Wilms' tumor natural history, complications and prognosis
|
Wilms' tumor Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Wilms' tumor natural history, complications and prognosis On the Web |
|
American Roentgen Ray Society Images of Wilms' tumor natural history, complications and prognosis |
|
FDA on Wilms' tumor natural history, complications and prognosis |
|
CDC on Wilms' tumor natural history, complications and prognosis |
|
Wilms' tumor natural history, complications and prognosis in the news |
|
Blogs on Wilms' tumor natural history, complications and prognosis |
|
Risk calculators and risk factors for Wilms' tumor natural history, complications and prognosis |
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
The prognosis for patients with Wilms tumor depends on the following:
- Stage of disease at diagnosis.
- Tumor size.
- 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 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.
- Patient age (adolescents and young adults).
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.