Prognosis and survival for Wilms tumour

If your child has Wilms tumour, you will have questions about their prognosis. A prognosis is the doctor’s best estimate of how cancer will affect someone and how well it will respond to treatment. Prognosis and survival depend on many factors. Only a doctor familiar with your child’s medical history, the type and stage and other features of the cancer, the treatments chosen and the response to treatment can put all of this information together with survival statistics to arrive at a prognosis.

A prognostic factor is an aspect of the cancer or a characteristic of the child that the doctor will consider when making a prognosis. A predictive factor influences how a cancer will respond to a certain treatment. Prognostic and predictive factors are often discussed together. They both play a part in deciding on a treatment plan and a prognosis.

The following are prognostic and predictive factors for Wilms tumour.

Type of tumour

The type of Wilms tumour is an important prognostic factor. Tumours with favourable histology have a better prognosis than those with anaplastic (unfavourable) histology. Diffuse anaplastic histology (anaplasia found throughout the tumour) is associated with higher rates of relapse and worse survival than a favourable histology.

In children who have had chemotherapy before surgery, several other types of tumour can be determined. Blastemal-predominant tumours (tumours with lots of anaplasia left after chemotherapy) have a poorer prognosis. Completely necrotic tumours (tumours where all cells have died with chemotherapy) have a better prognosis.

Stage

The earlier the stage of Wilms tumour, the more favourable the prognosis. Cancer that has spread to the lymph nodes or to other areas of the body has a poorer prognosis.

Certain chromosome changes

Some tumour cells have certain chromosome changes that can affect prognosis. Loss of heterozygosity (LOH) is when one allele, or form, of a gene or genes within tumour cells stops working. This can mean that a gene that normally helps limit the growth of cancer cells (called a tumour suppressor gene) stops working. LOH for DNA markers on chromosomes 1p and 16q has been associated with a higher rate of recurrence and poorer prognosis in children with favourable histology Wilms tumour. Having too much of a section of chromosome may also be associated with a higher chance of recurrence. An example of this is the gain of a section of chromosome called 1q.

Age

Younger age is a favourable prognostic factor. Children under 2 with favourable histology tumours that have not spread have a better prognosis than older children.

Genetic syndromes

Several genetic syndromes, including Beckwith-Wiedemann, Denys-Drash and WAGR, are associated with the risk of developing Wilms tumours in both kidneys. In general, children with these genetic syndromes have tumours that respond well to therapy and no increased risk of relapse. But there is a higher incidence of serious kidney disease (end-stage renal disease, or ESRD) in children with Wilms tumours in both kidneys.

Recurrent Wilms tumours

The prognosis for a recurrent Wilms tumour is generally better if:

  • the tumour has a favourable histology
  • no previous chemotherapy with doxorubicin (Adriamycin) was given
  • no previous radiation therapy was given
  • the recurrence happens at least 1 year after the initial diagnosis

Expert review and references

  • American Cancer Society. Wilms Tumor. Atlanta, GA: American Cancer Society; 2013.
  • Cendron, M. C. et al . Surgery for Wilms tumor. eMedicine.Medscape.com. WebMD LLC; 2011.
  • Children's Hospital Boston . Wilms tumor. Children's Hospital Boston - My Child Has.... Boston, MA: Children's Hospital Boston; 2007.
  • Dome, J.S., Perlman, E.J, et al . Renal tumors. Pizzo, P. A. & Poplack, D. G. (Eds.). Principles and Practice of Pediatric Oncology. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 2006: 30: 905-932.
  • Drigan, R. and Androkites, A. . Wilms Tumor. Baggott, C. R., Kelly, K. P., Fochtman, D. et al. Nursing Care of Children and Adolescents with Cancer. 3rd ed. Philadelphia, PA: W. B. Saunders Company; 2002: 26: pp.568-574.
  • Duffey-Lind, E . Tumours of the kidney. Kline, N. E. (Ed.). Essentials of Pediatric Oncology Nursing: A Core Curriculum. 2nd ed. Association of Pediatric Oncology Nurses; 2004: 2.10:46-49.
  • Fernandez C. et al . Renal tumors. Pizzo, P. A. & Poplack, D. G. (Eds.). Principles and Practice of Pediatric Oncology. 5th ed. Philadelphia: Lippincott Williams & Wilkins; 2011: 29:861-908.
  • Fernandez CV, Geller JI, Ehrlich PF et al . Renal Tumors. Pizzo PA & Poplack DG (eds.). Principles and Practice of Pediatric Oncology. 7th ed. Philadelphia: Wolters Kluwer; 2016: 29:753-768.
  • Hendershot E . Renal tumours. Tomlinson, D. & Kline, N. E. Pediatric Oncology Nursing: Advanced Clinical Handbook. Germany: Springer; 2005: 2.7: 57-61.
  • Wilms tumor. Janes-Hodder, H. & Keene, N. Childhood Cancer - A Parent's Guide to Solid Tumor Cancers. 2nd ed. O'Reilly; 2002: 9:137-149.
  • National Cancer Institute. Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) Health Professional Version. Bethesda, MD: National Cancer Institute; 2012.
  • National Cancer Institute. Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) Patient Version. Bethesda, MD: National Cancer Institute; 2012.
  • National Cancer Institute. Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) Patient Version. 2017.
  • National Cancer Institute. Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®) Health Professional Version. 2017.
  • Paulino, A. C. et al . Wilms tumor. eMedicine.Medscape.com. WebMD LLC; 2011.
  • Wilms' Tumor: The Basics. University of Pennsylvania. OncoLink. Reviewed ed. University of Pennsylvania; 2008.

Survival statistics for Wilms tumour

Learn about survival statistics for Wilms tumour, including observed survival and survival by stage. Survival varies with each stage of Wilms tumour.