Wilms Tumor and Childhood Cancer

Ellie Li

Abstract

Childhood cancers encompass all aspects of society today, with the diagnosis becoming more used as time goes on. Wilms Tumor, also known as a nephroblastoma, is a cancer in the kidneys that is prevalent among children. It is named after a renowned German surgeon, Max Wilms, who first uncovered the hidden details of this disease. This article connects the dots between Wilms Tumors and childhood cancers, deciphering the cause and prevention for this specified disease.

Biological Basis

Wilms tumor, named after a renowned German surgeon, is a type of kidney cancer that mainly affects children. Also known as nephroblastoma, it’s the most common type of kidney cancer and accounts for about seven percent of all pediatric cancers. Wilms tumor most often affects children ages three to four and becomes less common in children after the age of five, but can still affect older children and even adults [1].

Approximately five hundred children in the United States are diagnosed with this tumor each year [2]. The tumor can be very large and may metastasize to other body tissues. The most common site of spread is the lungs, but lesions may also occur in the other kidney, the brain, and/or the bones [2]. It’s also important to note that Wilms' tumor is unrelated to adult kidney cancer. The tumor usually affects a single kidney, but approximately five to ten percent of children with Wilms tumor have both kidneys involved [3]. 

Most of the time, Wilms tumor is caused by a gene mutation [4]. Gene alterations are the fundamental cause of kidney cells developing into tumors. A small proportion inherits a gene for Wilms tumor but scientists have yet to discover its causes. Children with genetic syndromes such as Beckwith-Wiedemann syndrome and Denys-Drash syndrome have a higher chance of developing Wilms tumor [4]. Scheduling frequent ultrasounds in children who have these genetic syndromes is a crucial step to taking further precautions.

Signs and Symptoms

Wilms tumor is commonly characterized by abdominal pain and swelling, hypertension, hematuria, and large swollen veins across the abdomen [3]. Abdominal pain is the most common initial presenting symptom, followed by hypertension, and then hematuria [8]. Children with Wilms tumor typically also experience fatigue, loss of appetite, and frequent urinary tract infections. 

Hypertension in children with Wilms tumor may further be accompanied by frequent nosebleeds, headaches, and blood in the eye [5]. Fevers may also be present in children with Wilms tumor and may worsen further into the diagnosis.

Diagnosis and Treatments

Physicians will typically order tests to confirm the diagnosis of Wilms tumor in children, with an abdominal ultrasound being the most common. Abdominal ultrasounds use high-frequency sound waves and a computer to create pictures of internal organs, blood vessels, and tissues [3]. Urine tests and biopsies are also a common option to confirm diagnoses. These laboratory tests help evaluate kidney and liver function [3].  These tests allow doctors to uncover the diagnosis of Wilms tumor and predict how it will metastasize throughout the body. 

Treatments for Wilms tumor are based mainly on the stage of the cancer and whether its histology is favorable or anaplastic [6]. Tumors with favorable histology have more differentiated cancer cells, which means the cancer cells look and act more like normal cells [7]. In anaplastic tumors, the cancer cells vary widely and tend to be larger and more distorted. Other factors that influence treatment would be the child’s age, the tumor size, and if the tumor cells have certain chromosome changes [6]. 

In children with Wilms tumor, a small biopsy sample is often taken from the tumor to confirm the diagnosis and determine its histology [6]. Treatments can vary based on the individual and what stage the tumor is currently at. Chemotherapy and surgery is a common option for tumors at later stages. Usually, the tumor will shrink enough within several weeks so that there is still time for surgery. If not, radiation might be given as well [6].

Prognosis and Survival

With marked improvements in chemotherapy and technology, the overwhelming majority of affected children now survive. The addition of radiation has contributed significantly to the survival rate in comparison to surgery alone [8]. The five-year overall survival rate for Wilms tumor in children ages zero to fourteen years old is about ninety-six percent. This means that, on average, about ninety-six of children diagnosed with Wilms tumor are expected to be alive at least five years after their diagnosis. Most relapses for Wilms tumor occur within the first two years of obtaining treatment [9].  As with any cancer, prognosis and long-term survival can vary greatly from child to child, but most children with Wilms tumor can be cured of the disease.

Coping with Wilms Tumor

A cancer diagnosis can raise a multitude of emotions for both the family and the patient. Pediatric cancers are some of the most distressing experiences and can be a lot to take in [10]. It’s normal to feel upset, angry, or hopeless but it’s crucial to prioritize your mental well-being during this time.

Whether enrolling the child in therapy or talking to a child’s specialist nurse or doctors, it’s essential to have people there to support you. With recent technological advancements and the positive outlook of this disease, it’s hopeful to make a full comeback.

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References

[1] Wilms Tumor. (05/04/2024). Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/wilms-tumor/symptoms-causes/syc-20352655 Retrieved: 08/08/2024

[2] Wilms Tumor. Cincinnati Childrens. https://www.cincinnatichildrens.org/health/w/wilms-tumor  Retrieved: 08/08/2024

[3] Wilms Tumor (Kidney Tumor). Children’s Hospital of Philadelphia. https://www.chop.edu/conditions-diseases/wilms-tumor-kidney-tumor Retrieved: 08/08/2024

[4] Anzilotti, Amy. Wilms Tumors. Nemours Kids Health. https://kidshealth.org/en/parents/wilms.html#:~:text=Most%20of%20the%20time%2C%20Wilms,kids%20who%20are%20otherwise%20healthy. Retrieved: 08/08/2024

[5] Wilms Tumor. (06/13/2022). Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/23259-wilms-tumor  Retrieved: 08/08/2024

[6] Treatment by Type and Stage of Wilms Tumor. (10/17/2018). American Cancer Society. https://www.cancer.org/cancer/types/wilms-tumor/treating/by-stage.html#:~:text=In%20these%20children%2C%20a%20small,might%20be%20given%20as%20well. Retrieved: 08/08/2024

[7] Prognosis and Survival for Neuroblastoma. Canadian Cancer Society. https://www.mdanderson.org/cancerwise/depression-in-cancer-patients-what-you-should-know.h00-158833590.html Retrieved: 08/08/2024

[8] Leslie, Stephen. Wilms Tumor. (05/30/2023) The National Library of Medicine. https://www.ncbi.nlm.nih.gov/books/NBK442004/#:~:text=With%20marked%20improvements%20in%20chemotherapy,specific%20syndromes%20including%20WAGR%20syndrome. Retrieved: 08/08/2024

[9] Survival Statistics for Wilms Tumor. Canadian Cancer Society. https://cancer.ca/en/cancer-information/cancer-types/wilms-tumour/prognosis-and-survival/survival-statistics#:~:text=The%205%2Dyear%20overall%20survival,2%20years%20of%20finishing%20treatment. Retrieved: 08/08/2024

[10] Coping with Wilms Tumor. Cancer Research UK. https://www.cancerresearchuk.org/about-cancer/childrens-cancer/wilms-tumour/coping Retrieved: 08/08/2024