Details of Disease

Disease Name

Childhood kidney Wilms tumor

kidney Wilms tumor; Wilms tumor; Wilms tumour; kidney Wilms tumour; childhood kidney Wilms tumor; childhood renal Wilms' tumor; childhood renal Wilms tumour; childhood renal Wilms' tumour; childhood Wilms tumour; childhood renal Wilms tumor; childhood Wilms tumor

Definition

A Wilms tumor of the kidney which occurs in children.

Disease Hierarchy

DIS7WJYB: Kidney Wilms tumor

DISMAKWG: Childhood malignant kidney neoplasm

DIS0NMK3: Childhood kidney Wilms tumor

Disease Identifiers

MONDO ID

MONDO_0024676

UMLS CUI

C1333015

MedGen ID

232073

General Information of Disease (ID: DIS0NMK3)

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 4 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Dactinomycin	DM2YGNW	Approved	Small molecular drug	[1]
Doxorubicin	DMVP5YE	Approved	Small molecular drug	[2]
Etoposide	DMNH3PG	Approved	Small molecular drug	[3]
Vincristine	DMINOX3	Approved	Small molecular drug	[4]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 23 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
FSHB	TT13GFV	Limited	Biomarker	[5]
FZD7	TTUQMO5	Limited	Biomarker	[6]
LMO2	TTFX379	Limited	Biomarker	[7]
S1PR2	TTVSMOH	Limited	Biomarker	[8]
SLC38A2	TTUSC27	Limited	Biomarker	[9]
TBXA2R	TT2O84V	Limited	Altered Expression	[10]
TRIM37	TTAMCSL	Limited	Genetic Variation	[11]
MKNK1	TTEZAUX	Disputed	Biomarker	[12]
SRPK1	TTU3WV6	Disputed	Biomarker	[13]
TRIM28	TTQ2BKV	Disputed	Biomarker	[14]
LIN28A	TTO50LN	moderate	Altered Expression	[15]
BMP7	TTKOBRA	Strong	Biomarker	[16]
E2F3	TTWIJYH	Strong	Altered Expression	[17]
FLT3	TTGJCWZ	Strong	Genetic Variation	[18]
NES	TTHZ752	Strong	Altered Expression	[19]
NPM1	TTHBS98	Strong	Genetic Variation	[18]
PDGFA	TTSM78N	Strong	Biomarker	[20]
RECK	TTRZBW7	Strong	Posttranslational Modification	[21]
VHL	TTEMWSD	Strong	Biomarker	[22]
WT1	TTZ8UT4	Strong	Biomarker	[23]
CDKN1C	TTBSUAR	Definitive	Altered Expression	[24]
MDK	TTV8UE7	Definitive	Biomarker	[25]
NCAM1	TTVXPHT	Definitive	Biomarker	[26]
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⏷ Show the Full List of 23 DTT(s)

This Disease Is Related to 79 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
AATF	OT1QOKLD	Limited	Altered Expression	[27]
BASP1	OTF4VS5G	Limited	Altered Expression	[28]
BLCAP	OTGJVU0C	Limited	Altered Expression	[29]
CDC73	OT6JASZ1	Limited	Genetic Variation	[30]
CIB2	OT9ZJX1I	Limited	Altered Expression	[31]
CRABP2	OTY01V9G	Limited	Altered Expression	[32]
CTNNA1	OTFC725Z	Limited	Genetic Variation	[33]
CTNNA3	OT9Z0P1E	Limited	Genetic Variation	[33]
CTR9	OTP151PZ	Limited	Genetic Variation	[34]
ELP4	OTP5GZ9V	Limited	Genetic Variation	[5]
EYA1	OTHU807A	Limited	Altered Expression	[35]
FRS2	OTDMD800	Limited	Biomarker	[36]
FZD1	OTZATHVS	Limited	Biomarker	[6]
FZD5	OTXFFY56	Limited	Biomarker	[6]
HACE1	OTEZULKD	Limited	Genetic Variation	[37]
HNRNPL	OT0DJX74	Limited	Biomarker	[38]
INTS2	OT2N5TCK	Limited	Biomarker	[39]
LUZP2	OTGGOSPW	Limited	Biomarker	[40]
MSX1	OT5U41ZP	Limited	Biomarker	[41]
NAP1L4	OTUEG3QY	Limited	Biomarker	[42]
NCKIPSD	OT24UORN	Limited	Biomarker	[43]
NDC80	OTS7D306	Limited	Biomarker	[44]
NNAT	OTNRLO7G	Limited	Altered Expression	[29]
NPHS1	OT21JD3P	Limited	Altered Expression	[45]
OSR1	OTB19LEQ	Limited	Altered Expression	[46]
PHF2	OTJCIQR2	Limited	Biomarker	[47]
PHLDA1	OTFTWMIQ	Limited	Altered Expression	[24]
PHLDA2	OTMV9DPP	Limited	Altered Expression	[24]
PHOX2B	OT3SFR2O	Limited	Genetic Variation	[48]
PIAS4	OTB7SVMZ	Limited	Biomarker	[49]
PKHD1	OTAH8SMF	Limited	Genetic Variation	[50]
PLCG2	OTGVC9MY	Limited	Altered Expression	[51]
PPP2R1A	OTYA3GB4	Limited	Genetic Variation	[52]
PRM1	OT6HWA11	Limited	Biomarker	[10]
QPRT	OT6SKKLL	Limited	Biomarker	[53]
RASSF7	OT0V4EIZ	Limited	Biomarker	[54]
RBP2	OTR8QG5V	Limited	Altered Expression	[32]
RNF40	OTC8SDA3	Limited	Biomarker	[55]
RPL10	OTBHOZGC	Limited	Biomarker	[56]
SALL1	OTYYZGLH	Limited	Altered Expression	[57]
SALL2	OTQWI68Q	Limited	Altered Expression	[58]
SAT2	OT28QL7H	Limited	Biomarker	[9]
SIX3	OTP5E3VU	Limited	Biomarker	[49]
SMR3B	OTL5HNM8	Limited	Biomarker	[59]
SOSTDC1	OTAKDNSM	Limited	Genetic Variation	[60]
TES	OTL8PP6V	Limited	Biomarker	[61]
TIMP4	OT8A68SW	Limited	Biomarker	[62]
ATG4B	OTQ4RNRM	Disputed	Altered Expression	[63]
BRINP1	OTEUVSCP	Disputed	Genetic Variation	[64]
CD151	OTF3UZS7	Disputed	Biomarker	[65]
FXR1	OTEMQ1SR	Disputed	Biomarker	[66]
NKX6-1	OT5QC0BF	Disputed	Genetic Variation	[64]
NPHP1	OTZHCFFQ	Disputed	Genetic Variation	[67]
SYCP3	OTKOF54H	Disputed	Altered Expression	[68]
BAALC	OTUZSRVF	Strong	Genetic Variation	[69]
CITED1	OTUJQ3VL	Strong	Biomarker	[19]
CTCF	OT8ZB70U	Strong	Altered Expression	[29]
DGCR8	OT62LXE4	Strong	Biomarker	[70]
DIS3L2	OTWABM04	Strong	Biomarker	[71]
DROSHA	OTCE68KZ	Strong	Genetic Variation	[70]
EWSR1	OT7SRHV3	Strong	Biomarker	[72]
FIBP	OTI7131S	Strong	Biomarker	[73]
LIN28B	OTVWP0FN	Strong	Genetic Variation	[15]
LMO1	OTB59SKB	Strong	Genetic Variation	[74]
NPHS2	OTLCNUII	Strong	Altered Expression	[75]
NR5A1	OTOULYR4	Strong	Biomarker	[76]
PODXL	OTPNQXF3	Strong	Biomarker	[77]
POU6F2	OTNSW95F	Strong	Biomarker	[78]
RBBP7	OTLB56HX	Strong	Altered Expression	[79]
RUNX1T1	OT30DED5	Strong	Biomarker	[80]
SIX2	OTYOVGSC	Strong	Biomarker	[81]
SLC22A18	OT9C3KR4	Strong	Genetic Variation	[82]
SRY	OT516T6D	Strong	Genetic Variation	[83]
STIM1	OT8CLQ1W	Strong	Altered Expression	[84]
TRIP13	OTFM3TI9	Strong	Genetic Variation	[85]
EMG1	OTFDX7HY	Definitive	Biomarker	[86]
PAX8	OTRPD9MI	Definitive	Altered Expression	[87]
PRDM2	OT8L7CGX	Definitive	Biomarker	[88]
TBC1D9	OTSSCTB5	Definitive	Biomarker	[89]
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⏷ Show the Full List of 79 DOT(s)

References

1	Dactinomycin FDA Label
2	Doxorubicin FDA Label
3	Etoposide FDA Label
4	Vincristine FDA Label
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7	LMO2 gene deletions significantly worsen the prognosis of Wilms' tumor development in patients with WAGR syndrome.Hum Mol Genet. 2019 Oct 1;28(19):3323-3326. doi: 10.1093/hmg/ddz168.
8	Induction of antiproliferative connective tissue growth factor expression in Wilms' tumor cells by sphingosine-1-phosphate receptor 2.Mol Cancer Res. 2008 Oct;6(10):1649-56. doi: 10.1158/1541-7786.MCR-07-2048.
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32	Regulation of CRABP-II expression by MycN in Wilms tumor.Exp Cell Res. 2008 Dec 10;314(20):3663-8. doi: 10.1016/j.yexcr.2008.09.029. Epub 2008 Oct 14.
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34	Identification of a novel CTR9 germline mutation in a family with Wilms tumor.Eur J Med Genet. 2018 May;61(5):294-299. doi: 10.1016/j.ejmg.2017.12.010. Epub 2017 Dec 29.
35	Loss of heterozygosity at 2q37 in sporadic Wilms' tumor: putative role for miR-562.Clin Cancer Res. 2009 Oct 1;15(19):5985-92. doi: 10.1158/1078-0432.CCR-09-1065. Epub 2009 Sep 29.
36	MicroRNA-613 attenuates the proliferation, migration and invasion of Wilms' tumor via targeting FRS2.Eur Rev Med Pharmacol Sci. 2017 Aug;21(15):3360-3369.
37	Association Between HACE1 Gene Polymorphisms and Wilms' Tumor Risk in a Chinese Population.Cancer Invest. 2017 Nov 26;35(10):633-638. doi: 10.1080/07357907.2017.1405016.
38	HnRNPL promotes Wilms tumor progression by regulating the p53 and Bcl2 pathways.Onco Targets Ther. 2019 May 29;12:4269-4279. doi: 10.2147/OTT.S203046. eCollection 2019.
39	11q13 allelic imbalance discriminates pulmonary carcinoids from tumorlets. A microdissection-based genotyping approach useful in clinical practice.Am J Pathol. 1999 Aug;155(2):633-40. doi: 10.1016/S0002-9440(10)65159-0.
40	Cloning, functional study and comparative mapping of Luzp2 to mouse chromosome 7 and human chromosome 11p13-11p14.Mamm Genome. 2003 May;14(5):323-34. doi: 10.1007/s00335-002-2248-6.
41	Expression profiling reveals MSX1 and EphB2 expression correlates with the invasion capacity of Wilms tumors.Pediatr Blood Cancer. 2011 Dec 1;57(6):950-7. doi: 10.1002/pbc.23003. Epub 2011 Mar 8.
42	Functional characterization of human nucleosome assembly protein-2 (NAP1L4) suggests a role as a histone chaperone.Genomics. 1997 Sep 15;44(3):253-65. doi: 10.1006/geno.1997.4868.
43	Multiple imprinted and stemness genes provide a link between normal and tumor progenitor cells of the developing human kidney.Cancer Res. 2006 Jun 15;66(12):6040-9. doi: 10.1158/0008-5472.CAN-05-4528.
44	Clinicopathological significance and prognostic value of Wilms' tumor gene expression in colorectal cancer.Cancer Biomark. 2015;15(6):789-97. doi: 10.3233/CBM-150521.
45	Nephrin loss in experimental diabetic nephropathy is prevented by deletion of protein kinase C alpha signaling in-vivo.Kidney Int. 2006 Oct;70(8):1456-62. doi: 10.1038/sj.ki.5001830. Epub 2006 Sep 6.
46	Clear cell sarcoma of the kidney demonstrates an embryonic signature indicative of a primitive nephrogenic origin.Genes Chromosomes Cancer. 2014 May;53(5):381-91. doi: 10.1002/gcc.22149. Epub 2014 Feb 1.
47	GRC5 and NMD3 function in translational control of gene expression and interact genetically.Curr Genet. 1999 Jan;34(6):419-29. doi: 10.1007/s002940050416.
48	Association between PHOX2B gene rs28647582 T>C polymorphism and Wilms tumor susceptibility.Biosci Rep. 2019 Oct 30;39(10):BSR20192529. doi: 10.1042/BSR20192529.
49	Array CGH Analysis of Paired Blood and Tumor Samples from Patients with Sporadic Wilms Tumor.PLoS One. 2015 Aug 28;10(8):e0136812. doi: 10.1371/journal.pone.0136812. eCollection 2015.
50	Concerted nonsyntenic allelic loss in human colorectal carcinoma.Science. 1988 Aug 19;241(4868):961-5. doi: 10.1126/science.2841761.
51	Molecular profiling of isolated histological components of wilms tumor implicates a common role for the Wnt signaling pathway in kidney and tumor development.Oncology. 2008;75(1-2):81-91. doi: 10.1159/000155210. Epub 2008 Sep 11.
52	Absence of PPP2R1A mutations in Wilms tumor.Oncogene. 2001 Apr 12;20(16):2050-4. doi: 10.1038/sj.onc.1204301.
53	Anti-apoptotic quinolinate phosphoribosyltransferase (QPRT) is a target gene of Wilms' tumor gene 1 (WT1) protein in leukemic cells.Biochem Biophys Res Commun. 2017 Jan 22;482(4):802-807. doi: 10.1016/j.bbrc.2016.11.114. Epub 2016 Nov 23.
54	HRAS1-selected chromosome transfer generates markers that colocalize aniridia- and genitourinary dysplasia-associated translocation breakpoints and the Wilms tumor gene within band 11p13.Proc Natl Acad Sci U S A. 1987 Aug;84(15):5355-9. doi: 10.1073/pnas.84.15.5355.
55	Induction of Rb-associated protein (RbAp46) by Wilms' tumor suppressor WT1 mediates growth inhibition.J Biol Chem. 1998 Oct 16;273(42):27047-50. doi: 10.1074/jbc.273.42.27047.
56	Biological Function of Ribosomal Protein L10 on Cell Behavior in Human Epithelial Ovarian Cancer.J Cancer. 2018 Feb 6;9(4):745-756. doi: 10.7150/jca.21614. eCollection 2018.
57	Hsal 1 is related to kidney and gonad development and is expressed in Wilms tumor.Pediatr Nephrol. 2001 Sep;16(9):701-9. doi: 10.1007/s004670100624.
58	Cloning and characterization of two promoters for the human HSAL2 gene and their transcriptional repression by the Wilms tumor suppressor gene product.J Biol Chem. 2001 Dec 21;276(51):48223-30. doi: 10.1074/jbc.M106468200. Epub 2001 Sep 27.
59	Expression of phosphatase of regenerating liver-3 is associated with prognosis of Wilms' tumor.Onco Targets Ther. 2017 Jan 10;10:311-317. doi: 10.2147/OTT.S107076. eCollection 2017.
60	Two candidate tumor suppressor genes, MEOX2 and SOSTDC1, identified in a 7p21 homozygous deletion region in a Wilms tumor.Genes Chromosomes Cancer. 2009 Dec;48(12):1037-50. doi: 10.1002/gcc.20705.
61	Wilms tumor suppressor 1 (WT1) and early growth response 1 (EGR1) are regulators of STIM1 expression.J Biol Chem. 2010 Apr 2;285(14):10591-6. doi: 10.1074/jbc.M109.083493. Epub 2010 Feb 1.
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63	Autophagy related markers (Beclin-1 and ATG4B) are strongly expressed in Wilms' tumor and correlate with favorable histology.Histol Histopathol. 2019 Jan;34(1):47-56. doi: 10.14670/HH-18-023. Epub 2018 Jul 10.
64	Methylation in the promoter regions of WT1, NKX6-1 and DBC1 genes in cervical cancer tissues of Uygur women in Xinjiang.Genet Mol Biol. 2018 Jan-Mar;41(1):9-17. doi: 10.1590/1678-4685-GMB-2016-0146.
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70	Loss or oncogenic mutation of DROSHA impairs kidney development and function, but is not sufficient for Wilms tumor formation.Int J Cancer. 2019 Mar 15;144(6):1391-1400. doi: 10.1002/ijc.31952. Epub 2018 Dec 3.
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73	A recessive syndrome of intellectual disability, moderate overgrowth, and renal dysplasia predisposing to Wilms tumor is caused by a mutation in FIBP gene. Am J Med Genet A. 2016 Aug;170(8):2111-8. doi: 10.1002/ajmg.a.37741. Epub 2016 May 17.
74	LMO1 Super-Enhancer rs2168101 G>T Polymorphism Reduces Wilms Tumor Risk.J Cancer. 2019 Apr 21;10(8):1808-1813. doi: 10.7150/jca.29842. eCollection 2019.
75	Amelioration of Diabetic Nephropathy Using a Retinoic Acid Receptor 2 Agonist.J Pharmacol Exp Ther. 2018 Oct;367(1):82-94. doi: 10.1124/jpet.118.249375. Epub 2018 Jul 27.
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77	Application of miR-193a/WT1/PODXL axis to estimate risk and prognosis of idiopathic membranous nephropathy.Ren Fail. 2019 Nov;41(1):704-717. doi: 10.1080/0886022X.2019.1642210.
78	The murine Pou6f2 gene is temporally and spatially regulated during kidney embryogenesis and its human homolog is overexpressed in a subset of Wilms tumors.J Pediatr Hematol Oncol. 2006 Dec;28(12):791-7. doi: 10.1097/MPH.0b013e31802d3e65.
79	Overexpression of RbAp46 facilitates stress-induced apoptosis and suppresses tumorigenicity of neoplastigenic breast epithelial cells. Int J Cancer. 2003 Jul 20;105(6):762-8. doi: 10.1002/ijc.11148.
80	Molecular monitoring of BAALC expression in patients with CD34-positive acute leukemia.Int J Hematol. 2010 May;91(4):636-45. doi: 10.1007/s12185-010-0550-8. Epub 2010 Apr 8.
81	Assessment of promoter methylation and expression of SIX2 as a diagnostic and prognostic biomarker in Wilms' tumor.Tumour Biol. 2015 Sep;36(10):7591-8. doi: 10.1007/s13277-015-3456-5. Epub 2015 Apr 29.
82	Somatic mutation of TSSC5, a novel imprinted gene from human chromosome 11p15.5.Cancer Res. 1998 Sep 15;58(18):4155-9.
83	Mutations in SRY and WT1 genes required for gonadal development are not responsible for XY partial gonadal dysgenesis.Braz J Med Biol Res. 2005 Jan;38(1):17-25. doi: 10.1590/s0100-879x2005000100004. Epub 2005 Jan 18.
84	WT1/EGR1-mediated control of STIM1 expression and function in cancer cells.Front Biosci (Landmark Ed). 2011 Jun 1;16(7):2402-15. doi: 10.2741/3862.
85	Absence of the TRIP13 c.1060C>T Mutation in Wilms Tumor Patients From Pakistan.J Pediatr Hematol Oncol. 2020 Apr;42(3):e128-e131. doi: 10.1097/MPH.0000000000001602.
86	Up-regulation of miR-190b promoted growth, invasion, migration and inhibited apoptosis of Wilms' tumor cells by repressing the PTEN expression.Eur Rev Med Pharmacol Sci. 2018 Feb;22(4):961-969. doi: 10.26355/eurrev_201802_14377.
87	Diagnostic Utility of Pax8, Pax2, and NGFR Immunohistochemical Expression in Pediatric Renal Tumors.Appl Immunohistochem Mol Morphol. 2018 Nov-Dec;26(10):721-726. doi: 10.1097/PAI.0000000000000520.
88	High levels of Wilms' tumor gene (wt1) mRNA in acute myeloid leukemias are associated with a worse long-term outcome.Blood. 1997 Aug 1;90(3):1217-25.
89	Simultaneous detection of MDR1 and WT1 gene expression to predict the prognosis of adult acute lymphoblastic leukemia.Hematology. 2010 Apr;15(2):74-80. doi: 10.1179/102453310X12583347009937.