Kidney Tumors

Symptoms of Wilms tumor

Common signs and symptoms of Wilms tumor include:

• Lump or swelling in the belly
• Blood in the urine
• Belly pain
• High blood pressure
• Fever 
• Loss of appetite
• Weight loss
• Constipation
• Fatigue
• Excessive thirst
• Vomiting

Some children may be treated for constipation for a few months before they are diagnosed with Wilms tumor.

Risk factors for Wilms tumor

In most cases, there is no clear cause for Wilms tumor.

• It affects males and females equally.
• Females are more likely to have it in both kidneys at diagnosis.
• It is more common in Black children than White children.
• It is less common in Asian populations.
• About 10% of patients with Wilms tumor have hereditary conditions that increase risk. These include:
  • WAGR syndrome
  • Denys-Drash syndrome
  • Overgrowth syndromes (such as Beckwith-Wiedemann syndrome)

Treatment of Wilms tumor

If your child’s care team suspects your child has Wilms tumor, they will usually have surgery to remove the kidney.

If only 1 kidney is affected, the entire kidney is often removed. People can live normal, healthy lives with only 1 kidney.

If your child’s kidney tumor cannot be removed at diagnosis or if they have tumors in both kidneys, your child will receive chemotherapy before surgery.

If your child has Wilms tumor in both kidneys, they may be able to have surgery to remove only part of each kidney. They will receive more chemotherapy after surgery.

Treatment may also include radiation therapy, depending on your child’s condition.

Eligible patients may think about taking part in a clinical trial.

Prognosis for Wilms tumor

In general, Wilms tumor is a curable disease. Five-year survival rates for Wilms tumor patients with less aggressive histology are above 90% in the United States.

Prognosis depends on:

• Age at diagnosis. Patients diagnosed before age 10 usually have a better prognosis.
• Stage of cancer at diagnosis
• Pathology (how the cells look under a microscope) and/or molecular features of the tumor. The cells are identified as favorable or unfavorable.
  • Unfavorable histology (also known as anaplasia) means there are more cancer cells that divide rapidly and look very different than normal cells. These cells are harder to treat and are linked to lower survival rates. They can be in one area (focal) or spread throughout the tumor (diffuse).
• Genetic factors and molecular markers of the tumor
• Whether the cancer has come back

Your child’s doctor is the best source of information on your child’s case. 

Renal cell carcinoma

Symptoms of renal cell carcinoma

Common signs and symptoms of RCC include:

• Lump or swelling in the belly
• Blood in the urine
• Belly pain

Other symptoms may include:

• High blood pressure
• Fever
• Loss of appetite
• Weight loss
• Constipation

Risk factors for renal cell carcinoma

Some inherited conditions put children at higher risk for renal cell carcinoma. These include:

• Von Hippel-Lindau disease
• Tuberous sclerosis
• Hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC)
• Other gene changes passed down in families

Renal medullary carcinoma is a rare type of renal cell carcinoma that may be linked to sickle cell disease.

Previous treatment for childhood cancers with radiation or chemotherapy is a risk factor for kidney tumors, specifically renal cell carcinoma.

Treatment of renal cell carcinoma

Surgery to remove the kidney and affected lymph nodes is the main treatment for renal cell carcinoma.

RCC does not respond well to radiation or chemotherapy. There is no standard chemotherapy or radiation therapy used for this cancer.

Small tumors may be treated with ablation to freeze or burn the cancer cells.

Immunotherapy may be used for people with advanced RCC or any RCC with a targetable lesion that cannot be completely removed.

Certain targeted therapies have also been used in adults with advanced RCC but have not been studied widely in children.

Eligible patients may think about taking part in a clinical trial.

Prognosis for renal cell carcinoma

The chance of cure for patients with renal cell carcinoma depends on:

• Stage of the cancer at diagnosis:
  • Stage 1: 90%
  • Stage 2: 80%
  • Stage 3: 70%
  • Stage 4: 15%
• Whether surgery can completely remove the tumor
• Whether the cancer is recurrent (has come back)

Your child’s doctor is the best source of information about your child’s case.

Malignant rhabdoid tumor of the kidney

A malignant rhabdoid tumor (MRT) most often occurs in young children and infants. These tumors are often not diagnosed until the disease is more advanced.

A rhabdoid tumor of the kidney can spread to the brain and lungs.

Occasionally, a rhabdoid tumor of the kidney will occur at the same time as a brain tumor called an atypical teratoid rhabdoid tumor.

Rhabdoid tumors have a common gene change. Genetic testing might be an option for your family after diagnosis.

Children may have this mutation in every cell in their body.

If your child has a rhabdoid tumor, you may meet with a genetic counselor and discuss testing both tumor and healthy cells for gene mutations that can cause these tumors to develop.

An MRT requires surgery. Chemotherapy medicines can be used. Radiation may also be used in some cases. But surgery is the main treatment. Researchers are looking at other options through clinical trials.

Older patients with rhabdoid tumors tend to have a better chance of recovery. The overall survival rate for children with MRT is less than 50% in the United States. Children with disease that has spread through the body have a poor long-term outlook.

Your child’s doctor is the best source of information about your child’s case. 

Clear cell sarcoma of the kidney

Clear cell sarcoma of the kidney (CCSK) is quite rare. It makes up less than 3% of pediatric kidney tumors. Only about 20 children are diagnosed with clear cell sarcoma in the United States each year.

It is most often found in children ages 1–4 years. It occurs more often in boys.

Compared to other kidney tumors, CCSK is more likely to spread to bone. But less than 5% of patients have cancer that has spread when they are diagnosed.

Common sites for spread of disease include:

• Lung
• Bone
• Brain
• Soft tissue

Treatment of CCSK includes surgery to remove the kidney. Treatment can also include chemotherapy and radiation after surgery. In the future, other treatment options such as targeted therapies may be an option.

Children treated for CCSK are at risk for the tumor to return later in life. The most common sites for recurrence are the brain and lungs.

Younger age and greater extent of disease at diagnosis are linked to less favorable outcomes. Your child’s doctor is the best source of information about your child’s case.

Mesoblastic nephroma or congenital mesoblastic nephroma

Mesoblastic nephroma occurs most often in newborns and infants. The main symptom is a lump or mass in the belly.

About 90% of patients are diagnosed before age 1. It is more common in boys. Sometimes a diagnosis is made before birth through prenatal ultrasound.

Younger age (less than 3 months) is linked to better outcomes for patients with mesoblastic nephroma.

There are 2 major types of mesoblastic nephroma:

• Classic mesoblastic nephroma: Diagnosed before birth or before 3 months of age.
• Cellular mesoblastic nephroma: Linked to genetic translocation. It has a greater risk of recurrence.

Surgery to remove the kidney is the main treatment. In some cases, chemotherapy may also be used. 

Targeted therapies for tumors with genetic factors are being studied in clinical trials.

The survival rate for mesoblastic nephroma is greater than 95% in the United States. Your child’s doctor is the best source of information about your child’s case.