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Wilms tumor 2(WT2)

MedGen UID:
854562
Concept ID:
C3887743
Disease or Syndrome
Synonyms: Familial Wilms tumor 2; WT2
 
Gene (location): H19-ICR (11p15.5)
 
Monarch Initiative: MONDO:0008680
OMIM®: 194071

Definition

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).

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 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. [from MedlinePlus Genetics]

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.

Professional guidelines

PubMed

Oguz A, Karadeniz C, Citak EC, Conly NA, Ileri F, Boyunaga O, Okur V, Uluoglu O
Pediatr Hematol Oncol 2005 Jul-Aug;22(5):423-35. doi: 10.1080/08880010590964417. PMID: 16020133
Gow KW, Lensing S, Hill DA, Krasin MJ, McCarville MB, Rai SN, Zacher M, Spunt SL, Strickland DK, Hudson MM
J Pediatr Surg 2003 Nov;38(11):1574-80. doi: 10.1016/s0022-3468(03)00563-3. PMID: 14614703

Recent clinical studies

Etiology

Gazzin A, Carli D, Sirchia F, Molinatto C, Cardaropoli S, Palumbo G, Zampino G, Ferrero GB, Mussa A
Am J Med Genet A 2019 Sep;179(9):1691-1702. Epub 2019 Jul 24 doi: 10.1002/ajmg.a.61301. PMID: 31339634
Loh A, Bishop M, Krasin M, Davidoff AM, Langham MR Jr
J Pediatr Surg 2015 Apr;50(4):550-5. Epub 2014 Dec 2 doi: 10.1016/j.jpedsurg.2014.11.044. PMID: 25840061
Laiq Z, Bishop JA, Ali SZ
Diagn Cytopathol 2012 Jul;40(7):586-91. Epub 2011 Feb 9 doi: 10.1002/dc.21587. PMID: 21309009
Murphy JJ, Tawfeeq M, Chang B, Nadel H
J Pediatr Surg 2008 Dec;43(12):2186-92. doi: 10.1016/j.jpedsurg.2008.08.064. PMID: 19040932
Basta-Jovanovic G, Gvozdenovic E, Dimitrijevic I, Brasanac D, Jovanovic M, Kalezic N, Baralic I, Radojevic-Skodric S, Arsic D
Fetal Pediatr Pathol 2008;27(2):63-70. doi: 10.1080/15513810802028704. PMID: 18568994

Diagnosis

Danner-Koptik KE, Majhail NS, Brazauskas R, Wang Z, Buchbinder D, Cahn JY, Dilley KJ, Frangoul HA, Gross TG, Hale GA, Hayashi RJ, Hijiya N, Kamble RT, Lazarus HM, Marks DI, Reddy V, Savani BN, Warwick AB, Wingard JR, Wood WA, Sorror ML, Jacobsohn DA
Bone Marrow Transplant 2013 Mar;48(3):363-8. Epub 2012 Sep 10 doi: 10.1038/bmt.2012.166. PMID: 22964594Free PMC Article
Laiq Z, Bishop JA, Ali SZ
Diagn Cytopathol 2012 Jul;40(7):586-91. Epub 2011 Feb 9 doi: 10.1002/dc.21587. PMID: 21309009
Oguz A, Karadeniz C, Citak EC, Conly NA, Ileri F, Boyunaga O, Okur V, Uluoglu O
Pediatr Hematol Oncol 2005 Jul-Aug;22(5):423-35. doi: 10.1080/08880010590964417. PMID: 16020133
Ahmed R, al-Salti W, Raafat F, Morland B
Pediatr Hematol Oncol 2003 Jan-Feb;20(1):55-63. PMID: 12687754
Leisenring WM, Breslow NE, Evans IE, Beckwith JB, Coppes MJ, Grundy P
Cancer Res 1994 Sep 1;54(17):4680-3. PMID: 8062264

Therapy

Danner-Koptik KE, Majhail NS, Brazauskas R, Wang Z, Buchbinder D, Cahn JY, Dilley KJ, Frangoul HA, Gross TG, Hale GA, Hayashi RJ, Hijiya N, Kamble RT, Lazarus HM, Marks DI, Reddy V, Savani BN, Warwick AB, Wingard JR, Wood WA, Sorror ML, Jacobsohn DA
Bone Marrow Transplant 2013 Mar;48(3):363-8. Epub 2012 Sep 10 doi: 10.1038/bmt.2012.166. PMID: 22964594Free PMC Article
Murphy JJ, Tawfeeq M, Chang B, Nadel H
J Pediatr Surg 2008 Dec;43(12):2186-92. doi: 10.1016/j.jpedsurg.2008.08.064. PMID: 19040932
Ahmed R, al-Salti W, Raafat F, Morland B
Pediatr Hematol Oncol 2003 Jan-Feb;20(1):55-63. PMID: 12687754

Prognosis

Loh A, Bishop M, Krasin M, Davidoff AM, Langham MR Jr
J Pediatr Surg 2015 Apr;50(4):550-5. Epub 2014 Dec 2 doi: 10.1016/j.jpedsurg.2014.11.044. PMID: 25840061
Danner-Koptik KE, Majhail NS, Brazauskas R, Wang Z, Buchbinder D, Cahn JY, Dilley KJ, Frangoul HA, Gross TG, Hale GA, Hayashi RJ, Hijiya N, Kamble RT, Lazarus HM, Marks DI, Reddy V, Savani BN, Warwick AB, Wingard JR, Wood WA, Sorror ML, Jacobsohn DA
Bone Marrow Transplant 2013 Mar;48(3):363-8. Epub 2012 Sep 10 doi: 10.1038/bmt.2012.166. PMID: 22964594Free PMC Article
Basta-Jovanovic G, Gvozdenovic E, Dimitrijevic I, Brasanac D, Jovanovic M, Kalezic N, Baralic I, Radojevic-Skodric S, Arsic D
Fetal Pediatr Pathol 2008;27(2):63-70. doi: 10.1080/15513810802028704. PMID: 18568994
Arcellana-Panlilio MY, Egeler RM, Ujack E, Pinto A, Demetrick DJ, Robbins SM, Coppes MJ
Genes Chromosomes Cancer 2000 Sep;29(1):63-9. doi: 10.1002/1098-2264(2000)9999:9999<::aid-gcc1006>3.0.co;2-l. PMID: 10918395
Ford EG, Lockhart SK, Sullivan MP, Andrassy RJ
J Pediatr Surg 1987 Dec;22(12):1155-9. doi: 10.1016/s0022-3468(87)80727-3. PMID: 3440903

Clinical prediction guides

Gazzin A, Carli D, Sirchia F, Molinatto C, Cardaropoli S, Palumbo G, Zampino G, Ferrero GB, Mussa A
Am J Med Genet A 2019 Sep;179(9):1691-1702. Epub 2019 Jul 24 doi: 10.1002/ajmg.a.61301. PMID: 31339634
Loh A, Bishop M, Krasin M, Davidoff AM, Langham MR Jr
J Pediatr Surg 2015 Apr;50(4):550-5. Epub 2014 Dec 2 doi: 10.1016/j.jpedsurg.2014.11.044. PMID: 25840061
Danner-Koptik KE, Majhail NS, Brazauskas R, Wang Z, Buchbinder D, Cahn JY, Dilley KJ, Frangoul HA, Gross TG, Hale GA, Hayashi RJ, Hijiya N, Kamble RT, Lazarus HM, Marks DI, Reddy V, Savani BN, Warwick AB, Wingard JR, Wood WA, Sorror ML, Jacobsohn DA
Bone Marrow Transplant 2013 Mar;48(3):363-8. Epub 2012 Sep 10 doi: 10.1038/bmt.2012.166. PMID: 22964594Free PMC Article
Basta-Jovanovic G, Gvozdenovic E, Dimitrijevic I, Brasanac D, Jovanovic M, Kalezic N, Baralic I, Radojevic-Skodric S, Arsic D
Fetal Pediatr Pathol 2008;27(2):63-70. doi: 10.1080/15513810802028704. PMID: 18568994
Palmer RE, Kotsianti A, Cadman B, Boyd T, Gerald W, Haber DA
Curr Biol 2001 Nov 13;11(22):1805-9. doi: 10.1016/s0960-9822(01)00560-7. PMID: 11719225

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