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Wilms' Tumor

Definition


Disease: Wilms' Tumor Wilms' Tumor
Category: Tumors

Disease Definition:

Wilms’ tumor, also called nephroblastoma, is an uncommon kidney cancer that mainly affects kids. Wilms’ tumor is the most common and nasty kidney cancer in children since it could affect either or both of the kidneys. The climax time for Wilms’ tumor is the age of three, but it could occur after the age of 8 in some rare cases. The tumor’s name is attributed to the German surgeon who discovered cancer developing from immature kidney cells. Recently, the outlook of children with Wilms’ tumor has changed a lot. Determining the expansion of the cancer and planning the treatment is improved with the help of improved imaging techniques.
 

Work Group:


Symptoms, Causes

Symptoms:

A mass could be felt in a child’s abdomen during showers, normal dressing or during a routine physical examination. The mass could feel firm to touch and could be felt on either of the child’s abdomen. It’s hard to detect Wilms’ tumor in early stages simply because the cancer can grow in size without causing any pain. The child might appear healthy or he/she might experience:

 

  • Fever
  • An abdominal mass
  • Loss of appetite
  • Loss of weight
  • Stomach ache
  • High blood pressure
  • Blood in the urine
  • Constipation
  • Dizziness
  • Vomiting
  • General discomfort
     

Causes:

Wilms’ tumor affects the kidneys, which are the filtering machine of the body. The two bean-shaped organs near the middle of the back clean the blood and keep up normal levels of water and electrolytes; minerals such as sodium and potassium, in the body. Blood coming through the renal arteries to the kidneys is filtered out of waste products, extra electrolytes and water which are ultimately sent out of the body as urine. The urine leaves the kidneys through two tubes, flows to the bladder then leaves it through a narrow tube called the urethra.

 

Doctors believe that Wilms’ tumor can arise when the child is still a fetus.  Some cells that are meant to form the kidneys fail to develop and take form. Yet, they multiply in their primitive form to become a tumor. The tumor is usually detected between ages 1 and 5. The mass formed by the tumor can distort kidneys and compress normal tissue into a rim. Moreover, it could cause bleeding in the kidneys and urethra, giving the urine its bloody color. Mostly, cancers occur after gene damage. The genes program cells to grow, develop, mature then die. When changes occur in the genes that control growth, cancer develops. This change allows cells to increase without limits. In Some cases of Wilms’ tumor, the genetic defects pass from parent to child. Some other cases are related to defects in one of two genes; either Wilms’ tumor 1 (WT1) or Wilms’ tumor 2(WT2). WT1 is necessary to the development of the genitals and urinary system. Scientists think that changes in other chromosomes also play a role in this cancer.
 

Complications

Complications:

None

Treatments:

Surgery and chemotherapy are the typical treatment of Wilms’ tumor. The stage of the tumor and the form of the cancer cells under the microscope helps in determining whether the child needs radiation therapy or not.

 

SURGERY:

The surgical elimination of the kidney tissue is called nephrectomy. Some of the types of nephrectomy are:

 

Simple nephrectomy:

In this type, the surgeon removes the entire kidney. Thus, the remaining kidney can boost its ability then take control of the other’s entire job; filtering the blood.

 

Partial nephrectomy:

In this method the tumor and part of the kidney tissue surrounding it are removed. This is usually performed when the other kidney is impaired or is already removed.

 

Radical nephrectomy:

Here, the doctors remove the kidney, the tissue surrounding it and maybe the neighboring lymph nodes too.

 

In case of surgery, the doctor may check both kidneys and the abdominal cavity for evidence of cancer. Also, sampling kidneys, lymph nodes, and any tissues that appear unnatural under the microscope helps in identifying cancer cells. A pathologist can check the child’s tumor cells and see if the cancer is aggressive or open to chemotherapy.

 

CHEMOTHERAPY:

Medications are used in chemotherapy in order to kill the cancer cells. It could be given in the form of pills, intravenous (IV) line, or injections. Chemotherapy enters the bloodstream and travels throughout the body to kill cancer cells wherever they are and that’s why it’s considered systemic. Rapidly dividing cells are affected by chemotherapy. So, normal cells with fast turnover, such as hair follicles, cells in the gastrointestinal tract and bone marrow, which is the tissue at the core of bone that manufactures blood cells, are also affected as much as cancer cells. As such, these medications can have side effects such as nausea, vomiting, loss of hair, loss of appetite and low white blood cell count. Generally, side effects stop after the drug is stopped, and some of them might improve during therapy. Parents should consult their child’s doctor about the side effects that could occur during the treatment.
Giving high doses of chemotherapy can destroy the bone marrow. Although it’s not standard, but if the child is to undergo high-dose chemotherapy, his/her doctor might suggest removing and freezing the marrow in advance. After chemotherapy, the marrow is returned through IV line in a procedure called autologous bone marrow reinfusion.

 

RADIATION THERAPY:

This method uses X-rays or other high-energy rays to destroy cancer cells. The radiation therapy is usually used a few days after the surgery. If the child is young, he/she might need a tranquilizer to remain still during therapy. A radiation therapist will mark the treatment area with a special dye and cover the area that shouldn’t receive treatment. Side effects may include skin irritation, nausea and fatigue. After treatment, parents have to apply a sunscreen lotion to the child’s skin in order to avoid sunburn. After abdomen radiation treatment, diarrhea might occur.  Parents could ask the doctor to suggest a nonprescription or prescription medication for relief.

 

TREATMENT REGIMENS BY STAGE:

Child’s treatment depends on the cancer stage, the type of cancer cells (histology), the child’s age and general health.

 

Stage 1 or 2 cancer:

When cancer is bound to the kidney or the nearby structures and the cell type is not aggressive, the child will undergo removal of the affected kidney and tissues and some of the lymph nodes near the kidney. The removal surgery is followed by chemotherapy. Radiation may be used in some cases of stage 2 cancer.

 

Stage 3 or 4 cancer:

When cancer has already expanded within the abdomen with the incapacity of removing it totally without jeopardizing structures like major blood vessels, radiation will be added to surgery and chemotherapy. Also, the child might have chemotherapy before the surgery in order to shrink the tumor.

 

Stage 5 cancer:

A portion of the cancer form both kidneys will be removed, in addition to neighboring lymph nodes to be checked if they contain tumor cells in case the tumor cells are found in both kidneys. Chemotherapy is used to shrink the remaining tumor. Surgery is repeated to remove as much tumor as possible while leaving functioning kidney tissue. More chemotherapy and radiation therapy might follow.

 

Children’s reactions to therapies differ. So, modifications could be necessary. Parents should be acquainted with the doctor’s therapy plan and make sure they know the pros and cons before giving their consent. They should also ask about and report side effects. The prognosis depends totally on the stage and cell type of the tumor.
 

Prognosis:

Not available

Expert's opinion

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Dr. Faisal Dibsi

Dr. Faisal Dibsi Specialist of Otolaryngology - Head and Neck Surgery

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