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Wilms tumor 5(WT5; WTSL)

MedGen UID:
316905
Concept ID:
C1832099
Neoplastic Process
Synonyms: Bilateral radial aplasia with Wilms tumor; Wilms tumor and radial bilateral aplasia; Wilms tumor susceptibility-5; WILMS TUMOR, SUSCEPTIBILITY TO
 
Gene (location): POU6F2 (7p14.1)
 
Monarch Initiative: MONDO:0011112
OMIM®: 601583

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

Park KD, Hong CR, Choi JY, Kim MS, Yi ES, Saysouliyo S, Phongsavath K, Shin HY
Pediatr Hematol Oncol 2018 May;35(4):268-275. Epub 2018 Dec 31 doi: 10.1080/08880018.2018.1477888. PMID: 30595064
Calvo FA, Sierrasesúmaga L, Martin I, Santos M, Voltas J, Berian JM, Cañadell J
Acta Oncol 1989;28(2):257-60. doi: 10.3109/02841868909111259. PMID: 2736116

Recent clinical studies

Etiology

Ottaviani G, Jaffe N
Cancer Treat Res 2009;152:3-13. doi: 10.1007/978-1-4419-0284-9_1. PMID: 20213383
Szavay P, Luithle T, Graf N, Furtwängler R, Fuchs J
J Pediatr Surg 2006 Jan;41(1):168-72; discussion 168-72. doi: 10.1016/j.jpedsurg.2005.10.021. PMID: 16410128
Homsy YL, Anderson JH, Oudjhane K, Russo P
J Urol 1997 Dec;158(6):2256-9; discussion 2259-60. doi: 10.1016/s0022-5347(01)68227-2. PMID: 9366370
Nakissa N, Constine LS, Rubin P, Strohl R
Oncology 1985;42(6):358-63. doi: 10.1159/000226064. PMID: 2999670
D'Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Feigl P, Goodwin W, Leape LL, Sinks LF, Sutow W, Tefft M, Wolff J
Cancer 1976 Aug;38(2):633-46. doi: 10.1002/1097-0142(197608)38:2<633::aid-cncr2820380203>3.0.co;2-s. PMID: 184912

Diagnosis

Szavay P, Luithle T, Graf N, Furtwängler R, Fuchs J
J Pediatr Surg 2006 Jan;41(1):168-72; discussion 168-72. doi: 10.1016/j.jpedsurg.2005.10.021. PMID: 16410128
Homsy YL, Anderson JH, Oudjhane K, Russo P
J Urol 1997 Dec;158(6):2256-9; discussion 2259-60. doi: 10.1016/s0022-5347(01)68227-2. PMID: 9366370
Nakissa N, Constine LS, Rubin P, Strohl R
Oncology 1985;42(6):358-63. doi: 10.1159/000226064. PMID: 2999670
D'Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Feigl P, Goodwin W, Leape LL, Sinks LF, Sutow W, Tefft M, Wolff J
Cancer 1976 Aug;38(2):633-46. doi: 10.1002/1097-0142(197608)38:2<633::aid-cncr2820380203>3.0.co;2-s. PMID: 184912

Therapy

D'Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Feigl P, Goodwin W, Leape LL, Sinks LF, Sutow W, Tefft M, Wolff J
Cancer 1976 Aug;38(2):633-46. doi: 10.1002/1097-0142(197608)38:2<633::aid-cncr2820380203>3.0.co;2-s. PMID: 184912

Prognosis

Ottaviani G, Jaffe N
Cancer Treat Res 2009;152:3-13. doi: 10.1007/978-1-4419-0284-9_1. PMID: 20213383
Szavay P, Luithle T, Graf N, Furtwängler R, Fuchs J
J Pediatr Surg 2006 Jan;41(1):168-72; discussion 168-72. doi: 10.1016/j.jpedsurg.2005.10.021. PMID: 16410128
D'Angio GJ, Evans AE, Breslow N, Beckwith B, Bishop H, Feigl P, Goodwin W, Leape LL, Sinks LF, Sutow W, Tefft M, Wolff J
Cancer 1976 Aug;38(2):633-46. doi: 10.1002/1097-0142(197608)38:2<633::aid-cncr2820380203>3.0.co;2-s. PMID: 184912

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