Disease

Brief Summary

Wilms tumor is the second most common intra abdominal cancer of childhood and the fifth most common pediatric malignancy overall. It represents approximately 6% of all pediatric cancers and accounts for more than 95% of all tumors of the kidney in the pediatric age group.

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Child with Turner syndrome

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Frequency

This condition varies among populations. Americans have a higher-than-average risk of contracting this disease and Asians have a lower-than-average risk. It affects 1 in 10,000 children in Europe and North America. While in the United States, 500 children develop Wilms tumor per year.

Brief Summary

History of Disease

Inheritance

Wilms tumor is inherited in an autosomal dominant pattern.

History of Disease

The most common cancer of the kidney in infants and children is named for Max Wilms, a German surgeon. There were excellent reviews of this lesion before Wilms, a second year surgical assistant, published “Die Mischgeschwulste Der Niere '' or The Mixed Tumors of the Kidney in 1899.

The survival rate for children with Wilms tumor was dismal until William Ladd, at the Boston Children’s hospital introduced rational surgical treatment. By mid century, Robert Gross achieved a 47% survival rate with surgery combined with postoperative radiation. Sydney Farber treated Wilms tumors with Actinomycin-d and opened the door to cancer chemotherapy. With protocols developed by the National Wilms Tumor Study Group, the survival rate of children with Wilms tumors reached 90% by the end of the twentieth century.

Dr. Willian Edwards Ladd (left) & Dr. Karl Maximilian Wilhelm ‘Max’ Wilms (right)

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WILMS TUMOR

Pathogenesis

Wilms tumor is a rare kidney cancer and commonly affected are children. Somatic or rare germline mutations cause this condition. Although this mutation follows an autosomal dominant inheritance, most of the studies are not known as inherited syndromes since the genetic basis for Wilms tumor and the mechanism caused by changes in genes is unclear. There are several genes involved that cause Wilms tumor to form. The mutation is caused by the alternation or loss of WT1 or WT2 genes that tumor suppressor genes found in chromosome 11, CTNNB1 gene on chromosome 3, as well as WTX gene and AMER1 gene on the X chromosome, resulting in overgrowth of specific body tissues. Another risk factor for developing Wilms tumor is a family history or the parent-specific differences in gene activation, known as genomic imprinting. The changes in the short (p) arm of chromosome 11 turn IGF2 and H19 genes on or off, depending on whether inherited the gene from the mother or the father.

Pathogenesis

Source

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Normal chromosome 11 vs Wilms' tumor patient

Symptoms

Abdominal pain, swelling or discomfort

Constipation

Nausea and vomiting

Loss of appetite

Fever

Discoloration or blood in the urine

High Blood pressure, which causes chest pain, shortness of breath, and headaches

Increased and uneven growth of one side of the body

Social Concerns

A treated patient can experience psychosocial and educational problems. It may be hard for the survivor to interact or befriend his/her classmate at school. Survivors are also prone to anxiety, depression, headstrong behavior and in attention.

Treatment

To know the best treatment for the patient that has a Wilms tumor they undergo a clinical trial conducted by his/her attending physician. There are also 3 main type of treatment which are:

Surgery for Wilms Tumors
Chemotherapy for Wilms Tumors
Radiation Therapy for Wilms tumor

There are also different approaches in treating the Wilms Tumor. In the United States, the surgery is conducted first and followed by chemotherapy or radiation therapy. On the other hand, in Europe patients undergo chemotherapy before having a surgery. This approach has the same results.

After the surgery, the doctors determine what stage of cancer the patient has. The doctors use the surgery to understand more of the case of the patient for further treatment. The Wilms tumor is treated depending on the stage of cancer.

You may also ask the doctor to have complementary and alternative methods to treat the Wilms Tumor or to ease the symptoms. You may take herbs, vitamins or a special diet or massage or acupuncture prescribed by the doctor. Contemporary treatment is treatment that goes along with your existing treatment while the alternative treatment is the treatment that is used instead of the standard treatment. Additionally, these treatments may help ease the symptoms and the pain, not all proven to work.

Radiation therapy

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Updates on Clinical Trials

Title: To Evaluate the Safety, Activity and Pharmacokinetics of Marqibo in Children and Adolescents With Refractory Cancer

Intervention:

Drug: Marqibo

Goal: Improvement of the drug’s ability to destroy cancer cells and help reduce the potential side effects of treatment.

Further details here.

Title: Combination Chemotherapy Alone or With Radiation Therapy in Treating Children With Kidney Cancer

Intervention:

Biological: dactinomycin
Biological: filgrastim
Drug: cyclophosphamide
Drug: doxorubicin hydrochloride
Drug: etoposide
Drug: vincristine sulfate
Procedure: conventional surgery
Radiation: radiation therapy

Goal: Drugs used in chemotherapy use different ways to stop tumor cells from diving so they stop growing or die. Radiation therapy uses high energy x-rays to damage tumor cells.

Further details here.

Title: Lorvotuzumab Mertansine in Treating Younger Patients With Relapsed or Refractory Wilms Tumor, Rhabdomyosarcoma, Neuroblastoma, Pleuropulmonary Blastoma, Malignant Peripheral Nerve Sheath Tumor, or Synovial Sarcoma

Intervention:

Other: Laboratory Biomarker Analysis
Biological: Lorvotuzumab Mertansine
Other: Pharmacological Study

Goal: The effective response of lorvotuzumab mertansine in the tumor. Antibody-drug conjugates, such aslorvotuzumab mertansine, are created by attaching an antibody (protein used by the body's immune system to fight foreign or diseased cells) to an anti-cancer drug.

Further details here.

Title: Combination Chemotherapy, Radiation Therapy, and/or. Surgery in Treating Patients With High-Risk Kidney Tumor

Intervention:

Drug: Doxorubicin Hydrochloride
Drug: Irinotecan Hydrochloride
Procedure: Conventional Surgery
Drug: Cyclophosphamide
Drug: Etoposide
Drug: Carboplatin
Biological: Dactinomycin
Drug: Vincristine Sulfate
Radiation: Radiation Therapy
Other: Laboratory Biomarker Analysis

Goal: Drugs used in chemotherapy work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. Giving more than one drug (combination chemotherapy) may kill more tumor cells. Radiation therapy uses high-energy x-rays to kill tumor cells. Giving combination chemotherapy together with radiation therapy before surgery may make the tumor smaller and reduce the amount of normal tissue that needs to be removed.

Further details here.

Expert Directory

Dr. Pablo O. Torre Memorial Hospital

Location:

B.S. Aquino Drive, Bacolod City 6100, Negros Occidental, Philippines

Contact No:

Tel. (034) 432 7624 local 8103
Mobile: 0939 109 7042 or 0995 394 2761

References

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