Wilms Tumor

Wilms Tumor

Wilms tumor, also known as nephroblastoma, is a type of cancer that primarily affects children. It typically originates in one or both kidneys, and can spread to other parts of the body if left untreated.

The cause of Wilms tumor is not fully understood, but it is believed to result from the abnormal growth of cells in the developing kidneys during fetal development. Certain genetic mutations and syndromes, such as WAGR syndrome and Beckwith-Wiedemann syndrome, can also increase the risk of developing a Wilms tumor. Even thpugh, the prognosis for Wilms tumor is generally favorable, with a high cure rate if detected and treated early among children who are facing this fatal disease.

Types of Wilms Tumor

There are several types of Wilms tumors, which are distinguished based on their microscopic appearance and genetic characteristics.

The types of Wilms tumors include –

1. Favorable histology – This is the most common type of Wilms tumor, accounting for approximately 90% of cases. Favorable histology tumors are characterized by a well-defined structure and a low degree of aggressiveness.
2. Anaplastic histology – This is a less common type of Wilms tumor, accounting for approximately 10% of cases. Anaplastic histology tumors are characterized by a high degree of aggressiveness and a less well-defined structure.

Wilms Tumor

3. Diffuse anaplastic histology – This is a rare and very aggressive type of Wilms tumor. It is characterized by the spread of anaplastic cells throughout the kidney and surrounding tissues.

4. Clear cell sarcoma of the kidney – This is a rare type of kidney cancer that is sometimes misdiagnosed as a Wilms tumor. It is characterized by the presence of clear cells in the tumor.

5. Rhabdoid tumor of the kidney – This is also a rare and very aggressive type of kidney cancer that occurs primarily in young children. It is characterized by the presence of rhabdoid cells in the tumor.

The treatment and prognosis determined by an expert doctor for Wilms tumor are dependent on the type and stage of the tumor, as well as the age and overall health of the patient.

Causes and Risk Factors

The exact cause of Wilms’s tumor is not yet known, but some factors may increase the risk of developing this condition. These include –

1. Genetic predisposition – Certain genetic syndromes, such as WAGR syndrome, Beckwith-Wiedemann syndrome, and Denys-Drash syndrome can increase the risk of developing a Wilms tumor.
2. Age – Wilms tumor typically affects children under the age of 5, with most cases occurring between the ages of 2 and 3.
3. Gender – Wilms tumor is slightly more common in girls than in boys.
4. Race – Wilms tumor is more common in African American children than in children of other races.
5. Family history – Children with a family history of Wilms tumor have an increased risk of developing the condition themselves.
6. Prenatal factors – Some studies have suggested that prenatal exposure to certain chemicals or medications may increase the risk of Wilms tumor.
7. Birth weight – Children who are born with a high birth weight have a slightly increased risk of developing Wilms tumor.
8. Congenital anomalies – Children born with certain birth defects, such as aniridia (absence of the iris in the eye) and hemihypertrophy (asymmetrical overgrowth of one side of the body), are at an increased risk of developing Wilms tumor.
9. Environmental factors – Some studies suggest that exposure to certain environmental factors, such as radiation, chemicals, and pesticides, may also increase the risk of Wilm’s tumor.

It’s important to note that having one or more of these risk factors does not necessarily mean that a child will develop a Wilms tumor, and the majority of cases occur without any known risk factors. If you’re concerned about your child’s risk of developing Wilms tumor, talk to your doctor for necessary guidance and precautions.

Symptoms

Wilms tumor, also known as nephroblastoma, is a type of childhood kidney cancer that usually occurs in children under the age of 5. The following are some common symptoms of Wilms tumor –

1. Abdominal swelling or mass – It is the most common symptom of Wilms tumor that leads to painless abdominal swelling or mass that can be felt by the child or parent.
2. Abdominal pain – Children with Wilms tumor may experience abdominal pain or discomfort, which can be intermittent or constant.
3. Blood in urine – Some children with Wilms tumor may have blood in their urine, which may be visible or only detected through a urine test.
4. High blood pressure – Wilms tumor can cause high blood pressure, which may lead to headaches, dizziness, or vision changes.
5. Fever – A low-grade fever may occur in some children with Wilms tumor.
6. Loss of appetite or weight loss – Children with Wilms tumor may experience a loss of appetite, weight loss, or fatigue.

Many of these symptoms can also be caused by other conditions, and not all children with Wilms tumor will experience all of these symptoms. If you suspect your child may have a Wilms tumor or any other medical condition, it is important to seek medical attention promptly.

Diagnosis of Wilms Tumor

The diagnosis of Wilms tumor usually involves a combination of medical history, physical examination, imaging tests, and biopsy.

1. Medical History – The doctor will ask questions about the child’s medical history and any symptoms they may be experiencing.
2. Physical Examination – The doctor will perform a physical examination to check for any signs of a tumor in the abdomen.
3. Imaging Tests – Imaging tests are used to look for abnormalities in the kidneys and other organs. These may include –
   • Ultrasound – An ultrasound uses sound waves to create images of the kidneys and other organs.
   • Computed Tomography (CT) Scan – A CT scan uses X-rays to create detailed images of the kidneys and surrounding organs.
   • Magnetic Resonance Imaging (MRI) – An MRI uses a strong magnetic field and radio waves to create detailed images of the kidneys and surrounding organs.
4. Biopsy – A biopsy is the removal of a small sample of tissue from the tumor for examination under a microscope. This is done to confirm the diagnosis of Wilms tumor and to determine the type and stage of cancer.

If Wilms tumor is suspected based on the above tests, the child has to likely undergo further testing to determine the extent of cancer and whether it has spread to other parts of the body. This may include blood tests, bone scans, and other imaging tests. A team of doctors and specialists will work together to determine the best treatment plan for the child based on their specific case.

Staging of Wilms Tumor

The staging system commonly used for Wilms tumors is the National Wilms Tumor Study Group (NWTSG) staging system.

The NWTSG staging system for Wilms tumor is as follows:

Stage I – The cancer is limited to the kidney and can be completely removed by surgery.

Stage II – Cancer has spread beyond the kidney to nearby structures, such as the renal vein or the peritoneum, but can still be completely removed by surgery.

Stage III – Cancer has spread beyond the kidney to nearby lymph nodes, cannot be completely removed by surgery, or has spread to other organs such as the lungs, liver, bone, or brain.

Stage IV – Cancer has spread to distant sites such as the lungs, liver, bone, or brain, and cannot be completely removed by surgery.

Stage V -The cancer is present in both kidneys at the time of diagnosis.

The staging of Wilms tumor is important for determining the appropriate treatment, including surgery, chemotherapy, and radiation therapy. The prognosis of Wilms tumor varies depending on the stage of diagnosis, but overall the prognosis proves to be beneficial, especially for early-stage disease.

Treatment Options for Wilms Tumor

The specific treatment plan for Wilms’s tumor is dependent on several factors, such as the size and stage of the tumor, whether it has spread to other parts of the body, and the overall health of the child.

1. Surgery – Surgery is usually the first step in treating Wilms tumor. The goal of surgery is to remove as much of the tumor as possible while preserving the function of the kidney. In some cases, the entire affected kidney may need to be removed. If cancer has spread to other parts of the body, surgery may be used to remove these areas as well.
2. Chemotherapy – Chemotherapy involves the use of drugs to kill cancer cells. It is typically used in conjunction with surgery to shrink the tumor before it is removed or to kill any remaining cancer cells after surgery. Chemotherapy may also be used if cancer has spread to other parts of the body.
3. Radiation therapy – Radiation therapy uses high-energy X-rays or other types of radiation to kill cancer cells. It may be used after surgery to kill any remaining cancer cells or to treat cancer that has spread to other parts of the body.
4. Targeted therapy – Targeted therapy is a newer type of cancer treatment that uses drugs to target specific molecules or pathways involved in the growth and spread of cancer cells. This approach may be used in some cases of Wilms tumor, particularly if cancer has not responded to other treatments.

Prevention

Wilms tumor is difficult to prevent from happening in an individual. If a child has any of the medical condition mentioned above than it is recommended to consult your heathcare provider who might suggest doing kidney ultrasounds to discover something unusual that can lead to cancer. These ultrasound helps in finding deadly disease at an early stage for saving of lives.

Clinical trials 

Clinical trials are research studies that test new treatments for cancer. Children with Wilms tumor may be eligible to participate in clinical trials to receive experimental treatments that are not yet available to the general public.

It is important to work closely with a pediatric oncologist who specializes in the treatment of Wilms tumor to determine the best course of treatment for each individual child.