Wilms tumor : Finding cancer specialists for Wilms tumor treatment

A syndrome is a collective group of either symptoms, or signs, or particular malformations that are seen together in the same person.

WAGR syndrome is also a syndrome or a collective group of symptoms. WAGR stands for the first alphabet or letter of the medical conditions seen in this syndrome (although not all symptoms may be seen together).

1. W- Wilms tumor : Wilms Tumour (also known as nephroblastoma) is basically an embryonic tumour of the KIDNEY. It occurs mostly in children under the age of 5 years. There is a strong link between Wilms tumour and particular birth defects.
2. A- Aniridia : It could either be a total or partial absence of the iris (or the coloured part) of the eye pupils.
3. Genitourinary tract abnormalities : These vary and range from defects of the either kidneys and the urinary tract, or of the reproductive organs like the penis, scrotum, testicles, or ovaries.
4. Mental Retardation

What are the other names for WAGR syndrome ?

• Wilms’ Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome.
• It is also called WAGR Complex.
• Chromosome 11p deletion syndrome.
• 11p deletion syndrome.

What do we know about the kidney ?
We all have a general idea about the kidney. It is a part of the urinary system. It is a paired organ in the right and left side of the abdomen, its job being to make urine and excrete it out of the body.Urine is first made by the bean-shaped kidneys and is then carried to the urinary bladder through thin tubes called the ureters. As you urinate, the bladder contracts and the urine thus is forced to flow out of the bladder through another connecting tube called the urethra, and then to the outside of the body.The kidneys have some additional work as well, such as regulating & controlling blood pressure by producing the hormone ‘renin’. What’s more, they synthesize erythropoietin. It is erythropoietin that makes the bone marrow produce additional red blood cells.

What are the causes of Wilms tumour ?
Children with the WAGR syndrome have a much higher risk of developing a Wilms tumor. The chromosomal make-up in children with WAGR syndrome is slightly different. The chromosome 11, where the WT1 gene is normally found, is missing. Children with WAGR tend to get Wilms tumors at a much earlier age as well as develop tumours in both the kidneys bilaterally.

Wilms tumours, also known as nephroblastoma, occur mostly in children under the age of 5 years. There is a strong link between Wilms tumour and certain kinds of birth defects. Most birth defects linked to Wilms tumour occur in syndromes. A syndrome is a collective group of either symptoms, or signs, or particular malformations that are seen together in the same person. Syndromes linked to Wilms tumor include:

1. Beckwith-Wiedemann Syndrome
2. Denys-Drash syndrome
3. WAGR syndrome

What are the types of Wilms Tumours ?
Wilms tumors are categorized into 2 major types based their appearance in a microscope (that is based on their histology).

1. The tumours with a favorable histology: These are better tumours, as chances of curing them are also good. There is no or minimal anaplasia. Most of the Wilms tumour cases have a favourable histology and therefore, good prognosis.
2. The tumours with an unfavorable histology (or anaplastic Wilms tumor): In these tumours, the cancer cells look large, distorted or anaplastic.

What are the clinical features of WAGR syndrome ?
Wilms tumors are the most common cancers in children that start in the kidneys. Most Wilms tumors are one-sided or unilateral, though they could be bilateral also.Many cases of Wilms tumours can be asymptomatic, though later with progress and growth of the tumour, symptoms like pain, malaise, hematuria or the presence of an abdominal mass can be noted. Due to excess production of rennin, hypertension could result.

• Wilms’ Tumor: About one half of individuals who have WAGR syndrome develop a specific type of kidney tumour called nephroblastoma or Wilms’ tumor. Many cases of Wilms tumours can be asymptomatic, though later with progress and growth of the tumour, symptoms like pain, malaise, hematuria or the presence of an abdominal mass can be noted. Due to excess production of rennin, hypertension could result.
• Aniridia: In infants who are born with aniridia in WAGR syndrome, the irises of the eyes fail to develop normally before birth. This causes partial or complete absence of the round coloured part of the pupil of the eye (iris).
  Other eye problems could be cataract of the eye lens, rapid, involuntary movements or nystagmus and high pressure of eye fluid or glaucoma which could affect vision.
• Genital and Urinary (GU) problems: The urinary tract might open in the penis shaft than at the penis tip or testes that are still in the abdomen and have not descended (that is cryptorchidism), in boys. In girls, underdeveloped (streak) ovaries, and developmental malformations of the uterus, fallopian tubes or vagina might be seen. Individuals with WAGR syndrome may have an increased risk for gonadoblastoma, a cancer of the gonads.
• Mental Retardation: The affected children might have a range of developmental delays. The severity of the condition varies from affected person to person.

How is WAGR syndrome diagnosed ?
A genetic test known as a karyotype and a more specific test, the Fluorescent In- Situ Hybridization or FISH, is done to locate the deleted area (11p13) of genes on the chromosome 11.

1. A complete physical examination of the body is mandatory.
2. A test can be done for Catecholamines: To rule out neuroblastomas.
3. Urinalysis: One way to test is to check and examine for blood in the urine (called hematuria). This can be tested by a urinalysis, which is an uncomplicated test to check for blood in a urine sample.
4. Urine Cytology: In this test, the doctor looks for cancer cells under a microscope in urine.
5. Cytoscopy: The urinary tract can be viewed with a flexible scope.
6. Imaging Tests: To determine the exact extent of disease, additional tests may need to be performed so doctors can view, assess and judge the cancer and determine how far it has progressed or spread. These important diagnostic tests include the following such as X- rays, CT Scan, MRI Scan or PET Scans.A urologist can recommend specific radiographic imaging techniques like an intravenous pylegram (IVP) or a CT urogram. In the IVP, dye or contrast media is combined with conventional x-ray technology to assess the urinary tract system for any abnormalities. In the CT urogram, a CT scan with contrast is used to examine the kidneys, ureters, and bladder. This is the recommended procedure nowadays.
7. Biopsy : Surgically removed tissue examined in a microscope by a pathologist gives the definitive Wilms tumour diagnosis.

How are Wilms tumour staged ?
Grade and Stage describe the kidney tumour, helping to provide guidance for the urologist in choosing the best treatment option(s). Staging is to determine the extent and spread of the cancer. The higher the stage the further the cancer has progressed or grown away from its primary site in kidney. Grade refers to what the cancer cells look like, and how much they resemble their cell of origin or differentiation. The higher the grade is, the more destructive the tumour is.

How is WAGR syndrome treated ?

1. Wilms’ Tumor: Children with WAGR syndrome must have ultrasonography of their abdomen at the time of birth itself. After that, abdominal ultrasounds are recommended every three months, until the age of 8 years. A keen watch should be kept out for signs and symptoms of Wilms tumor like fever, loss of appetite or anorexia, sudden weight loss, lack of energy or lethargy or abdominal swelling. Wilms’ tumor can often be treated successfully, if found early. The treatment includes surgery to remove the kidney, radiation therapy & chemotherapy.
2. Aniridia: The treatment of aniridia is for maintaining the affected person’s vision. Drugs or surgery will be of use when there is glaucoma or cataracts.
3. Genital and Urinary problems: Children with WAGR syndrome need to have regular abdominal evaluations to detect abnormal or delayed development of their ovaries or testes. Surgery may be needed for removing the abnormal gonads or for preventing gonadal cancer (that is gonadoblastoma).
4. Mental Retardation: Children with WAGR syndrome should be referred for vision therapy, physical therapy, as well as occupational and speech therapies. Specialized education should be given to these children to develop to their fullest capabilities.

What is the care to be taken after the removal of a Wilms Tumour ?
Your child’s health care team will formulate and discuss a follow-up schedule with you, which encompasses physical examinations and imaging tests (chest X-rays, ultrasounds, MRIs and CT scans) to be on the look-out of the tumor growing or returning. If your child had part or all of his or her kidney removed, they will need blood and urine lab work-ups to check how well the rest of the kidney tissue is working. The recommended schedule for follow-up exams and tests will be determined by the stage and histology of the tumor, if there is the genetic syndrome related to the tumor, the treatment the child received and any other problems that the child may have had during treatment. Follow-up visits to the doctor and tests will have to be frequent at first (atleast 6 to 12 weeks for the first couple of years), but later as time passes, the time between visits may be extended. It is important for you to be aware and report old or new symptoms right away to your child’s doctor, so that the disease can be treated, as early as possible. Your child’s doctor will let you know what to watch out for. Children with severe or bilateral Wilms tumors (that is tumors in both kidneys) will need routine and repetitive tests to look out for early kidney failure (such as urine tests, blood pressure checks, and regular kidney function tests).

Are there any long-term effects of treatment ?
As treatment is advancing in strides, most children who are treated for Wilms tumor are now surviving into adulthood. Modern medicine has come to terms with the treatment of childhood cancer, care and follow-up after treatment all requiring a very specialized approach. The earlier the problem is recognized, the better it can be treated. It is imperative to discuss with your child’s medical team what possible side effects and new symptoms might ensue. The later effects of treatment can include diminished kidney function, heart or lung problems after chemotherapy or radiotherapy, retarded growth and development, changes in sexual development and fertility, and rarely, other cancers may develop.

What care will the parents or families have to take ?
A child’s health will affect parents and other family members emotionally as well as in other ways. Common family concerns through treatment include financial stresses, traveling to and staying near the cancer center/hospital, compromise at professional levels, and home schooling. Health care professionals, friends, family and social workers can help families sort through these issues.

Can WAGR syndrome be prevented ?
Genetic counselling is helpful for determining whether there may be an increased risk of having another child with WAGR syndrome.

How to find and reach cancer specialists for Wilms’ tumor treatment ?
Now you can find and reach cancer specialists for Wilms’ tumor treatment from different cancer hospitals and destinations on a single platform, Hinfoways. You can avail opinions and information from multiple cancer specialists, cost estimates for Wilms’ tumor treatment from different cancer hospitals, compare things and then choose a cancer specialist or a cancer hospital for Wilms’ tumor treatment.

Find, reach and choose a cancer specialist for Wilms’ tumor treatment on Hinfoways. Make an informed choice.

Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.