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Wilms tumor 6(WT6)

MedGen UID:
855962
Concept ID:
C3891301
Neoplastic Process
Synonyms: WILMS TUMOR 6, SUSCEPTIBILITY TO; WT6
 
Gene (location): REST (4q12)
 
Monarch Initiative: MONDO:0014779
OMIM®: 616806

Definition

Wilms tumor is the most common renal tumor of childhood, occurring with an incidence of 1 in 10,000. It is often described as an embryonal tumor, as it arises from embryonal cells in which growth and/or differentiation have become dysregulated during development (summary by Mahamdallie et al., 2015). For a general phenotypic description and discussion of genetic heterogeneity of Wilms tumor, see WT1 (194070). [from OMIM]

Additional description

From MedlinePlus Genetics
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.

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 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.  https://medlineplus.gov/genetics/condition/wilms-tumor

Clinical features

From HPO
Nephroblastoma
MedGen UID:
10221
Concept ID:
C0027708
Neoplastic Process
The presence of a nephroblastoma, which is a neoplasm of the kidney that primarily affects children.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Chan PT, Palermo GD, Veeck LL, Rosenwaks Z, Schlegel PN
Cancer 2001 Sep 15;92(6):1632-7. doi: 10.1002/1097-0142(20010915)92:6<1632::aid-cncr1489>3.0.co;2-i. PMID: 11745242

Recent clinical studies

Etiology

Murphy AJ, Cheng C, Williams J, Shaw TI, Pinto EM, Dieseldorff-Jones K, Brzezinski J, Renfro LA, Tornwall B, Huff V, Hong AL, Mullen EA, Crompton B, Dome JS, Fernandez CV, Geller JI, Ehrlich PF, Mulder H, Oak N, Maciezsek J, Jablonowski CM, Fleming AM, Pichavaram P, Morton CL, Easton J, Nichols KE, Clay MR, Santiago T, Zhang J, Yang J, Zambetti GP, Wang Z, Davidoff AM, Chen X
Nat Commun 2023 Dec 18;14(1):8006. doi: 10.1038/s41467-023-43730-0. PMID: 38110397Free PMC Article
Prasad M, Vora T, Agarwala S, Laskar S, Arora B, Bansal D, Kapoor G, Chinnaswamy G, Radhakrishnan V, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 Jun;84(6):437-445. Epub 2017 Apr 3 doi: 10.1007/s12098-017-2305-5. PMID: 28367612
Pietras W
Adv Clin Exp Med 2012 Nov-Dec;21(6):809-820. PMID: 23457141
Davidoff AM
Curr Opin Pediatr 2009 Jun;21(3):357-64. doi: 10.1097/MOP.0b013e32832b323a. PMID: 19417665Free PMC Article
Haase GM, Ritchey ML
Semin Pediatr Surg 1997 Feb;6(1):11-6. PMID: 9117268

Diagnosis

Murphy AJ, Cheng C, Williams J, Shaw TI, Pinto EM, Dieseldorff-Jones K, Brzezinski J, Renfro LA, Tornwall B, Huff V, Hong AL, Mullen EA, Crompton B, Dome JS, Fernandez CV, Geller JI, Ehrlich PF, Mulder H, Oak N, Maciezsek J, Jablonowski CM, Fleming AM, Pichavaram P, Morton CL, Easton J, Nichols KE, Clay MR, Santiago T, Zhang J, Yang J, Zambetti GP, Wang Z, Davidoff AM, Chen X
Nat Commun 2023 Dec 18;14(1):8006. doi: 10.1038/s41467-023-43730-0. PMID: 38110397Free PMC Article
Cunningham ME, Klug TD, Nuchtern JG, Chintagumpala MM, Venkatramani R, Lubega J, Naik-Mathuria BJ
J Surg Res 2020 Mar;247:34-51. Epub 2019 Dec 3 doi: 10.1016/j.jss.2019.10.044. PMID: 31810638
Prasad M, Vora T, Agarwala S, Laskar S, Arora B, Bansal D, Kapoor G, Chinnaswamy G, Radhakrishnan V, Kaur T, Rath GK, Bakhshi S
Indian J Pediatr 2017 Jun;84(6):437-445. Epub 2017 Apr 3 doi: 10.1007/s12098-017-2305-5. PMID: 28367612
Davidoff AM
Curr Opin Pediatr 2009 Jun;21(3):357-64. doi: 10.1097/MOP.0b013e32832b323a. PMID: 19417665Free PMC Article
Haase GM, Ritchey ML
Semin Pediatr Surg 1997 Feb;6(1):11-6. PMID: 9117268

Therapy

Heck JE, He D, Janzen C, Federman N, Olsen J, Ritz B, Hansen J
Pediatr Blood Cancer 2019 Jan;66(1):e27461. Epub 2018 Sep 25 doi: 10.1002/pbc.27461. PMID: 30255546Free PMC Article
Dix DB, Seibel NL, Chi YY, Khanna G, Gratias E, Anderson JR, Mullen EA, Geller JI, Kalapurakal JA, Paulino AC, Perlman EJ, Ehrlich PF, Malogolowkin M, Gastier-Foster JM, Wagner E, Grundy PE, Fernandez CV, Dome JS
J Clin Oncol 2018 Jun 1;36(16):1564-1570. Epub 2018 Apr 16 doi: 10.1200/JCO.2017.77.1931. PMID: 29659330Free PMC Article
Ehrlich P, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Ritchey ML, Dome JS
Ann Surg 2017 Sep;266(3):470-478. doi: 10.1097/SLA.0000000000002356. PMID: 28795993Free PMC Article
Pietras W
Adv Clin Exp Med 2012 Nov-Dec;21(6):809-820. PMID: 23457141
Warrier RP, Regueira O
Pediatr Nephrol 1992 Jul;6(4):358-64. doi: 10.1007/BF00869738. PMID: 1323319

Prognosis

Cunningham ME, Klug TD, Nuchtern JG, Chintagumpala MM, Venkatramani R, Lubega J, Naik-Mathuria BJ
J Surg Res 2020 Mar;247:34-51. Epub 2019 Dec 3 doi: 10.1016/j.jss.2019.10.044. PMID: 31810638
Schmidt A, Warmann SW, Urla C, Schaefer J, Fideler F, Fuchs J
Surg Oncol 2019 Jun;29:14-19. Epub 2019 Feb 12 doi: 10.1016/j.suronc.2019.02.007. PMID: 31196478
Heck JE, He D, Janzen C, Federman N, Olsen J, Ritz B, Hansen J
Pediatr Blood Cancer 2019 Jan;66(1):e27461. Epub 2018 Sep 25 doi: 10.1002/pbc.27461. PMID: 30255546Free PMC Article
Dix DB, Seibel NL, Chi YY, Khanna G, Gratias E, Anderson JR, Mullen EA, Geller JI, Kalapurakal JA, Paulino AC, Perlman EJ, Ehrlich PF, Malogolowkin M, Gastier-Foster JM, Wagner E, Grundy PE, Fernandez CV, Dome JS
J Clin Oncol 2018 Jun 1;36(16):1564-1570. Epub 2018 Apr 16 doi: 10.1200/JCO.2017.77.1931. PMID: 29659330Free PMC Article
Davidoff AM
Curr Opin Pediatr 2009 Jun;21(3):357-64. doi: 10.1097/MOP.0b013e32832b323a. PMID: 19417665Free PMC Article

Clinical prediction guides

Wu NL, Chen Y, Dieffenbach BV, Ehrhardt MJ, Hingorani S, Howell RM, Jefferies JL, Mulrooney DA, Oeffinger KC, Robison LL, Weil BR, Yuan Y, Yasui Y, Hudson MM, Leisenring WM, Armstrong GT, Chow EJ
J Clin Oncol 2023 Apr 20;41(12):2258-2268. Epub 2023 Feb 16 doi: 10.1200/JCO.22.01926. PMID: 36795981Free PMC Article
Wang YQ, Wu DH, Wei D, Shen JY, Huang ZW, Liang XY, Cho WCS, Ma J, Lv J, Chen YP
Sci Adv 2023 Jan 6;9(1):eadd0960. doi: 10.1126/sciadv.add0960. PMID: 36608137Free PMC Article
Foster KL, Salehabadi SM, Green DM, Xing M, Ness KK, Krull KR, Brinkman TM, Ehrhardt MJ, Chemaitilly W, Dixon SB, Bhakta N, Brennan RC, Krasin MJ, Davidoff AM, Robison LL, Hudson MM, Mulrooney DA
Pediatrics 2022 Nov 1;150(5) doi: 10.1542/peds.2022-056918. PMID: 36300342Free PMC Article
Madsen PJ, Rahman RK, Patel YA, Lang SS, Storm PB, Tucker AM
Childs Nerv Syst 2022 Jun;38(6):1105-1111. Epub 2022 Apr 4 doi: 10.1007/s00381-022-05502-x. PMID: 35377009
Dix DB, Seibel NL, Chi YY, Khanna G, Gratias E, Anderson JR, Mullen EA, Geller JI, Kalapurakal JA, Paulino AC, Perlman EJ, Ehrlich PF, Malogolowkin M, Gastier-Foster JM, Wagner E, Grundy PE, Fernandez CV, Dome JS
J Clin Oncol 2018 Jun 1;36(16):1564-1570. Epub 2018 Apr 16 doi: 10.1200/JCO.2017.77.1931. PMID: 29659330Free PMC Article

Recent systematic reviews

Abdelhafeez AH, Reljic T, Kumar A, Banu T, Cox S, Davidoff AM, Elgendy A, Ghandour K, Gerstle JT, Karpelowsky J, Kaste SC, Kechiche N, Esiashvili N, Nasir A, Ngongola A, Marollano J, Moreno AA, Muzira A, Parkes J, Saldaña LJ, Shalkow J, Vujanić GM, Velasquez T, Lakhoo K, Mukkada S, Abib S
Pediatr Blood Cancer 2022 Dec;69(12):e29906. Epub 2022 Aug 5 doi: 10.1002/pbc.29906. PMID: 35929184
Doganis D, Katsimpris A, Panagopoulou P, Bouka P, Bouka E, Moschovi M, Polychronopoulou S, Papakonstantinou E, Tragiannidis A, Katzilakis N, Dana H, Antoniadi K, Stefanaki K, Strantzia K, Dessypris N, Schüz J, Petridou ET
Cancer Epidemiol 2020 Aug;67:101769. Epub 2020 Jul 10 doi: 10.1016/j.canep.2020.101769. PMID: 32659726
Han Q, Li K, Dong K, Xiao X, Yao W, Liu G
J Pediatr Surg 2018 Dec;53(12):2465-2469. Epub 2018 Sep 1 doi: 10.1016/j.jpedsurg.2018.08.022. PMID: 30274708
Vanden Berg RN, Bierman EN, Noord MV, Rice HE, Routh JC
Urol Oncol 2016 Jan;34(1):24-32. Epub 2015 Aug 5 doi: 10.1016/j.urolonc.2015.07.003. PMID: 26254695Free PMC Article
Gómez FM, Patel PA, Stuart S, Roebuck DJ
Pediatr Radiol 2014 Oct;44(10):1281-9. Epub 2014 May 13 doi: 10.1007/s00247-014-3001-5. PMID: 24821394

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