deletions uniquely implicates an 11p13 zinc finger gene as the disease locus. 62 57
1646159 1991
38
Parental origin of de novo constitutional deletions of chromosomal band 11p13. 62 57
1971994 1990
39
Constitutional and somatic deletions of two different regions of maternal chromosome 11 in Wilms tumor. 62 57
1973142 1990
40
Role for the Wilms tumor gene in genital development? 62 57
1973540 1990
41
An internal deletion within an 11p13 zinc finger gene contributes to the development of Wilms' tumor. 62 57
2163761 1990
42
Molecular genetic evidence for common pathogenesis of childhood and adult Wilms' tumor. 62 57
2540899 1989
43
Molecular analysis of a reciprocal translocation t(5;11) (q11;p13) in a WAGR patient. 62 57
2841227 1988
44
Analysis of WAGR deletions and related translocations with gene-specific DNA probes, using FACS-selected cell hybrids. 62 57
2829363 1988
45
HRAS1-selected chromosome transfer generates markers that colocalize aniridia- and genitourinary dysplasia-associated translocation breakpoints and the Wilms tumor gene within band 11p13. 62 57
3037545 1987
46
Genetic mechanisms of tumor-specific loss of 11p DNA sequences in Wilms tumor. 62 57
3039839 1987
47
Nonrandom loss of maternal chromosome 11 alleles in Wilms tumors. 62 57
2883892 1987
48
Introduction of a normal human chromosome 11 into a Wilms' tumor cell line controls its tumorigenic expression. 62 57
3031816 1987
49
Molecular analysis of chromosome 11 deletions in aniridia-Wilms tumor syndrome. 62 57
3001710 1985
50
Insulin-like growth factor-II gene expression in Wilms' tumour and embryonic tissues. 62 57
2995818 1985

Variations for Wilms Tumor 1

ClinVar genetic disease variations for Wilms Tumor 1:

5 (show top 50) (show all 1667)
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 FZD6 NM_003506.4(FZD6):c.346C>T (p.Arg116Ter) SNV Other
438765 rs769116796 GRCh37: 8:104330986-104330986
GRCh38: 8:103318758-103318758
2 MED12 NM_005120.3(MED12):c.131G>A (p.Gly44Asp) SNV Other
92220 rs199469672 GRCh37: X:70339254-70339254
GRCh38: X:71119404-71119404
3 TET2-AS1, TET2 NM_001127208.3(TET2):c.4456T>C (p.Ser1486Pro) SNV Other
438791 rs1553918194 GRCh37: 4:106193994-106193994
GRCh38: 4:105272837-105272837
4 CTNNB1 NM_001904.4(CTNNB1):c.133_135del (p.Ser45del) DEL Other
17576 rs587776850 GRCh37: 3:41266136-41266138
GRCh38: 3:41224643-41224645
5 CTNNB1 NM_001904.4(CTNNB1):c.770C>T (p.Thr257Ile) SNV Other
438758 rs1553630452 GRCh37: 3:41267186-41267186
GRCh38: 3:41225695-41225695
6 CTNNB1 NM_001904.4(CTNNB1):c.134C>T (p.Ser45Phe) SNV Other
17588 rs121913409 GRCh37: 3:41266137-41266137
GRCh38: 3:41224646-41224646
7 WT1 NM_024426.6(WT1):c.893_909del (p.Asn298fs) DEL Pathogenic
3484 rs587776573 GRCh37: 11:32439179-32439195
GRCh38: 11:32417633-32417649
8 WT1 NM_024426.6(WT1):c.1094del (p.Gly365fs) DEL Pathogenic
3485 rs587776574 GRCh37: 11:32421513-32421513
GRCh38: 11:32399967-32399967
9 WT1 NM_024426.6(WT1):c.1387C>T (p.Arg463Ter) SNV Pathogenic
Pathogenic
3494 rs121907909 GRCh37: 11:32413578-32413578
GRCh38: 11:32392032-32392032
10 WT1 NM_024426.6(WT1):c.1303C>T (p.Arg435Ter) SNV Pathogenic
Pathogenic
Pathogenic
3497 rs121907906 GRCh37: 11:32414263-32414263
GRCh38: 11:32392717-32392717
11 WT1, LOC107982234 NM_024426.6(WT1):c.546C>A (p.Tyr182Ter) SNV Pathogenic
3506 rs121907911 GRCh37: 11:32456361-32456361
GRCh38: 11:32434815-32434815
12 GPC3 NC_000023.10:g.(?_132887503)_(132888209_?)del DEL Pathogenic
1453456 GRCh37: X:132887503-132888209
GRCh38:
13 GPC3 NC_000023.10:g.(?_132670152)_(132888213_?)del DEL Pathogenic
1458423 GRCh37: X:132670152-132888213
GRCh38:
14 GPC3 NM_004484.3(GPC3):c.1574-?_*379+?del DEL Pathogenic
239938 GRCh37:
GRCh38:
15 GPC3 NC_000023.10:g.(?_132795752)_(132888209_?)del DEL Pathogenic
1069348 GRCh37: X:132795752-132888209
GRCh38:
16 GPC3 NM_004484.4(GPC3):c.892G>T (p.Glu298Ter) SNV Pathogenic
1074502 GRCh37: X:132887649-132887649
GRCh38: X:133753622-133753622
17 GPC3 NC_000023.10:g.(?_132670132)_(133119496_?)del DEL Pathogenic
1460460 GRCh37: X:132670132-133119496
GRCh38:
18 GPC3 NM_004484.4(GPC3):c.175+2T>C SNV Pathogenic
1455474 GRCh37: X:133119300-133119300
GRCh38: X:133985273-133985273
19 GPC3 NM_004484.4(GPC3):c.80dup (p.Pro28fs) DUP Pathogenic
656012 rs1602581162 GRCh37: X:133119396-133119397
GRCh38: X:133985369-133985370
20 GPC3 NM_004484.4(GPC3):c.1269G>A (p.Trp423Ter) SNV Pathogenic
941417 rs2071072718 GRCh37: X:132826420-132826420
GRCh38: X:133692392-133692392
21 GPC3 NM_004484.4(GPC3):c.1307del (p.Ala436fs) DEL Pathogenic
575598 rs1569408743 GRCh37: X:132795864-132795864
GRCh38: X:133661836-133661836
22 GPC3 NM_004484.4(GPC3):c.361C>T (p.His121Tyr) SNV Pathogenic
11690 rs122453119 GRCh37: X:132888180-132888180
GRCh38: X:133754153-133754153
23 GPC3 NM_004484.4(GPC3):c.885C>A (p.Tyr295Ter) SNV Pathogenic
1433596 GRCh37: X:132887656-132887656
GRCh38: X:133753629-133753629
24 GPC3 NM_004484.4(GPC3):c.662del (p.Lys221fs) DEL Pathogenic
1455674 GRCh37: X:132887879-132887879
GRCh38: X:133753852-133753852
25 GPC3 NM_004484.4(GPC3):c.1379_1382del (p.Ser460fs) MICROSAT Pathogenic
1453391 GRCh37: X:132795789-132795792
GRCh38: X:133661761-133661764
26 CHEK2 NM_007194.4(CHEK2):c.1100_1101del (p.Thr367fs) DEL Pathogenic
438773 rs1555913934 GRCh37: 22:29091856-29091857
GRCh38: 22:28695868-28695869
27 GPC3 NC_000023.10:g.(?_132795758)_(132888203_?)del DEL Pathogenic
417449 GRCh37: X:132795758-132888203
GRCh38:
28 GPC3 NM_004484.4(GPC3):c.460_463dup (p.Tyr155fs) MICROSAT Pathogenic
940806 rs2071701505 GRCh37: X:132888077-132888078
GRCh38: X:133754050-133754051
29 TRIM28 NM_005762.3(TRIM28):c.2101C>T (p.Gln701Ter) SNV Pathogenic
973190 rs2053798835 GRCh37: 19:59061222-59061222
GRCh38: 19:58549855-58549855
30 GPC3 NM_004484.4(GPC3):c.629_654delinsCTTGCA (p.Asn210fs) INDEL Pathogenic
476661 rs1556297749 GRCh37: X:132887887-132887912
GRCh38: X:133753860-133753885
31 GPC3 NC_000023.10:g.(?_132838213)_(132888223_?)del DEL Pathogenic
830784 GRCh37: X:132838213-132888223
GRCh38:
32 GPC3 NC_000023.11:g.(?_133753472)_(133754186_?)del DEL Pathogenic
832201 GRCh37: X:132887499-132888213
GRCh38:
33 GPC3 NC_000023.10:g.(?_132670132)_(132888223_?)del DEL Pathogenic
832421 GRCh37: X:132670132-132888223
GRCh38:
34 GPC3 NC_000023.10:g.(?_132670146)_(132670327_?)del DEL Pathogenic
1069347 GRCh37: X:132670146-132670327
GRCh38:
35 GPC3 NM_004484.4(GPC3):c.513dup (p.Asp172Ter) DUP Pathogenic
664988 rs1603240717 GRCh37: X:132888027-132888028
GRCh38: X:133754000-133754001
36 GPC3 NM_004484.4(GPC3):c.256C>T (p.Arg86Ter) SNV Pathogenic
935437 rs2076400520 GRCh37: X:133087158-133087158
GRCh38: X:133953131-133953131
37 GPC3 NM_004484.4(GPC3):c.1471G>T (p.Glu491Ter) SNV Pathogenic
565351 rs1569392947 GRCh37: X:132730570-132730570
GRCh38: X:133596542-133596542
38 GPC3 NC_000023.10:g.(?_132670152)_(133119476_?)del DEL Pathogenic
1069346 GRCh37: X:132670152-133119476
GRCh38:
39 GPC3 NM_004484.4(GPC3):c.1159C>T (p.Arg387Ter) SNV Pathogenic
11694 rs122453121 GRCh37: X:132833930-132833930
GRCh38: X:133699902-133699902
40 GPC3 NM_004484.4(GPC3):c.595C>T (p.Arg199Ter) SNV Pathogenic
11689 rs104894855 GRCh37: X:132887946-132887946
GRCh38: X:133753919-133753919
41 GPC3 NC_000023.10:g.(?_132795738)_(133119496_?)del DEL Pathogenic
583690 GRCh37: X:132795738-133119496
GRCh38:
42 GPC3 NC_000023.10:g.(?_132670146)_(133119482_?)del DEL Pathogenic
830889 GRCh37: X:132670146-133119482
GRCh38:
43 GPC3 NM_004484.4(GPC3):c.271C>T (p.Gln91Ter) SNV Pathogenic
941300 rs2076400400 GRCh37: X:133087143-133087143
GRCh38: X:133953116-133953116
44 WT1 NM_024426.6(WT1):c.1399C>T (p.Arg467Trp) SNV Pathogenic
Not Provided
3487 rs121907900 GRCh37: 11:32413566-32413566
GRCh38: 11:32392020-32392020
45 WT1 NM_024426.6(WT1):c.812del (p.Pro271fs) DEL Pathogenic
Pathogenic
406680 rs1060501253 GRCh37: 11:32449577-32449577
GRCh38: 11:32428031-32428031
46 WT1, LOC107982234 NM_024426.6(WT1):c.592del (p.Ala198fs) DEL Pathogenic
429033 rs1131690795 GRCh37: 11:32456315-32456315
GRCh38: 11:32434769-32434769
47 WT1 NM_024426.6(WT1):c.880_882del (p.Tyr294del) DEL Pathogenic
438757 rs1554945033 GRCh37: 11:32449507-32449509
GRCh38: 11:32427961-32427963
48 WT1 NC_000011.10:g.(?_32396251)_(32417660_?)del DEL Pathogenic
476672 GRCh37: 11:32417797-32439206
GRCh38: 11:32396251-32417660
49 WT1 NM_024426.6(WT1):c.682dup (p.Asp228fs) DUP Pathogenic
476713 rs1554945232 GRCh37: 11:32450144-32450145
GRCh38: 11:32428598-32428599
50 WT1 NM_024426.6(WT1):c.1120C>T (p.Arg374Ter) SNV Pathogenic
Pathogenic
449416 rs1423753702 GRCh37: 11:32417947-32417947
GRCh38: 11:32396401-32396401

UniProtKB/Swiss-Prot genetic disease variations for Wilms Tumor 1:

73
# Symbol AA change Variation ID SNP ID
1 WT1 p.Pro181Ser VAR_007739 rs2234584
2 WT1 p.Ser223Asn VAR_007740
3 WT1 p.Gly253Ala VAR_007741
4 WT1 p.Arg366Cys VAR_007745
5 WT1 p.Arg366His VAR_007746
6 WT1 p.His373Gln VAR_007747
7 WT1 p.Arg394Trp VAR_007750
8 WT1 p.Cys355Gly VAR_043799
9 WT1 p.Arg394Leu VAR_043807

Copy number variations for Wilms Tumor 1 from CNVD:

6
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 24429 1 180300000 185800000 Microamplification CACNA1E Nephroblastoma

Expression for Wilms Tumor 1

Search GEO for disease gene expression data for Wilms Tumor 1.

Pathways for Wilms Tumor 1



Pathways directly related to Wilms Tumor 1:

# Pathway Source
1 Signaling by AMER1 mutants Reactome 66

Pathways related to Wilms Tumor 1 according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.12 TRIM28 CHEK2 BRCA2
2 10.71 TRIM28 CHEK2 BRCA2

GO Terms for Wilms Tumor 1

Biological processes related to Wilms Tumor 1 according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cellular response to xenobiotic stimulus GO:0071466 9.63 REST CHEK2 BRAF
2 regulation of protein localization to membrane GO:1905475 9.26 GPC4 GPC3
3 embryonic placenta morphogenesis GO:0060669 8.92 TRIM28 IGF2

Sources for Wilms Tumor 1

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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