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==Overview==
==Overview==
==Natural History==
The symptoms of wilms tumor usually develop in the first decade of life, and start with symptoms such as an [[Abdominal mass|abdominal/flank mass]]. If left untreated, patients with wilms tumor may progress to develop [[hypertension]], [[anemia]], [[weight loss]], [[Renal insufficiency|renal failure]] and [[metastasis]]. The overall 5-year [[survival rate]] is approximately 63% for patients aged 10 to 16 years. Depending on various factors at the time of diagnosis, the [[prognosis]] may vary. These factors are [[histology]] of tumor, stage of tumor, [[Genetics|genetic]] and [[Molecular marker|molecular markers]] and age of the patient.


==Complications==
==Natural History, Complications, and Prognosis==
The tumor may become quite large, but usually remains self-enclosed. Spread of the tumor to the lungs, liver, bone, or brain is the most worrisome complication.


High blood pressure and kidney damage may occur as the result of the tumor or its treatment.
=== Natural History ===
* The symptoms of wilms tumor usually develop in the first decade of life, and start with symptoms such as an abdominal/flank mass.
* If left untreated, patients with wilms tumor may progress to develop hypertension, anemia, weight loss, renal failure and metastasis.
* The overall 5-year survival rate is approximately 63% for patients aged 10 to 16 years.  


Removal of Wilms tumor from both kidneys may affect kidney function.
===Complications===
*Common complications of wilms tumor include:
**Hypertension
**Anu
**Anemia
**Weight loss
**Renal failure
**Metastasis to:<ref name="pmid21384541">{{cite journal |vauthors=Termuhlen AM, Tersak JM, Liu Q, Yasui Y, Stovall M, Weathers R, Deutsch M, Sklar CA, Oeffinger KC, Armstrong G, Robison LL, Green DM |title=Twenty-five year follow-up of childhood Wilms tumor: a report from the Childhood Cancer Survivor Study |journal=Pediatr Blood Cancer |volume=57 |issue=7 |pages=1210–6 |date=December 2011 |pmid=21384541 |pmc=4634648 |doi=10.1002/pbc.23090 |url=}}</ref>
***Lungs
***Liver
***Bone
***Brain 


==Prognosis==
===Prognosis===
* Depending on various factors at the time of diagnosis, the prognosis may vary.<ref name="pmid26304882">{{cite journal |vauthors=Dome JS, Graf N, Geller JI, Fernandez CV, Mullen EA, Spreafico F, Van den Heuvel-Eibrink M, Pritchard-Jones K |title=Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration |journal=J. Clin. Oncol. |volume=33 |issue=27 |pages=2999–3007 |date=September 2015 |pmid=26304882 |pmc=4567702 |doi=10.1200/JCO.2015.62.1888 |url=}}</ref>
* However, the prognosis can be tumor recurrence or death. 
* These factors are:
** Histology of tumor
** Stage of the tumor
** Genetic and molecular markers
** Age of the patient
===== Histology of tumor =====
* 2 research groups have classified wilms tumor based on histology. These are:
**  National Wilms Tumor Study / Children's Oncology Group (NWTS/COG) .
** International Society of Pediatric Oncology (SIOP).
*  Prognosis is dependant on presence or absence of anaplasia.
*  If anaplasia is positive then it signifies a poor prognosis in children.<ref name="pmid2844645">{{cite journal |vauthors=Zuppan CW, Beckwith JB, Luckey DW |title=Anaplasia in unilateral Wilms' tumor: a report from the National Wilms' Tumor Study Pathology Center |journal=Hum. Pathol. |volume=19 |issue=10 |pages=1199–209 |date=October 1988 |pmid=2844645 |doi= |url=}}</ref><ref name="pmid6164480">{{cite journal |vauthors=D'Angio GJ, Evans A, Breslow N, Beckwith B, Bishop H, Farewell V, Goodwin W, Leape L, Palmer N, Sinks L, Sutow W, Tefft M, Wolff J |title=The treatment of Wilms' tumor: results of the Second National Wilms' Tumor Study |journal=Cancer |volume=47 |issue=9 |pages=2302–11 |date=May 1981 |pmid=6164480 |doi= |url=}}</ref>


The prognosis for patients with Wilms tumor depends on the following:[100-103]
==== Stage of the tumor ====
 
* Staging of wilms tumor is done on the basis of:<ref name="pmid16314292">{{cite journal |vauthors=Metzger ML, Dome JS |title=Current therapy for Wilms' tumor |journal=Oncologist |volume=10 |issue=10 |pages=815–26 |date=2005 |pmid=16314292 |doi=10.1634/theoncologist.10-10-815 |url=}}</ref>
* Stage of disease at diagnosis.
** Genetics
* Tumor size.
** Histology
* Histopathologic features of the tumor (FH vs. anaplastic histology). (Refer to the Histologic Findings in Wilms Tumor section of this summary for more information.)
** Molecular markers
* Molecular features of the tumor. B7-H1, an immune costimulatory molecule, has been found to be associated with an increased risk of tumor recurrence in favorable histology Wilms tumor.[104]
* Stage I (43% of patients)
* Patient age (adolescents and young adults).
** Outcome: 98% 4-year survival; 85% 4-year survival if [[anaplastic]]
 
* Stage II (23% of patients)
'''Children with Wilms tumor'''
** Outcome: 96% 4-year survival; 70% 4-year survival if [[Anaplasia|anaplastic]]
 
* Stage III (23% of patients)
Wilms tumor is a curable disease in most affected children. Since the 1980s, the 5-year survival rate for Wilms tumor with FH has been consistently above 90%.[98] This favorable outcome occurred despite reductions in the length of therapy, dose of radiation, extent of fields irradiated, and the percentage of patients receiving radiation therapy.[99]
** Outcome: 95% 4-year survival; 56% 4-year survival if [[Anaplasia|anaplastic]]
 
* Stage IV (10% of patients)
'''Adolescents and young adults with Wilms tumor'''
** Outcome: 90% 4-year survival; 17% 4-year survival if [[Anaplasia|anaplastic]]
 
In an analysis of Wilms tumor patients in the Surveillance, Epidemiology, and End Results (SEER) database, adults (n = 152) had a statistically worse OS (69% vs. 88%, P < .001) than did pediatric patients (n = 2,190),[105] despite previous studies showing comparable outcome with treatment on protocol.[106,107] The inferior outcome of the adult patients on this study may be the result of differences in tumor biology between children and adults, incorrect diagnosis, inadequate staging (e.g., more likely to be staged as localized disease or to not receive lymph node sampling), or undertreatment (e.g., not receiving radiation therapy). Additional factors in this SEER report that may have contributed to a worse OS in adult patients include the size of the study and lack of central review of pathology.[105] Adolescent and young adult patients up to age 30 years are now eligible for treatment on the COG Wilms tumor protocols.
 
The inferior outcome of older patients is not explained entirely by inadequate treatment or not being treated according to the pediatric Wilms tumor protocol. In a U.K. study looking at the outcome of patients aged 10 to 16 years (N = 50) registered on the U.K. Wilms Tumor 3 and SIOP 2001 Wilms tumor trials, patients in this age group had a higher percentage of diffuse anaplastic tumors. The overall 5-year survival was 63% for patients aged 10 to 16 years (43% for anaplastic tumors), which is significantly lower than the outcome for younger patients with Wilms tumor.[108] However, SEER 5-year relative survival of nephroblastoma between 2003 and 2009 did not show differences among age groups from younger than 1 year to age 10 to 14 years.[109]


==== Genetic and molecular markers ====
* Multiple genetic and molecular markers can predict prognosis.<ref name="pmid21189373">{{cite journal |vauthors=Perlman EJ, Grundy PE, Anderson JR, Jennings LJ, Green DM, Dome JS, Shamberger RC, Ruteshouser EC, Huff V |title=WT1 mutation and 11P15 loss of heterozygosity predict relapse in very low-risk wilms tumors treated with surgery alone: a children's oncology group study |journal=J. Clin. Oncol. |volume=29 |issue=6 |pages=698–703 |date=February 2011 |pmid=21189373 |pmc=3056654 |doi=10.1200/JCO.2010.31.5192 |url=}}</ref> <ref name="pmid18757319">{{cite journal |vauthors=D'Angio GJ |title=Pre- or postoperative therapy for Wilms' tumor? |journal=J. Clin. Oncol. |volume=26 |issue=25 |pages=4055–7 |date=September 2008 |pmid=18757319 |doi=10.1200/JCO.2008.16.5316 |url=}}</ref>
* These markers are:
** Loss of heterozygosity (LOH) at chromosomes:
*** 1p
*** 11p15
*** 16q
** Gain in :
*** 1q
* These markers are associated with increased risk of relapse and mortality.


====Age of the patient====
* Patient with age lass than 2 years have a better prognosis.<ref name="pmid205340">{{cite journal |vauthors=Breslow NE, Palmer NF, Hill LR, Buring J, D'Angio GJ |title=Wilms' tumor: prognostic factors for patients without metastases at diagnosis: results of the National Wilms' Tumor Study |journal=Cancer |volume=41 |issue=4 |pages=1577–89 |date=April 1978 |pmid=205340 |doi= |url=}}</ref>
* As the patient gets old the treatment related complication rate also increases.<ref name="pmid15542800">{{cite journal |vauthors=Reinhard H, Aliani S, Ruebe C, Stöckle M, Leuschner I, Graf N |title=Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study |journal=J. Clin. Oncol. |volume=22 |issue=22 |pages=4500–6 |date=November 2004 |pmid=15542800 |doi=10.1200/JCO.2004.12.099 |url=}}</ref>


==References==
==References==
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{{reflist|2}}
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[[Category:Kidney diseases]]
[[Category:Kidney diseases]]
[[Category:Types of cancer]]
[[Category:Types of cancer]]
[[Category:Urology]]
[[Category:Urology]]
[[Category:Pediatrics]]
[[Category:Pediatrics]]
[[Category:Oncology]]
[[Category:Hematology]]
[[Category:Hematology]]
[[Category:Needs content]]

Latest revision as of 21:29, 11 June 2018

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

Overview

The symptoms of wilms tumor usually develop in the first decade of life, and start with symptoms such as an abdominal/flank mass. If left untreated, patients with wilms tumor may progress to develop hypertension, anemia, weight loss, renal failure and metastasis. The overall 5-year survival rate is approximately 63% for patients aged 10 to 16 years. Depending on various factors at the time of diagnosis, the prognosis may vary. These factors are histology of tumor, stage of tumor, genetic and molecular markers and age of the patient.

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of wilms tumor usually develop in the first decade of life, and start with symptoms such as an abdominal/flank mass.
  • If left untreated, patients with wilms tumor may progress to develop hypertension, anemia, weight loss, renal failure and metastasis.
  • The overall 5-year survival rate is approximately 63% for patients aged 10 to 16 years.

Complications

  • Common complications of wilms tumor include:
    • Hypertension
    • Anu
    • Anemia
    • Weight loss
    • Renal failure
    • Metastasis to:[1]
      • Lungs
      • Liver
      • Bone
      • Brain

Prognosis

  • Depending on various factors at the time of diagnosis, the prognosis may vary.[2]
  • However, the prognosis can be tumor recurrence or death.
  • These factors are:
    • Histology of tumor
    • Stage of the tumor
    • Genetic and molecular markers
    • Age of the patient
Histology of tumor
  • 2 research groups have classified wilms tumor based on histology. These are:
    •  National Wilms Tumor Study / Children's Oncology Group (NWTS/COG) .
    • International Society of Pediatric Oncology (SIOP).
  •  Prognosis is dependant on presence or absence of anaplasia.
  •  If anaplasia is positive then it signifies a poor prognosis in children.[3][4]

Stage of the tumor

  • Staging of wilms tumor is done on the basis of:[5]
    • Genetics
    • Histology
    • Molecular markers
  • Stage I (43% of patients)
    • Outcome: 98% 4-year survival; 85% 4-year survival if anaplastic
  • Stage II (23% of patients)
    • Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic
  • Stage III (23% of patients)
    • Outcome: 95% 4-year survival; 56% 4-year survival if anaplastic
  • Stage IV (10% of patients)
    • Outcome: 90% 4-year survival; 17% 4-year survival if anaplastic

Genetic and molecular markers

  • Multiple genetic and molecular markers can predict prognosis.[6] [7]
  • These markers are:
    • Loss of heterozygosity (LOH) at chromosomes:
      • 1p
      • 11p15
      • 16q
    • Gain in :
      • 1q
  • These markers are associated with increased risk of relapse and mortality.

Age of the patient

  • Patient with age lass than 2 years have a better prognosis.[8]
  • As the patient gets old the treatment related complication rate also increases.[9]

References

  1. ↑ Termuhlen AM, Tersak JM, Liu Q, Yasui Y, Stovall M, Weathers R, Deutsch M, Sklar CA, Oeffinger KC, Armstrong G, Robison LL, Green DM (December 2011). "Twenty-five year follow-up of childhood Wilms tumor: a report from the Childhood Cancer Survivor Study". Pediatr Blood Cancer. 57 (7): 1210–6. doi:10.1002/pbc.23090. PMC 4634648. PMID 21384541.
  2. ↑ Dome JS, Graf N, Geller JI, Fernandez CV, Mullen EA, Spreafico F, Van den Heuvel-Eibrink M, Pritchard-Jones K (September 2015). "Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration". J. Clin. Oncol. 33 (27): 2999–3007. doi:10.1200/JCO.2015.62.1888. PMC 4567702. PMID 26304882.
  3. ↑ Zuppan CW, Beckwith JB, Luckey DW (October 1988). "Anaplasia in unilateral Wilms' tumor: a report from the National Wilms' Tumor Study Pathology Center". Hum. Pathol. 19 (10): 1199–209. PMID 2844645.
  4. ↑ D'Angio GJ, Evans A, Breslow N, Beckwith B, Bishop H, Farewell V, Goodwin W, Leape L, Palmer N, Sinks L, Sutow W, Tefft M, Wolff J (May 1981). "The treatment of Wilms' tumor: results of the Second National Wilms' Tumor Study". Cancer. 47 (9): 2302–11. PMID 6164480.
  5. ↑ Metzger ML, Dome JS (2005). "Current therapy for Wilms' tumor". Oncologist. 10 (10): 815–26. doi:10.1634/theoncologist.10-10-815. PMID 16314292.
  6. ↑ Perlman EJ, Grundy PE, Anderson JR, Jennings LJ, Green DM, Dome JS, Shamberger RC, Ruteshouser EC, Huff V (February 2011). "WT1 mutation and 11P15 loss of heterozygosity predict relapse in very low-risk wilms tumors treated with surgery alone: a children's oncology group study". J. Clin. Oncol. 29 (6): 698–703. doi:10.1200/JCO.2010.31.5192. PMC 3056654. PMID 21189373.
  7. ↑ D'Angio GJ (September 2008). "Pre- or postoperative therapy for Wilms' tumor?". J. Clin. Oncol. 26 (25): 4055–7. doi:10.1200/JCO.2008.16.5316. PMID 18757319.
  8. ↑ Breslow NE, Palmer NF, Hill LR, Buring J, D'Angio GJ (April 1978). "Wilms' tumor: prognostic factors for patients without metastases at diagnosis: results of the National Wilms' Tumor Study". Cancer. 41 (4): 1577–89. PMID 205340.
  9. ↑ Reinhard H, Aliani S, Ruebe C, Stöckle M, Leuschner I, Graf N (November 2004). "Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study". J. Clin. Oncol. 22 (22): 4500–6. doi:10.1200/JCO.2004.12.099. PMID 15542800.

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