Wilms' tumor natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
The complications of wilms' tumor include metastasis, high blood pressure, and kidney damage. Prognosis is generally good. The 5-year survival rate for Wilms tumor in children is around 90%, whereas older patients suffer worse outcome. The overall 5-year survival rate is approximately 63% for patients aged 10 to 16 years. Also, according to the different stage, anaplastic tumors have the relatively poorer outcome.
Natural History
Complications
- Common complications of wilms tumor include:
- Hypertension
- 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
- Stage I (43% of patients)
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
- Loss of heterozygosity (LOH) at chromosomes:
- These markers are associated with increased risk of relapse and mortality.
- risk of relapse and mortality in some patients with favorable histology Wilms tumor
Age
Children with Wilms tumor
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%. 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.
Adolescents and young adults with Wilms tumor
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), despite previous studies showing comparable outcome with treatment on protocol. 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. 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. 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.
Staging
- 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
- Stage V (5% of patients)
- Stage V Wilms’ tumor is defined as bilateral renal involvement at the time of initial diagnosis.
- Note: For patients with bilateral involvement, an attempt should be made to stage each side according to the above criteria (stage I to III) on the basis of extent of disease prior to biopsy.
- The 4-year survival was 94% for those patients whose most advanced lesion was stage I or stage II; 76% for those whose most advanced lesion was stage III.
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Metzger ML, Dome JS (2005). "Current therapy for Wilms' tumor". Oncologist. 10 (10): 815–26. doi:10.1634/theoncologist.10-10-815. PMID 16314292.
- ↑ 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.
- ↑ 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.