Wilms Tumor Pathology

Wilms Tumor is the most common type of kidney cancer in children, although it can also affect some adults. In the majority of cases, the tumor is localized to the kidney and has not formed metastases in other parts of the body at diagnosis, which is favorable for the prognosis.

Image Credit: crystal light / Shutterstock.com

Affected area

The vast majority of nephroblastomas are unilateral, with disease in a single kidney accounting for 95% percent of all cases. These are usually encapsulated and often present as a single tumor.

A small number of people with Wilms tumor are associated with metastasis to other parts of the body. The most common location for this to occur is the lung, where it causes specific symptoms that are related to this area of the body.

If the tumor ruptures, the patient is at risk of hemorrhage and peritoneal dissemination of the tumor, which usually requires immediate surgical intervention. There are three main elements of a Wilms tumor found in the kidney, which include blastema, mesenchyme and epithelium.

Associated conditions

The majority of children that suffer from Wilms tumor are otherwise healthy and do not have any other notable health conditions. However, approximately 1 in 10 of all cases are associated with malformations that may increase the risk of tumor development.

Overgrowth syndromes may lead to Wilms tumor with symptoms of excessive somatic growth leading to macroglossia, nephromegaly and hemihypertrophy. Some examples of the health conditions that are related to overgrowth include Beckwith-Wiedemann syndrome, Perlman syndrome, Sotos’ syndrome and Simpson-Golabi-Behmel syndrome.

Other conditions that may be linked to Wilms tumor but do not display characteristics of overgrowth include Bloom’s syndrome and Denys-Drash syndrome.

Familial or sporadic

Although the majority of Wilms tumor cases do not appear to have a genetic link, some families have been identified to be at an increased risk of the disease due to a hereditary factor.

Gene mutations are usually passed on in an autosomal dominant pattern as a result of one of several currently known mutations, such as:

  • Mutation of chromosome 11 of the WT1 gene, which is responsible for the encoding the protein to down-regulate the growth factor IGF-11.
  • Mutation of chromosome 11 of the WT2 gene.
  • Mutation of chromosome 16 or 1p on WT3 gene.

Symptoms

As most Wilms tumors are localized to the kidneys and often on one side, symptoms may take some time to emerge and are usually associated with this area of the body.

Abdominal pain and swelling is common, particularly when the tumor grows to a considerable size and obstructs the body tissues. Urine discoloration may also be evident due to the effects of the tumor.

More generalized symptoms, such as fever, nausea, vomiting and diarrhea, may also present. This often leads to a loss of appetite and a general feeling of malaise. Hypertension is also an indicative symptom of the disease, which presents due to the malfunction of the kidney and renal system associated with the tumor.

Categorization by prognosis

The prognosis of Wilms tumor depends on the type of cells present and whether they display anaplastic characteristics. Anaplasia refers to cell defects with large and distorted DNA in the cell nucleus, which is associated with a more aggressive disease and poorer prognosis.

Wilms tumor without anaplasia is termed favorable, as it tends to be managed more effectively and has a higher cure rate.

References

Further Reading

Last Updated: Mar 17, 2021

Yolanda Smith

Written by

Yolanda Smith

Yolanda graduated with a Bachelor of Pharmacy at the University of South Australia and has experience working in both Australia and Italy. She is passionate about how medicine, diet and lifestyle affect our health and enjoys helping people understand this. In her spare time she loves to explore the world and learn about new cultures and languages.

Citations

Please use one of the following formats to cite this article in your essay, paper or report:

  • APA

    Smith, Yolanda. (2021, March 17). Wilms Tumor Pathology. News-Medical. Retrieved on October 31, 2024 from https://www.news-medical.net/health/Wilms-Tumor-Pathology.aspx.

  • MLA

    Smith, Yolanda. "Wilms Tumor Pathology". News-Medical. 31 October 2024. <https://www.news-medical.net/health/Wilms-Tumor-Pathology.aspx>.

  • Chicago

    Smith, Yolanda. "Wilms Tumor Pathology". News-Medical. https://www.news-medical.net/health/Wilms-Tumor-Pathology.aspx. (accessed October 31, 2024).

  • Harvard

    Smith, Yolanda. 2021. Wilms Tumor Pathology. News-Medical, viewed 31 October 2024, https://www.news-medical.net/health/Wilms-Tumor-Pathology.aspx.

Comments

The opinions expressed here are the views of the writer and do not necessarily reflect the views and opinions of News Medical.
Post a new comment
Post

While we only use edited and approved content for Azthena answers, it may on occasions provide incorrect responses. Please confirm any data provided with the related suppliers or authors. We do not provide medical advice, if you search for medical information you must always consult a medical professional before acting on any information provided.

Your questions, but not your email details will be shared with OpenAI and retained for 30 days in accordance with their privacy principles.

Please do not ask questions that use sensitive or confidential information.

Read the full Terms & Conditions.