Wilms Tumor Overview

Wilms tumor, also known as nephroblastoma, is a type of cancer that develops in the kidneys of children. It is the most common kidney cancer in children and the fourth most common overall.

The exact cause of Wilms tumor is unknown, but it's believed to arise due to genetic alterations impacting the normal development of the urinary system.

Wilms tumor is most commonly diagnosed in children under five years old, making it a primarily childhood cancer.

The tumor is named after German physician Dr. Max Wilms, who first described it in 1899.

Common symptoms include an abdominal mass, pain, and blood in the urine. These are the most frequent signs of Wilms tumor.

Treatment usually involves a combination of surgery, chemotherapy, and radiation therapy. These treatments are chosen based on the stage and characteristics of the tumor.

Genetic markers linked to Wilms tumor include WT1, CTNNB1, and WTX. These genes are involved in the developing urinary system.

The prognosis of Wilms tumor is generally good, especially with early detection and treatment. However, factors like stage and specific gene mutations can influence the outcome.

A type of renal disease that starts with protein in the urine and can progress to kidney failure.

Immediately after surgery, patients must have their blood pressure, electrolytes, temperature, and blood sugar levels monitored.

A type of cancer that affects the kidneys, often developing in children.

Patients with Wilms tumor usually have follow-up visits every 3 months for the first 2 years after diagnosis.

A syndrome characterized by a range of physical abnormalities, including enlarged kidneys, a large tongue, and an extra body part.

A specific type of Wilms tumor that has a generally better outcome.

After 2 years, Wilms tumor follow-up visits become less frequent, occurring every 6 months for another 2 years.

During follow-up visits for Wilms tumor, doctors might check for signs of tumor recurrence or spread by feeling the abdomen, listening to the lungs, and examining relevant areas.

The most primitive and potentially aggressive component of a Wilms tumor.

The part of a Wilms tumor that contains developing kidney structures, resembling different stages of kidney formation.

Monitoring for side effects from treatment, such as peripheral neuropathy (nerve damage) from Vincristine and liver problems from radiation therapy, is important during follow-up.

The connective tissue part of a Wilms tumor, which can be either dense or loose.

The presence of abnormal kidney tissue that can lead to a higher risk of developing Wilms tumor in the other kidney.

A type of Wilms tumor with a poorer prognosis due to its increased cell abnormality.

The abnormal cell growth and structure found in "Unfavorable Histology" Wilms tumors.

The loss of genetic material at specific locations on chromosomes 1p and 16q, which can indicate a worse prognosis in Wilms tumors.

Presence of different types of tissue cells, such as muscle, fat, or cartilage, in a Wilms tumor, often seen after chemotherapy.

The most common initial symptom of Wilms tumor, often presenting as a painless swelling.

Blood in the urine, which can be a symptom of Wilms tumor.

Elevated blood pressure that occurs in about a third of Wilms tumor patients.

Lab tests that assess the function of kidneys, including blood chemistry and urine analysis.

Anaplastic histology is associated with a poorer prognosis in stage II to IV Wilms tumors.

Diffuse anaplasia indicates a more widespread abnormal cell development, leading to a worse prognosis compared to focal anaplasia.

Loss of heterozygosity (LOH) at specific chromosome locations and the presence of the TP53 gene mutation contribute to a poorer prognosis.

Wilms tumor patients often have increased bone, breast, colon, and thyroid cancer risk due to radiation therapy.

Chemotherapy for Wilms tumor may lead to secondary malignancies as well as specific toxicities like hearing loss, heart dysfunction, and nerve issues.

The Children's Oncology Group (COG) is a large clinical trials group focused on childhood cancers, including Wilms tumor.

The COG has conducted several national studies (National Wilms Tumor Studies 1-5) to improve understanding and treatment of Wilms tumor.

The COG studies on Wilms tumor have enrolled a large number of patients, allowing for analysis of various outcomes, including survival rates based on different factors like tumor stage or metastasis.

A focus of COG research is investigating the impact of different medical imaging techniques on survival rates in patients with Wilms tumor.

Wilms tumor patients with bone metastasis have a specific outcome which is studied by the COG.