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WT5
MCID: WLM018
MIFTS: 61

Wilms Tumor 5 (WT5)

Categories: Blood diseases, Cancer diseases, Fetal diseases, Genetic diseases, Nephrological diseases, Rare diseases
Genes Variations Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Wilms Tumor 5

About this section

MalaCards integrated aliases for Wilms Tumor 5:

Name: Wilms Tumor 5 56 52 29 6
Wilms Tumor 25 54 6 42 39
Wilms Tumor Susceptibility-5 56 29 13
Wt5 56 52 73
Wilms Tumor and Radial Bilateral Aplasia 52 71
Nephroblastoma 25 71
Hereditary Susceptibility to Wilms Tumor 5 73
Bilateral Radial Aplasia with Wilms Tumor 52
Kidney, Adenomyosarcoma, Embryonal 25
Kidney, Carcinosarcoma, Embryonal 25
Wilms Tumor, Susceptibility to 56
Kidney, Embryonal Mixed Tumor 25
Embryonal Adenosarcoma 25
Renal Cancer, Wilms 25
Wilms Tumor, Type 5 39
Wilms Tumor ; Wtsl 56
Embryonal Nephroma 25
Kidney Wilms Tumor 25
Renal Adenosarcoma 25
Renal Wilms Tumor 25
Kidney, Embryoma 25
Wilms' Tumor 25
Tumor, Wilms 25
Nephroma 25
Wtsl 56

Characteristics:

OMIM:

56
Inheritance:
somatic mutation
autosomal dominant

Miscellaneous:
most cases are sporadic
tumor suppressor gene


HPO:

31
wilms tumor 5:
Inheritance autosomal dominant inheritance somatic mutation


Classifications:

MalaCards categories:
Global: Genetic diseases Rare diseases Fetal diseases Cancer diseases
Anatomical: Nephrological diseases Blood diseases
See all MalaCards categories (disease lists)


External Ids:

OMIM 56 601583
OMIM Phenotypic Series 56 PS194070
MedGen 41 C1832099
UMLS 71 C0027708 C1832099
Sources

Summaries for Wilms Tumor 5

About this section
Genetics Home Reference : 25 Wilms tumor is a form of kidney cancer that primarily develops in children. Nearly all cases of Wilms tumor are diagnosed before the age of 10, with two-thirds being found before age 5. Wilms tumor is often first noticed because of abdominal swelling or a mass in the kidney that can be felt upon physical examination. Some affected children have abdominal pain, fever, a low number of red blood cells (anemia), blood in the urine (hematuria), or high blood pressure (hypertension). Additional signs of Wilms tumor can include loss of appetite, weight loss, nausea, vomiting, and tiredness (lethargy). Wilms tumor can develop in one or both kidneys. About 5 to 10 percent of affected individuals develop multiple tumors in one or both kidneys. Wilms tumor may spread from the kidneys to other parts of the body (metastasize). In rare cases, Wilms tumor does not involve the kidneys and occurs instead in the genital tract, bladder, abdomen, chest, or lower back. It is unclear how Wilms tumor develops in these tissues. With proper treatment, children with Wilms tumor have a 90 percent survival rate. However, the risk that the cancer will come back (recur) is between 15 and 50 percent, depending on traits of the original tumor. Tumors usually recur in the first 2 years following treatment and develop in the kidneys or other tissues, such as the lungs. Individuals who have had Wilms tumor may experience related health problems or late effects of their treatment in adulthood, such as decreased kidney function, heart disease, and development of additional cancers.

MalaCards based summary : Wilms Tumor 5, also known as wilms tumor, is related to wilms tumor predisposition and simpson-golabi-behmel syndrome, and has symptoms including abdominal pain An important gene associated with Wilms Tumor 5 is POU6F2 (POU Class 6 Homeobox 2), and among its related pathways/superpathways are Proteoglycans in cancer and Glioblastoma Multiforme. The drugs Clotrimazole and Miconazole have been mentioned in the context of this disorder. Affiliated tissues include kidney, myeloid and t cells, and related phenotypes are nephroblastoma and embryo

MedlinePlus : 42 Wilms tumor is a rare type of kidney cancer. It causes a tumor on one or both kidneys. It usually affects children, but can happen in adults. Having certain genetic conditions or birth defects can increase the risk of getting it. Children that are at risk should be screened for Wilms tumor every three months until they turn eight. Symptoms include a lump in the abdomen, blood in the urine, and a fever for no reason. Tests that examine the kidney and blood are used to find the tumor. Doctors usually diagnose and remove the tumor in surgery. Other treatments include chemotherapy and radiation and biologic therapies. Biologic therapy boosts your body's own ability to fight cancer. NIH: National Cancer Institute

UniProtKB/Swiss-Prot : 73 Hereditary susceptibility to Wilms tumor 5: Pediatric malignancy of kidney and one of the most common solid cancers in childhood.

More information from OMIM: 601583 PS194070
Sources

Related Diseases for Wilms Tumor 5

About this section

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 5 via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 833)
# Related Disease Score Top Affiliating Genes
1 wilms tumor predisposition 33.0 WT1 IGF2 H19 GPC3 CDKN1C
2 simpson-golabi-behmel syndrome 32.8 GPC3 CTNNB1
3 aniridia 1 32.5 WT1 PAX6 PAX2
4 li-fraumeni syndrome 32.2 IGF2 CTNNB1 CDKN1C
5 benign mesothelioma 30.5 WT1 PAX8
6 teratoma 30.4 H19 GPC3 CTNNB1
7 adrenocortical carcinoma, hereditary 30.4 IGF2 H19 CTNNB1
8 hemihyperplasia, isolated 30.2 WT1 IGF2 H19 CDKN1C
9 wilms tumor 1 30.1 WTAP WT1 POU6F2 PAX8 PAX6 PAX2
10 adrenal cortical carcinoma 30.1 IGF2 CTNNB1 CDKN1C
11 focal segmental glomerulosclerosis 30.1 WT1 PAX2 CTNNB1 CDKN1C
12 embryonal sarcoma 30.1 GPC3 CTNNB1
13 neural tube defects 29.9 PAX6 IGF2 H19 FZD6 CTNNB1
14 carcinosarcoma 29.8 WT1 PAX8 CTNNB1
15 umbilical hernia 29.8 IGF2 H19 CDKN1C
16 sarcoma, synovial 29.8 WT1 IGF2 CTNNB1
17 silver-russell syndrome 1 29.7 SLC22A18 IGF2 H19 CDKN1C
18 pax6-related aniridia 29.7 WT1 PAX6 IGF2 H19 GPC3
19 rhabdomyosarcoma 29.7 WT1 SLC22A18 IGF2 H19 CTNNB1 CCN3
20 female reproductive endometrioid cancer 29.5 WT1 PAX8 CTNNB1
21 renal cell carcinoma, papillary, 1 29.4 WT1 PAX8 PAX2 CTNNB1
22 medulloblastoma 29.4 PRDM2 PAX6 PAX2 IGF2 H19 CTNNB1
23 ovary adenocarcinoma 29.4 WT1 PAX8 CTNNB1
24 renal cell carcinoma, nonpapillary 29.4 WTAP WT1 PAX8 PAX2 H19 CCN3
25 renal hypodysplasia/aplasia 1 29.2 WT1 PAX8 PAX2 IGF2
26 hepatoblastoma 28.7 SLC22A18 IGF2 H19 GPC3 CTNNB1 CDKN1C
27 beckwith-wiedemann syndrome 28.5 WT1 SLC22A18 NAP1L4 IGF2 H19 GPC3
28 cervical wilms' tumor 12.7
29 global developmental delay, lung cysts, overgrowth, and wilms tumor 12.6
30 adult cystic nephroma 12.6
31 wilms tumor 4 12.5
32 epithelial predominant wilms' tumor 12.5
33 stromal predominant kidney wilms' tumor 12.5
34 wilms tumor 3 12.5
35 blastema predominant kidney wilms' tumor 12.5
36 familial wilms tumor 2 12.4
37 cellular congenital mesoblastic nephroma 12.4
38 wilms tumor, aniridia, genitourinary anomalies, mental retardation, and obesity syndrome 12.4
39 mixed cell type kidney wilms' tumor 12.3
40 metachronous kidney wilms' tumor 12.3
41 classic congenital mesoblastic nephroma 12.3
42 frasier syndrome 11.9
43 mulibrey nanism 11.7
44 hyperparathyroidism 2 with jaw tumors 11.7
45 mosaic variegated aneuploidy syndrome 3 11.7
46 mosaic variegated aneuploidy syndrome 11.7
47 li-fraumeni syndrome 2 11.6
48 bohring-opitz syndrome 11.6
49 thauvin-robinet-faivre syndrome 11.6
50 dicer1 tumor predisposition 11.5

Graphical network of the top 20 diseases related to Wilms Tumor 5:



Diseases related to Wilms Tumor 5
Sources

Symptoms & Phenotypes for Wilms Tumor 5

About this section

Human phenotypes related to Wilms Tumor 5:

31
# Description HPO Frequency HPO Source Accession
1 nephroblastoma 31 HP:0002667

Symptoms via clinical synopsis from OMIM:

56
Genitourinary Kidneys:
nephroblastoma (wilms tumor)

Neoplasia:
nephroblastoma (wilms tumor)

Clinical features from OMIM:

601583

UMLS symptoms related to Wilms Tumor 5:


abdominal pain

MGI Mouse Phenotypes related to Wilms Tumor 5:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 embryo MP:0005380 9.85 CCN1 CTNNB1 FZD6 GPC3 IGF2 PAX2
2 hearing/vestibular/ear MP:0005377 9.43 CCN5 CTNNB1 FZD6 PAX2 PAX6 PAX8
3 renal/urinary system MP:0005367 9.23 CCN3 CTNNB1 GPC3 IGF2 PAX2 PAX6
Sources

Drugs & Therapeutics for Wilms Tumor 5

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Drugs for Wilms Tumor 5 (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 248)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Clotrimazole Approved, Vet_approved Phase 4 23593-75-1 2812
2
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
3
Itraconazole Approved, Investigational Phase 4 84625-61-6 55283
4
Vincristine Approved, Investigational Phase 4 57-22-7, 2068-78-2 5978
5
Doxorubicin Approved, Investigational Phase 4 23214-92-8 31703
6
Dactinomycin Approved, Investigational Phase 4 50-76-0 457193 2019
7
Epirubicin Approved Phase 4 56420-45-2 41867
8
Cyclophosphamide Approved, Investigational Phase 4 50-18-0, 6055-19-2 2907
9
Oxytetracycline Approved, Investigational, Vet_approved Phase 4 79-57-2 54715139 5280972
10
Pirarubicin Investigational Phase 4 72496-41-4
11 Hormones Phase 4
12 Hormone Antagonists Phase 4
13 Antifungal Agents Phase 4
14 Cytochrome P-450 Enzyme Inhibitors Phase 4
15 Cytochrome P-450 CYP3A Inhibitors Phase 4
16
Hydroxyitraconazole Phase 4
17
Liposomal doxorubicin Phase 4 31703
18 Tubulin Modulators Phase 4
19 Antibiotics, Antitubercular Phase 4
20 Antimitotic Agents Phase 4
21
Decitabine Approved, Investigational Phase 2, Phase 3 2353-33-5 451668
22
Sulfamethoxazole Approved Phase 3 723-46-6 5329
23
Trimethoprim Approved, Vet_approved Phase 3 738-70-5 5578
24
Caspofungin Approved Phase 3 162808-62-0, 179463-17-3 2826718 468682
25
Captopril Approved Phase 3 62571-86-2 44093
26
Fluconazole Approved, Investigational Phase 3 86386-73-4 3365
27
Ribavirin Approved Phase 3 36791-04-5 37542
28
Palivizumab Approved, Investigational Phase 3 188039-54-5
29
Acyclovir Approved Phase 3 59277-89-3 2022
30
Amphotericin B Approved, Investigational Phase 3 1397-89-3 5280965 14956
31
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
32
Etoposide Approved Phase 3 33419-42-0 36462
33
Daunorubicin Approved Phase 3 20830-81-3 30323
34
Sorafenib Approved, Investigational Phase 3 284461-73-0 216239 406563
35
Bortezomib Approved, Investigational Phase 3 179324-69-7 387447 93860
36
Mitoxantrone Approved, Investigational Phase 3 65271-80-9 4212
37
Cytarabine Approved, Experimental, Investigational Phase 3 147-94-4, 65-46-3 6253
38
Histamine Approved, Investigational Phase 3 51-45-6 774
39
Cyproheptadine Approved Phase 3 129-03-3 2913
40
Glutamic acid Approved, Nutraceutical Phase 3 56-86-0 33032
41
Aspartic acid Approved, Nutraceutical Phase 3 56-84-8 5960
42
Asparagine Approved, Investigational, Nutraceutical Phase 3 70-47-3 6267
43 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
44 Liver Extracts Phase 3
45 Immunoglobulins Phase 3
46 Antibodies Phase 3
47 Antibodies, Monoclonal Phase 3
48 Cola Phase 3
49 Anesthetics Phase 3
50 Antihypertensive Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 221)
# Name Status NCT ID Phase Drugs
1 A Pharmacokinetic Study of Actinomycin-D and Vincristine in Children With Cancer Unknown status NCT00491946 Phase 4 Actinomycin-D;Vincristine
2 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
3 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
4 Nephroblastoma Clinical Trial and Study Unknown status NCT00003804 Phase 3 vincristine sulfate
5 Nephroblastoma (Wilms Tumour) Clinical Trial And Study Unknown status NCT00047138 Phase 3 carboplatin;cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
6 Treatment for Very Low and Standard Risk Favorable Histology Wilms Tumor Unknown status NCT00352534 Phase 3 vincristine sulfate;doxorubicin hydrochloride
7 Decitabine Augments for Post Allogeneic Stem Cell Transplantation in Patients With Acute Myeloid Leukemia and Myelodysplastic Syndrome Unknown status NCT01809392 Phase 2, Phase 3 decitabine
8 Treatment of Newly Diagnosed Higher Risk Favorable Histology Wilms Tumors Unknown status NCT00379340 Phase 3 doxorubicin hydrochloride;liposomal vincristine sulfate;cyclophosphamide;etoposide
9 Prospective and Randomized Study of Fixed Versus Flexible Prophylactic Administration of Granulocyte Colony-Stimulating Factor (G-CSF) in Children With Cancer Unknown status NCT01987596 Phase 3
10 NATIONAL WILMS TUMOR STUDY-5 -- THERAPEUTIC TRIAL AND BIOLOGY STUDY Completed NCT00002611 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
11 Glutamic Acid to Decrease Vincristine Toxicity in Children With Cancer Completed NCT00369564 Phase 3 glutamic acid
12 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
13 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
14 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
15 Phase III Study of Captopril in Patients Undergoing Autologous Bone Marrow/Stem Cell Transplantation Completed NCT00004230 Phase 3 captopril;cyclophosphamide
16 A Randomized, Comparative Study of Itraconazole Versus Fluconazole for Prevention of Aspergillus Infections in Peripheral Blood Stem Cell and Marrow Transplant Recipients Completed NCT00003883 Phase 3 fluconazole;itraconazole
17 A Randomized Double-Blind Placebo-Controlled Phase III Study To Evaluate The Safety And Efficacy Of Palivizumab Combined With Aerosolized Ribavirin Compared To Ribavirin Alone To Treat RSV Pneumonia In Patients With Bone Marrow Transplants (BMT) Completed NCT00014391 Phase 3 ribavirin
18 A Phase III Multicenter Study of Cytomegalovirus Prophylaxis With Valacyclovir for the Prevention of Serious Fungal and Bacterial Infections Among Cytomegalovirus Seronegative Recipients of Cytomegalovirus Seropositive Sx Stem Cell Transplants Completed NCT00045292 Phase 3 acyclovir;acyclovir sodium;valacyclovir
19 Music Video and Adolescent/Young Adult Resilience During Transplant Completed NCT00305851 Phase 3
20 A Strategic Study to Determine the Optimal Moment to Initiate Systemic Antifungal Therapy With Ambisome in Granulocytopenic Cancer Patients With Unexplained Fever Refractory to Empirical Antibacterials Completed NCT00003938 Phase 3 liposomal amphotericin B
21 A Multicenter, Double-Blind, Randomized, Comparative Study To Evaluate The Safety, Tolerability, And Efficacy Of MK-0991 Versus (Amphotericin B) Liposome For Injection As Empirical Therapy In Patients With Persistent Fever And Neutropenia Completed NCT00008359 Phase 3 caspofungin acetate;liposomal amphotericin B
22 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
23 Treatment for Patients With Bilateral, Multicentric, or Bilaterally-Predisposed Unilateral Wilms Tumor Active, not recruiting NCT00945009 Phase 3 Doxorubicin Hydrochloride;Vincristine Sulfate
24 A Phase III Randomized Trial for Patients With De Novo AML Using Bortezomib and Sorafenib (NSC# 681239, NSC# 724772) for Patients With High Allelic Ratio FLT3/ITD Active, not recruiting NCT01371981 Phase 3 Asparaginase;Bortezomib;Cytarabine;Daunorubicin Hydrochloride;Etoposide;Mitoxantrone Hydrochloride;Sorafenib Tosylate
25 Randomized Open-label Non-inferiority Phase 3 Clinical Trial for Patients With a Stage IV Childhood Renal Tumor, Comparing Upfront Vincristine, Actinomycin-D and Doxorubicin (Standard Arm) With Upfront Vincristine, Carboplatin and Etoposide (Experimental Arm) Not yet recruiting NCT03669783 Phase 3 treatment Vincristin;treatment Actinomycin-D;treatment Doxorubicin;treatment Carboplatin;Etoposide
26 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
27 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
28 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
29 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
30 A Randomized, Placebo-Controlled Pilot Study of Genistein Supplementation in Pediatric Cancer Patients Receiving Myelosuppressive Chemotherapy Unknown status NCT02624388 Phase 2 Genistein;Placebo
31 A Multiinstitutional Trial To Evaluate The Prophylactic Use Of NASA-Developed Light Emitting Diodes For The Prevention Of Oral Mucositis In Bone Marrow Transplant Patients Unknown status NCT00036712 Phase 2
32 Radiation Therapy in Combination With Indinavir / Ritonavir (Crixivan / Norvir) for the Treatment of Brain Metastases: a Randomized Phase II Study Unknown status NCT00637637 Phase 2 indinavir sulfate;ritonavir
33 A Phase II Study of Topotecan in Children With Recurrent Wilms Tumor Completed NCT00187031 Phase 2 Topotecan, Filgrastim (G-CSF), Pegfilgrastim
34 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
35 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
36 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
37 Phase II Study of Arsenic Trioxide in Neuroblastoma and Other Pediatric Solid Tumors Completed NCT00024258 Phase 2 arsenic trioxide
38 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
39 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
40 Myeloablative Chemotherapy With Stem Cell Rescue for Rare Poor-Prognosis Cancers Completed NCT00141765 Phase 2
41 A Pilot Study Investigating the Effects of Glutamine and Vincristine-Induced Neuropathy in Pediatric Patients With Cancer Completed NCT00365768 Phase 2 Glutamine
42 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
43 A Phase I/II Trial of Temodar in Pediatric Patients and Young Adults With High-Risk or Recurrent Solid Tumors Completed NCT00005952 Phase 1, Phase 2 temozolomide
44 Treatment of High Risk Renal Tumors: A Groupwide Phase II Study Completed NCT00335556 Phase 2 Doxorubicin Hydrochloride;Irinotecan Hydrochloride;Cyclophosphamide;Etoposide;Carboplatin;Vincristine Sulfate
45 Myeloablative Chemotherapy With Bone Marrow Rescue For Rare Poor-Prognosis Cancers Completed NCT00002515 Phase 2 carboplatin;thiotepa;topotecan hydrochloride
46 A Phase I/IIA Dose-Escalating Trial of BCL-2 Antisense (G3139) Treatment for Patients With Androgen-Independent Prostate Cancer or Other Advanced Solid Tumor Malignancies Completed NCT00003103 Phase 1, Phase 2 docetaxel
47 Randomized Phase II Study of Adjuvant WT-1 Analog Peptide Vaccine in Patients With Malignant Pleural Mesothelioma (MPM) After Completion of Combined Modality Therapy Completed NCT01265433 Phase 2
48 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
49 Phase II Liposomal Vincristine for Pediatric and Adolescent Patients With Relapsed Malignancies Completed NCT00038207 Phase 2 Liposomal Vincristine
50 Phase I/II Study of Adoptive Immunotherapy With CD8+ WT1-Specific CTL Clones for Patients With Advanced MDS, CML, AML or ALL After Allogeneic Hematopoietic Stem Cell Transplant Completed NCT00052520 Phase 1, Phase 2

Search NIH Clinical Center for Wilms Tumor 5

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Genetic Tests for Wilms Tumor 5

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Genetic tests related to Wilms Tumor 5:

# Genetic test Affiliating Genes
1 Wilms Tumor 5 29 POU6F2
2 Wilms Tumor Susceptibility-5 29
Sources

Anatomical Context for Wilms Tumor 5

About this section

MalaCards organs/tissues related to Wilms Tumor 5:

40
Kidney, Myeloid, T Cells, Lung, Bone, Bone Marrow, Breast
Sources

Publications for Wilms Tumor 5

About this section

Articles related to Wilms Tumor 5:

(show top 50) (show all 7261)
# Title Authors PMID Year
1
Germline mutations of the POU6F2 gene in Wilms tumors with loss of heterozygosity on chromosome 7p14. 56 6 61
15459955 2004
2
Refinement within single yeast artificial chromosome clones of a minimal region commonly deleted on the short arm of chromosome 7 in Wilms tumours. 56 6
11284034 2001
3
Localization of a novel t(1;7) translocation associated with Wilms' tumor predisposition and skeletal abnormalities. 61 56
8946193 1996
4
Germline and somatic abnormalities of chromosome 7 in Wilms' tumor. 56 61
7954327 1994
5
Wilms Tumor of the Ovary: Review of the Literature and Report of 2 Cases. 61 42
31815892 2020
6
Intra-tumor genetic heterogeneity in Wilms tumor samples. 61 42
31994632 2019
7
The parathyroid hormone-responsive B1 gene is interrupted by a t(1;7)(q42;p15) breakpoint associated with Wilms' tumour. 56
12618763 2003
8
Loss of heterozygosity for the short arm of chromosome 7 in sporadic Wilms tumour. 56
9690521 1998
9
Mapping of a putative tumor suppressor locus to proximal 7p in Wilms tumors. 56
8938443 1996
10
Paediatric metanephric tumours: a clinicopathological and molecular characterisation. 42
32371339 2020
11
Initial testing of a monoclonal antibody (IMC-A12) against IGF-1R by the Pediatric Preclinical Testing Program. 54 61
20166202 2010
12
Prognostic implications of mutations and expression of the Wilms tumor 1 (WT1) gene in adult acute T-lymphoblastic leukemia. 61 54
20435628 2010
13
Wilms tumor suppressor 1 (WT1) and early growth response 1 (EGR1) are regulators of STIM1 expression. 54 61
20123987 2010
14
Subtype-specific FBXW7 mutation and MYCN copy number gain in Wilms' tumor. 61 54
20332316 2010
15
The Wilms' tumor suppressor WT1 induces estrogen-independent growth and anti-estrogen insensitivity in ER-positive breast cancer MCF7 cells. 61 54
20204298 2010
16
Simultaneous detection of MDR1 and WT1 gene expression to predict the prognosis of adult acute lymphoblastic leukemia. 54 61
20423567 2010
17
Conditionally immortalized human podocyte cell lines established from urine. 54 61
19955187 2010
18
Integrative genomic analyses on interferon-lambdas and their roles in cancer prediction. 54 61
20043142 2010
19
Nuclear accumulation of beta-catenin protein indicates activation of wnt signaling in chemically induced rat nephroblastomas. 61 54
19348510 2010
20
High frequency of loss of allelic integrity at Wilms' tumor suppressor gene-1 locus in advanced breast tumors associated with aggressiveness of the tumor. 61 54
19749460 2009
21
Peptide vaccines for patients with acute myeloid leukemia. 61 54
19803762 2009
22
WNT/beta-catenin pathway activation in Wilms tumors: a unifying mechanism with multiple entries? 61 54
19530245 2009
23
Myocardial adenomatoid tumor in eight cattle: evidence for mesothelial origin of bovine myocardial epithelial inclusions. 61 54
19430001 2009
24
Nuclear degradation of Wilms tumor 1-associating protein and survivin splice variant switching underlie IGF-1-mediated survival. 54 61
19605357 2009
25
Expression of stem cell markers in the human fetal kidney. 61 54
19696931 2009
26
Paratesticular müllerian-type papillary serous tumor in a child. 54 61
19348511 2009
27
A novel WT1 mutation in a 46,XY boy with congenital bilateral cryptorchidism, nystagmus and Wilms tumor. 54 61
19048299 2009
28
Calcifying nested stromal-epithelial tumors of the liver: a clinicopathologic, immunohistochemical, and molecular genetic study of 9 cases with a long-term follow-up. 54 61
19363442 2009
29
Detection and treatment of molecular relapse in acute myeloid leukemia with RUNX1 (AML1), CBFB, or MLL gene translocations: frequent quantitative monitoring of molecular markers in different compartments and correlation with WT1 gene expression. 54 61
19463768 2009
30
Serous adenocarcinoma of the retroperitoneum, as a type of multifocal müllerian carcinoma. 54 61
19593619 2009
31
The tumor suppressor WTX shuttles to the nucleus and modulates WT1 activity. 61 54
19416806 2009
32
Identification of an immunogenic HLA-A*0201-binding T-cell epitope of the transcription factor PAX2. 61 54
19342968 2009
33
TTF-1 expression in nephroblastoma. 61 54
19011567 2009
34
Radiosensitization by inhibiting STAT1 in renal cell carcinoma. 54 61
19100922 2009
35
WNT5A is regulated by PAX2 and may be involved in blastemal predominant Wilms tumorigenesis. 61 54
19048125 2008
36
Perilobar nephrogenic rests are nonobligate molecular genetic precursor lesions of insulin-like growth factor-II-associated Wilms tumors. 54 61
19047088 2008
37
Wilms' tumor gene 1 (WT1) in endometrial carcinoma. 61 54
18929401 2008
38
Regulation of CRABP-II expression by MycN in Wilms tumor. 61 54
18955045 2008
39
Expression of pax8 as a useful marker in distinguishing ovarian carcinomas from mammary carcinomas. 61 54
18724243 2008
40
Induction of antiproliferative connective tissue growth factor expression in Wilms' tumor cells by sphingosine-1-phosphate receptor 2. 54 61
18922980 2008
41
Clinical relevance of mutations in the Wilms tumor suppressor 1 gene WT1 and the cadherin-associated protein beta1 gene CTNNB1 for patients with Wilms tumors: results of long-term surveillance of 71 patients from International Society of Pediatric Oncology Study 9/Society for Pediatric Oncology. 61 54
18618575 2008
42
Low prevalence of NPHS2 mutations in African American children with steroid-resistant nephrotic syndrome. 54 61
18543005 2008
43
Brain-derived neurotrophic factor and obesity in the WAGR syndrome. 54 61
18753648 2008
44
Wilms' tumor protein (-KTS) modulates renin gene transcription. 54 61
18496514 2008
45
The Wilms' tumor suppressor WT1 inhibits malignant progression of neoplastigenic mammary epithelial cells. 54 61
18751389 2008
46
TSA downregulates Wilms tumor gene 1 (Wt1) expression at multiple levels. 61 54
18535006 2008
47
Ectopic Pax2 expression in chick ventral optic cup phenocopies loss of Pax2 expression. 61 54
18485342 2008
48
Expression of multidrug resistance-related protein (MRP-1), lung resistance-related protein (LRP) and topoisomerase-II (TOPO-II) in Wilms' tumor: immunohistochemical study using TMA methodology. 54 61
18795074 2008
49
Wilms tumor genetics: mutations in WT1, WTX, and CTNNB1 account for only about one-third of tumors. 61 54
18311776 2008
50
In silico regulatory analysis for exploring human disease progression. 61 54
18564415 2008
Sources

Variations for Wilms Tumor 5

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ClinVar genetic disease variations for Wilms Tumor 5:

6 (show all 41) ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 CTNNB1 NM_001904.4(CTNNB1):c.770C>T (p.Thr257Ile)SNV other 438758 rs1553630452 3:41267186-41267186 3:41225695-41225695
2 TET2 NM_001127208.2(TET2):c.4456T>C (p.Ser1486Pro)SNV other 438791 rs1553918194 4:106193994-106193994 4:105272837-105272837
3 MED12 NM_005120.3(MED12):c.131G>A (p.Gly44Asp)SNV other 92220 rs199469672 X:70339254-70339254 X:71119404-71119404
4 POU6F2 NM_007252.4(POU6F2):c.573G>T (p.Gln191His)SNV Pathogenic 1871 rs121918261 7:39379302-39379302 7:39339703-39339703
5 POU6F2 POU6F2, C-G, EXON 1C, 5-PRIME UTRSNV Pathogenic 1872
6 FZD6 NM_003506.4(FZD6):c.346C>T (p.Arg116Ter)SNV Pathogenic 438765 rs769116796 8:104330986-104330986 8:103318758-103318758
7 CTNNB1 NM_001904.4(CTNNB1):c.133_135del (p.Ser45del)deletion Pathogenic 17576 rs587776850 3:41266136-41266138 3:41224643-41224645
8 CTNNB1 NM_001904.4(CTNNB1):c.134C>T (p.Ser45Phe)SNV Pathogenic/Likely pathogenic 17588 rs121913409 3:41266137-41266137 3:41224646-41224646
9 VHL NM_000551.4(VHL):c.241C>T (p.Pro81Ser)SNV Conflicting interpretations of pathogenicity 2233 rs104893829 3:10183772-10183772 3:10142088-10142088
10 MSH2 NM_000251.2(MSH2):c.2321T>C (p.Ile774Thr)SNV Uncertain significance 408514 rs878853811 2:47705521-47705521 2:47478382-47478382
11 WT1 NM_024426.6(WT1):c.411_413GCC[5] (p.Pro141dup)short repeat Uncertain significance 406690 rs760304811 11:32456484-32456485 11:32434938-32434939
12 SMAD4 NM_005359.6(SMAD4):c.875C>T (p.Pro292Leu)SNV Uncertain significance 184326 rs786201404 18:48584797-48584797 18:51058427-51058427
13 WT1 NM_024426.6(WT1):c.*1058G>TSNV Uncertain significance 304376 rs886048211 11:32409546-32409546 11:32388000-32388000
14 WT1 NM_024426.6(WT1):c.*1021_*1022GT[18]short repeat Uncertain significance 304380 rs58549495 11:32409549-32409550 11:32388003-32388004
15 WT1 NM_024426.6(WT1):c.*685G>TSNV Uncertain significance 304395 rs886048218 11:32409919-32409919 11:32388373-32388373
16 WT1 NM_024426.6(WT1):c.-82A>CSNV Uncertain significance 304433 rs886048236 11:32456988-32456988 11:32435442-32435442
17 WT1 NM_024426.6(WT1):c.*1021_*1022GT[14]short repeat Uncertain significance 304381 rs58549495 11:32409550-32409555 11:32388004-32388009
18 WT1 NM_024426.6(WT1):c.*1021_*1022GT[20]short repeat Uncertain significance 304378 rs58549495 11:32409549-32409550 11:32388003-32388004
19 WT1 NM_024426.6(WT1):c.*768A>CSNV Uncertain significance 304392 rs886048215 11:32409836-32409836 11:32388290-32388290
20 WT1 NM_024426.6(WT1):c.*841C>ASNV Uncertain significance 304388 rs886048213 11:32409763-32409763 11:32388217-32388217
21 WT1 NM_024426.6(WT1):c.*707C>ASNV Uncertain significance 304394 rs886048217 11:32409897-32409897 11:32388351-32388351
22 WT1 NM_024426.6(WT1):c.*574deldeletion Uncertain significance 304400 rs5030319 11:32410030-32410030 11:32388484-32388484
23 WT1 NM_024426.6(WT1):c.*393G>TSNV Uncertain significance 304405 rs886048222 11:32410211-32410211 11:32388665-32388665
24 WT1 NM_024426.6(WT1):c.247G>T (p.Ala83Ser)SNV Uncertain significance 304427 rs886048231 11:32456660-32456660 11:32435114-32435114
25 WT1 NM_024426.6(WT1):c.136G>T (p.Ala46Ser)SNV Uncertain significance 304430 rs886048233 11:32456771-32456771 11:32435225-32435225
26 WT1 NM_024426.6(WT1):c.-86T>CSNV Uncertain significance 304434 rs886048237 11:32456992-32456992 11:32435446-32435446
27 WT1 NM_024426.6(WT1):c.-94G>CSNV Uncertain significance 304436 rs886048239 11:32457000-32457000 11:32435454-32435454
28 WT1 NM_024426.6(WT1):c.*1021_*1022GT[15]short repeat Uncertain significance 304379 rs58549495 11:32409550-32409553 11:32388004-32388007
29 WT1 NM_024426.6(WT1):c.*1021_*1022GT[19]short repeat Uncertain significance 304377 rs58549495 11:32409549-32409550 11:32388003-32388004
30 WT1 NM_024426.6(WT1):c.*741G>TSNV Uncertain significance 304393 rs886048216 11:32409863-32409863 11:32388317-32388317
31 WT1 NM_024426.6(WT1):c.*366C>ASNV Uncertain significance 304406 rs886048223 11:32410238-32410238 11:32388692-32388692
32 WT1 NM_024426.6(WT1):c.*354G>TSNV Uncertain significance 304407 rs886048224 11:32410250-32410250 11:32388704-32388704
33 WT1 NM_024426.6(WT1):c.*269G>TSNV Uncertain significance 304409 rs886048225 11:32410335-32410335 11:32388789-32388789
34 WT1 NM_024426.6(WT1):c.-90T>CSNV Uncertain significance 304435 rs886048238 11:32456996-32456996 11:32435450-32435450
35 WT1 NM_024426.6(WT1):c.285C>T (p.Gly95=)SNV Uncertain significance 304426 rs886048230 11:32456622-32456622 11:32435076-32435076
36 WT1 NM_024426.6(WT1):c.-31G>TSNV Uncertain significance 304431 rs886048234 11:32456937-32456937 11:32435391-32435391
37 WT1 NM_024426.6(WT1):c.*542G>ASNV Likely benign 304401 rs142726499 11:32410062-32410062 11:32388516-32388516
38 WT1 NM_024426.6(WT1):c.*138G>ASNV Likely benign 304414 rs111351882 11:32410466-32410466 11:32388920-32388920
39 WT1 NM_024426.6(WT1):c.*897G>CSNV Likely benign 304385 rs5030324 11:32409707-32409707 11:32388161-32388161
40 WT1 NM_024426.6(WT1):c.*1049deldeletion Benign 304382 rs568803808 11:32409555-32409555 11:32388009-32388009
41 WT1 NM_024426.6(WT1):c.*978dupduplication Benign 304383 rs5030326 11:32409625-32409626 11:32388079-32388080

UniProtKB/Swiss-Prot genetic disease variations for Wilms Tumor 5:

73
# Symbol AA change Variation ID SNP ID
1 POU6F2 p.Gln192His VAR_022419

Sources

Expression for Wilms Tumor 5

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Search GEO for disease gene expression data for Wilms Tumor 5.
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Pathways for Wilms Tumor 5

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Pathways related to Wilms Tumor 5 according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Proteoglycans in cancer 36
11.57 IGF2 GPC3 FZD6 CTNNB1
2
Glioblastoma Multiforme 63
11.48 IGF2 FZD6 CTNNB1
3
Primary Focal Segmental Glomerulosclerosis FSGS 1
10.79 WT1 PAX2 CTNNB1 CDKN1C
4
ID signaling pathway 1
10.36 PAX8 PAX2
Sources

GO Terms for Wilms Tumor 5

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Biological processes related to Wilms Tumor 5 according to GeneCards Suite gene sharing:

(show all 36)
# Name GO ID Score Top Affiliating Genes
1 multicellular organism development GO:0007275 10.18 WTAP POU6F2 PAX8 PAX6 PAX2 IGF2
2 cell differentiation GO:0030154 10.14 POU6F2 PAX8 PAX6 PAX2 NAP1L4 CTNNB1
3 negative regulation of cell death GO:0060548 9.82 CCN5 CCN3 CCN1
4 negative regulation of epithelial cell proliferation GO:0050680 9.81 PAX6 GPC3 CDKN1C
5 positive regulation of transcription by RNA polymerase II GO:0045944 9.81 WT1 PRDM2 POU6F2 PAX8 PAX6 PAX2
6 kidney development GO:0001822 9.8 WT1 PAX8 GPC3 CTNNB1
7 positive regulation of transcription, DNA-templated GO:0045893 9.8 WT1 PRDM2 PAX8 PAX6 PAX2 CTNNB1
8 camera-type eye development GO:0043010 9.79 WT1 PAX6 PAX2
9 inner ear morphogenesis GO:0042472 9.78 PAX8 PAX2 FZD6
10 positive regulation of branching involved in ureteric bud morphogenesis GO:0090190 9.67 PAX8 PAX2
11 male genitalia development GO:0030539 9.67 WT1 CTNNB1
12 anterior/posterior axis specification GO:0009948 9.66 GPC3 CTNNB1
13 urogenital system development GO:0001655 9.65 PAX8 PAX2
14 mesonephros development GO:0001823 9.65 PAX8 PAX2
15 mesenchymal to epithelial transition GO:0060231 9.64 WT1 PAX2
16 metanephric mesenchyme development GO:0072075 9.63 WT1 PAX2
17 cellular response to gonadotropin stimulus GO:0071371 9.62 WT1 PAX8
18 dorsal/ventral axis specification GO:0009950 9.62 PAX6 CTNNB1
19 positive regulation of core promoter binding GO:1904798 9.6 PAX6 CTNNB1
20 mesenchymal to epithelial transition involved in metanephros morphogenesis GO:0003337 9.59 PAX8 PAX2
21 regulation of metanephric nephron tubule epithelial cell differentiation GO:0072307 9.58 PAX8 PAX2
22 pronephros development GO:0048793 9.58 PAX8 PAX2
23 metanephric S-shaped body morphogenesis GO:0072284 9.57 WT1 PAX8
24 positive regulation of epithelial cell differentiation GO:0030858 9.56 PAX6 CTNNB1
25 metanephric nephron tubule formation GO:0072289 9.55 PAX8 PAX2
26 metanephric distal convoluted tubule development GO:0072221 9.54 PAX8 PAX2
27 positive regulation of mesenchymal to epithelial transition involved in metanephros morphogenesis GO:0072108 9.52 PAX8 PAX2
28 negative regulation of mesenchymal cell apoptotic process involved in metanephros development GO:1900212 9.51 PAX8 PAX2
29 cell fate determination GO:0001709 9.5 PAX6 PAX2 CTNNB1
30 positive regulation of metanephric DCT cell differentiation GO:2000594 9.48 PAX8 PAX2
31 negative regulation of apoptotic process involved in metanephric nephron tubule development GO:1900218 9.46 PAX8 PAX2
32 negative regulation of apoptotic process involved in metanephric collecting duct development GO:1900215 9.43 PAX8 PAX2
33 negative regulation of mesenchymal cell apoptotic process involved in metanephric nephron morphogenesis GO:0072305 9.4 PAX8 PAX2
34 pronephric field specification GO:0039003 9.37 PAX8 PAX2
35 metanephric epithelium development GO:0072207 9.13 WT1 PAX8 PAX2
36 branching involved in ureteric bud morphogenesis GO:0001658 9.02 WT1 PAX8 PAX2 GPC3 CTNNB1

Molecular functions related to Wilms Tumor 5 according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 DNA-binding transcription factor activity, RNA polymerase II-specific GO:0000981 9.85 WT1 PRDM2 POU6F2 PAX8 PAX6 PAX2
2 DNA-binding transcription factor activity GO:0003700 9.7 WT1 PRDM2 POU6F2 PAX8 PAX6 PAX2
3 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.65 WT1 PRDM2 POU6F2 PAX8 PAX6
4 transcription regulatory region DNA binding GO:0044212 9.62 WT1 PAX8 PAX6 PAX2
5 integrin binding GO:0005178 9.26 IGF2 CCN5 CCN3 CCN1
6 insulin-like growth factor binding GO:0005520 8.8 CCN5 CCN3 CCN1
Sources

Sources for Wilms Tumor 5

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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