What are Wilms Tumors?

Wilms tumors are a type of kidney cancer that is found in children. Cancer begins when cells grow out of control, forming lumps called tumors, which can either be benign (noncancerous) or malignant (cancerous). The American Cancer Society estimates that 90% of kidney cancers in children are Wilms tumors.

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The kidneys are two bean-shaped organs that filter blood to remove excess salt, water, and wastes; control blood pressure; and help regulate red blood cell production. An adrenal gland sits on top of each kidney, which is responsible for hormone production. The kidneys and adrenal glands are surrounded by a Gerota's fascia and are protected by the lower rib cage.

Most Wilms tumors are unilateral or only occur in one kidney at a time. Only 5-10 percent of Wilms tumor cases are bilateral or found in both kidneys. Most of these tumors are found before they have metastasized or spread to other organs, but not before they have become larger than the kidney itself. While it is not optimal, it is possible to live with only 1 functioning kidney.

Types of Wilms Tumors

Wilms tumors are classified based on how the tissues look under the microscope (histology):

Favorable Histology

The cancer cells present in the tumors appear abnormal, but they are not anaplastic, and the prognosis is quite good.

Anaplastic Histology

These tumors have cells and tissues that vary in appearance, and the nucleus tends to be distorted and large. This characteristic is known as anaplasia. If the anaplasia is spread throughout the tumor (diffuse anaplasia), it is harder to treat compared to when anaplasia is just limited to certain parts (focal anaplasia).

Other Types of Kidney Cancers in Children

These are even rarer types of kidney cancer and may have different diagnostic and treatment requirements:

  • Mesoblastic nephroma
  • Clear cell sarcoma of the kidney (CSSK)
  • Malignant rhabdoid tumor of the kidney
  • Renal cell carcinoma

Wilms Tumor Symptoms

The following symptoms may indicate the presence of Wilms tumors in your child:

  • Abdominal swelling or a large abdominal lump
  • Fever
  • Nausea
  • Appetite loss
  • Shortness of breath
  • Constipation
  • Blood in the urine
  • High blood pressure

These are not exclusive to Wilms tumors and may be caused by other more common conditions. But it is important to immediately seek medical help if your child is experiencing any of these symptoms, especially if the child has any of the inherited diseases that increase Wilms tumor risk.

Wilms Tumor Risk Factors

Risk factors are things that increase the likelihood of getting cancer. They can be immutable or beyond your power to change them, like age, sex, race and inherited conditions or syndromes. They can also be lifestyle-related, such as vices, environment, frequent activities, and workplace-related.

Immutable Risk Factors

Wilms tumors are usually found in children ages 3-4 years old. They become less common as the child grows, and are rarely found in adults. There are more cases of Wilms tumors in African American children than in children of other ethnicities in the US. Wilms tumors are also slightly more common in girls than in boys.

Family History

Children can inherit chromosomal abnormalities that can trigger the development of Wilms tumors. About 1-2% of Wilms tumor cases are found in children who had relatives with the same type of cancer. Having these genetic risks also slightly increases the chance of getting bilateral tumors (in both kidneys).

Genetic Syndromes and Birth Defects

While there is no strong link between birth defects and inherited syndromes, about 1 in 10 children who have Wilms tumors also have the following abnormalities:

WAGR Syndrome

This syndrome is a genetic disorder characterized by a combination of physical and mental problems, including:

  • Wilms tumor; 
  • Aniridia (complete or partial lack of the iris in the eyes); 
  • Genitourinary tract abnormalities; and 
  • Mental Retardation. 

Children with this syndrome have a 30% to 50% chance of developing Wilms tumor. The cells in children with WAGR syndrome are missing part of chromosome 11, where the WT1 gene is normally found. These children tend to develop Wilms tumors at an earlier age and often have tumors in both kidneys.

Denys-Drash Syndrome

This is a rare disorder linked to mutations in the WT1 gene. In this syndrome, the kidneys become diseased and stop functioning at a very young age. Wilms tumors usually develop in these diseased kidneys. The reproductive organs also do not develop normally, leading to potential misidentification of gender in boys. Due to the high risk of Wilms tumors, doctors often recommend removing the kidneys soon after the diagnosis of Denys-Drash syndrome.

Frasier Syndrome

This is another rare disorder associated with WT1 gene mutations. It also involves kidney disease, but the kidneys usually continue to function into adolescence. As with Denys-Drash syndrome, the reproductive organs do not develop normally. Children with Frasier syndrome have an increased risk of Wilms tumors, as well as an even higher risk of cancers in the reproductive organs.

Beckwith-Wiedemann Syndrome

Children with Beckwith-Wiedemann syndrome tend to be larger than average for their age, with enlarged internal organs and often an enlarged tongue. They may have hemihypertrophy, an oversized arm and/or leg on one side of the body, and other medical problems. These children have about a 5% risk of developing Wilms tumors or, less often, other childhood cancers. Beckwith-Wiedemann syndrome is caused by a defect in chromosome 11 that affects the WT2 gene.

Other less common syndromes and birth defects that increase Wilms tumor risk:

  • Perlman syndrome
  • Sotos syndrome
  • Simpson-Golabi-Behmel syndrome
  • Bloom syndrome
  • Li-Fraumeni syndrome
  • Trisomy 18
  • Aniridia
  • Hemihypertrophy
  • Cryptorchidism
  • Hypospadias

If you or a loved one have suffered from a misdiagnosis or delayed diagnosis for your child's Wilms tumors or from negligence during their treatment that caused them more harm, contact us at 833-PORTER9, or e-mail us at info@porterlawteam.com to discuss the details of our experience representing other clients and the results we were able to obtain in the past for clients who are suffering as you are. In many ways, our results speak for themselves, and we will stand ready to help you and your family in your time of greatest need.

Last Updated on April 4, 2024 by Michael S. Porter
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