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Wilms tumor 4(WT4)

MedGen UID:
318623
Concept ID:
C1832426
Neoplastic Process
Synonyms: FAMILIAL WILMS TUMOR 1; WT4
 
Monarch Initiative: MONDO:0011056
OMIM®: 601363

Definition

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.

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. [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

Qureshi SS, Kembhavi SA, Bhagat M, Kapadia T, Prasad M, Vora T, Chinnaswamy G, Ramadwar M, Laskar S, Khanna N, Baheti A, Shah S, Kurkure P, Talole S
Pediatr Blood Cancer 2019 Aug;66 Suppl 3:e27815. Epub 2019 May 16 doi: 10.1002/pbc.27815. PMID: 31099132
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

Recent clinical studies

Etiology

Ayaz E, Ozcan HN, Oguz B, Haliloglu M
Pediatr Radiol 2022 Dec;52(13):2557-2567. Epub 2022 Jun 28 doi: 10.1007/s00247-022-05422-7. PMID: 35760918
Tantawy AA, Ebeid FS, Mahmoud MA, Shepl OE
J Pediatr Hematol Oncol 2013 Apr;35(3):232-6. doi: 10.1097/MPH.0b013e318270b210. PMID: 23511492
Rangel M, Cypriano M, de Martino Lee ML, Luisi FA, Petrilli AS, Strufaldi MW, Franco Mdo C
Eur J Pediatr 2010 Jul;169(7):875-81. Epub 2010 Jan 26 doi: 10.1007/s00431-010-1139-1. PMID: 20101509

Diagnosis

Ayaz E, Ozcan HN, Oguz B, Haliloglu M
Pediatr Radiol 2022 Dec;52(13):2557-2567. Epub 2022 Jun 28 doi: 10.1007/s00247-022-05422-7. PMID: 35760918
Gupta S, Vanderbilt CM, Leibovich BC, Herrera-Hernandez L, Raghunathan A, Sukov WR, Voss JS, Barr Fritcher EG, Reed KA, Lohse CM, Reuter VE, Jimenez RE, Thompson RH, Cheville JC
Hum Pathol 2020 Sep;103:1-13. Epub 2020 Jul 15 doi: 10.1016/j.humpath.2020.07.014. PMID: 32681944Free PMC Article
Tantawy AA, Ebeid FS, Mahmoud MA, Shepl OE
J Pediatr Hematol Oncol 2013 Apr;35(3):232-6. doi: 10.1097/MPH.0b013e318270b210. PMID: 23511492
Fortney JT, Halperin EC, Hertz CM, Schulman SR
Int J Radiat Oncol Biol Phys 1999 Jun 1;44(3):587-91. doi: 10.1016/s0360-3016(99)00058-9. PMID: 10348288
Helson L, Krochmal P, Hajdu SI
Ann Clin Lab Sci 1975 Jul-Aug;5(4):294-7. PMID: 169727

Therapy

Gupta S, Vanderbilt CM, Leibovich BC, Herrera-Hernandez L, Raghunathan A, Sukov WR, Voss JS, Barr Fritcher EG, Reed KA, Lohse CM, Reuter VE, Jimenez RE, Thompson RH, Cheville JC
Hum Pathol 2020 Sep;103:1-13. Epub 2020 Jul 15 doi: 10.1016/j.humpath.2020.07.014. PMID: 32681944Free PMC Article
Fortney JT, Halperin EC, Hertz CM, Schulman SR
Int J Radiat Oncol Biol Phys 1999 Jun 1;44(3):587-91. doi: 10.1016/s0360-3016(99)00058-9. PMID: 10348288
Garrett RA, Battersby JS
J Urol 1971 Dec;106(6):942-6. doi: 10.1016/s0022-5347(17)61443-5. PMID: 4330020

Prognosis

Ayaz E, Ozcan HN, Oguz B, Haliloglu M
Pediatr Radiol 2022 Dec;52(13):2557-2567. Epub 2022 Jun 28 doi: 10.1007/s00247-022-05422-7. PMID: 35760918
Tantawy AA, Ebeid FS, Mahmoud MA, Shepl OE
J Pediatr Hematol Oncol 2013 Apr;35(3):232-6. doi: 10.1097/MPH.0b013e318270b210. PMID: 23511492
Fortney JT, Halperin EC, Hertz CM, Schulman SR
Int J Radiat Oncol Biol Phys 1999 Jun 1;44(3):587-91. doi: 10.1016/s0360-3016(99)00058-9. PMID: 10348288
Morrow CE, Grage TB, Sutherland DE, Najarian JS
Surgery 1982 Oct;92(4):610-4. PMID: 6289486
Garrett RA, Battersby JS
J Urol 1971 Dec;106(6):942-6. doi: 10.1016/s0022-5347(17)61443-5. PMID: 4330020

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