Wilms' tumor causes: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
 
(23 intermediate revisions by 4 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{CMG}}
{{Wilms' tumor}}
{{Wilms' tumor}}
{{CMG}}; {{AE}} {{SC}}{{SSW}}


==Overview==
==Overview==
The causes of wilms' tumor include genetic mutations and several associated syndromes.
Wilms' tumor may be caused by either [[Genetics|genetic]] [[Mutation|mutations]] or [[Chromosome|chromosomal]] alterations.
 
==Causes==
==Causes==
Wilms tumor is the most common form of childhood kidney cancer. The exact cause of this tumor in most children is unknown.
* [[Genetic]] mutations: <ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq </ref>
===Molecular biology===
** Wilms tumor 1 gene ([[WT1]])
 
** WT2<ref name="pmid9441738">{{cite journal |vauthors=Crider-Miller SJ, Reid LH, Higgins MJ, Nowak NJ, Shows TB, Futreal PA, Weissman BE |title=Novel transcribed sequences within the BWS/WT2 region in 11p15.5: tissue-specific expression correlates with cancer type |journal=Genomics |volume=46 |issue=3 |pages=355–63 |date=December 1997 |pmid=9441738 |doi=10.1006/geno.1997.5061 |url=}}</ref>
Mutations of  the [[WT1]] gene on chromosome 11 are observed in approximately 20% of Wilms' tumors.<ref>{{cite journal |author=Call K, Glaser T, Ito C, Buckler A, Pelletier J, Haber D, Rose E, Kral A, Yeger H, Lewis W |title=Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus |journal=Cell |volume=60 |issue=3 |pages=509-20 |year=1990 |pmid=2154335}}</ref><ref>{{cite journal |author=Huff V |title=Wilms tumor genetics |journal=Am J Med Genet |volume=79 |issue=4 |pages=260-7 |year=1998 |pmid=9781905}}</ref> At least half of the Wilms' tumors with mutations in WT1 also carry mutations in CTNNB1, the gene encoding the proto-oncogene beta-catenin.<ref>{{cite journal |author=Maiti S, Alam R, Amos CI, Huff V |title=Frequent association of beta-catenin and WT1 mutations in Wilms tumors |journal=Cancer Res |volume=60 |issue=22 |pages=6288-92 |year=2000 |pmid=11103785}}</ref>
** WTX<ref name="pmid17204608">{{cite journal |vauthors=Rivera MN, Kim WJ, Wells J, Driscoll DR, Brannigan BW, Han M, Kim JC, Feinberg AP, Gerald WL, Vargas SO, Chin L, Iafrate AJ, Bell DW, Haber DA |title=An X chromosome gene, WTX, is commonly inactivated in Wilms tumor |journal=Science |volume=315 |issue=5812 |pages=642–5 |date=February 2007 |pmid=17204608 |doi=10.1126/science.1137509 |url=}}</ref>
 
A gene on the X chromosome, WTX, is inactivated in up to 30% of Wilms' tumor cases, according to research published in 2007.<ref>{{cite journal |author=Rivera M, Kim W, Wells J, Driscoll D, Brannigan B, Han M, Kim J, Feinberg A, Gerald W, Vargas S, Chin L, Iafrate A, Bell D, Haber D |title=An X chromosome gene, WTX, is commonly inactivated in Wilms tumor |journal=Science |volume=315 |issue=5812 |pages=642-5 |year=2007 |pmid=17204608}}</ref>
 
===Associated Syndromes===
It is commonly associated with the following syndromes:
 
1) [[Denys-Drash syndrome]] is a syndrome with a very high risk of Wilms’ tumor (upto 90%). Other features of this syndrome are gonadal dysgenesis and renal mesangial sclerosis which leads to renal failure in the first few years of life.
 
2) [[WAGR syndrome]] is a rare genetic syndrome in which affected children are predisposed to develop Wilms tumour (a tumour of thekidneys), Aniridia (absence of the coloured part of the eye, the iris), Genitourinary anomalies, and mental Retardation.
<ref>Kumar, V., Abbas, A. K., Fausto, N., Aster, J. C. (2010), Robbins and Cotran Pathologic Basis of Disease-Eighth Edition. Saunders Elsevier.</ref>
 
The G is sometimes instead given as "gonadoblastoma," since the genitourinary anomalies are tumours of the gonads (testes or ovaries).
<ref>^ a b Fischbach BV, Trout KL, Lewis J, Luis CA, Sika M (2005). "WAGR syndrome: a clinical review of 54 cases". Pediatrics 116 (4): 984–8. doi:10.1542/peds.2004-0467.PMID 16199712.</ref>
 
3) [[Beckwith–Wiedemann syndrome]] (pron.: /ˈbɛkˌwɪθ ˈviːdə.mən/; abbreviated BWS) is an overgrowth disorder usually (but not always) present at birth characterized by an increased risk of childhood cancer and certain congenital features. Originally, Dr. Hans-Rudolf Wiedemann coined the term exomphalos-macroglossia-gigantism (EMG) syndrome to describe the combination of congenitalabdominal wall defects as hernia (exomphalos), large tongues (macroglossia), and large bodies and/or long limbs (gigantism). Over time, this constellation was renamed Beckwith–Wiedemann syndrome following the autoptical observations of Prof. John Bruce Beckwith, who observed also severe increase in the size of the adrenal glands in some of these patients. Five common features used to define BWS are: macroglossia, macrosomia (birth weight and length greater than the 90th percentile), midline abdominal wall defects (omphalocele/exomphalos, umbilical hernia, diastasis recti), ear creases or ear pits, and neonatal hypoglycemia (low blood sugar after birth).<ref>Clericuzio CL (2004). "WAGR syndrome". In Cassidy SB, Allanson JE. Management of Genetic Syndromes (2nd ed.). New York, NY: John Wiley & Sons. ISBN 0-471-30870-6.</ref> <ref>DeBaun MR, Tucker MA (March 1998). "Risk of cancer during the first four years of life in children from The Beckwith-Wiedemann Syndrome Registry". The Journal of pediatrics 132 (3 Pt 1): 398–400. doi:10.1016/S0022-3476(98)70008-3.PMID 9544889.</ref>
 
4)[[Sotos syndrome]]


* Other genes and chromosomal alterations:
** 1q<ref name="pmid1850292">{{cite journal |vauthors=Truong HT, Pratt EA, Ho C |title=Interaction of the membrane-bound D-lactate dehydrogenase of Escherichia coli with phospholipid vesicles and reconstitution of activity using a spin-labeled fatty acid as an electron acceptor: a magnetic resonance and biochemical study |journal=Biochemistry |volume=30 |issue=16 |pages=3893–8 |date=April 1991 |pmid=1850292 |doi= |url=}}</ref>
** 16q and 1p<ref name="pmid8162576">{{cite journal |vauthors=Grundy PE, Telzerow PE, Breslow N, Moksness J, Huff V, Paterson MC |title=Loss of heterozygosity for chromosomes 16q and 1p in Wilms' tumors predicts an adverse outcome |journal=Cancer Res. |volume=54 |issue=9 |pages=2331–3 |date=May 1994 |pmid=8162576 |doi= |url=}}</ref>
** CACNA1E<ref name="pmid17189400">{{cite journal |vauthors=Natrajan R, Little SE, Reis-Filho JS, Hing L, Messahel B, Grundy PE, Dome JS, Schneider T, Vujanic GM, Pritchard-Jones K, Jones C |title=Amplification and overexpression of CACNA1E correlates with relapse in favorable histology Wilms' tumors |journal=Clin. Cancer Res. |volume=12 |issue=24 |pages=7284–93 |date=December 2006 |pmid=17189400 |doi=10.1158/1078-0432.CCR-06-1567 |url=}}</ref>
** 7p21
** SKCG-1
** TP53 (tumor suppressor gene)
** FBXW7
** PTCH1
** DICER1
** MYCN
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
{{WH}}
{{WH}}
{{WS}}
{{WS}}
Line 38: Line 33:
[[Category:Urology]]
[[Category:Urology]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
[[Category:Oncology]]
[[Category:Hematology]]
[[Category:Hematology]]

Latest revision as of 19:53, 18 June 2018

Wilms' tumor Microchapters

Home

Patient Information

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating Wilms' Tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Staging

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

Echocardiography or Ultrasound

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Wilms' tumor causes On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Wilms' tumor causes

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Wilms' tumor causes

CDC on Wilms' tumor causes

Wilms' tumor causes in the news

Blogs on Wilms' tumor causes

Directions to Hospitals Treating Wilms' tumor

Risk calculators and risk factors for Wilms' tumor causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]Sargun Singh Walia M.B.B.S.[3]

Overview

Wilms' tumor may be caused by either genetic mutations or chromosomal alterations.

Causes

  • Other genes and chromosomal alterations:
    • 1q[4]
    • 16q and 1p[5]
    • CACNA1E[6]
    • 7p21
    • SKCG-1
    • TP53 (tumor suppressor gene)
    • FBXW7
    • PTCH1
    • DICER1
    • MYCN

References

  1. ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq
  2. ↑ Crider-Miller SJ, Reid LH, Higgins MJ, Nowak NJ, Shows TB, Futreal PA, Weissman BE (December 1997). "Novel transcribed sequences within the BWS/WT2 region in 11p15.5: tissue-specific expression correlates with cancer type". Genomics. 46 (3): 355–63. doi:10.1006/geno.1997.5061. PMID 9441738.
  3. ↑ Rivera MN, Kim WJ, Wells J, Driscoll DR, Brannigan BW, Han M, Kim JC, Feinberg AP, Gerald WL, Vargas SO, Chin L, Iafrate AJ, Bell DW, Haber DA (February 2007). "An X chromosome gene, WTX, is commonly inactivated in Wilms tumor". Science. 315 (5812): 642–5. doi:10.1126/science.1137509. PMID 17204608.
  4. ↑ Truong HT, Pratt EA, Ho C (April 1991). "Interaction of the membrane-bound D-lactate dehydrogenase of Escherichia coli with phospholipid vesicles and reconstitution of activity using a spin-labeled fatty acid as an electron acceptor: a magnetic resonance and biochemical study". Biochemistry. 30 (16): 3893–8. PMID 1850292.
  5. ↑ Grundy PE, Telzerow PE, Breslow N, Moksness J, Huff V, Paterson MC (May 1994). "Loss of heterozygosity for chromosomes 16q and 1p in Wilms' tumors predicts an adverse outcome". Cancer Res. 54 (9): 2331–3. PMID 8162576.
  6. ↑ Natrajan R, Little SE, Reis-Filho JS, Hing L, Messahel B, Grundy PE, Dome JS, Schneider T, Vujanic GM, Pritchard-Jones K, Jones C (December 2006). "Amplification and overexpression of CACNA1E correlates with relapse in favorable histology Wilms' tumors". Clin. Cancer Res. 12 (24): 7284–93. doi:10.1158/1078-0432.CCR-06-1567. PMID 17189400.

Template:WH Template:WS

Retrieved from "https://www.wikidoc.org/index.php?title=Wilms%27_tumor_causes&oldid=1477767"