Treatment FAQ

how to choose treatment regimen for wilms tumor

by Prof. Lula Berge Published 2 years ago Updated 2 years ago
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What is the best treatment for Wilms tumor?

Treatment for Wilms' tumor usually involves surgery and chemotherapy, and sometimes radiation therapy. Treatments may vary by the stage of the cancer....Surgery to remove all or part of a kidneyRemoving part of the affected kidney. ... Removing the affected kidney and surrounding tissue. ... Removing all or part of both kidneys.

What is the treatment for Stage 4 Wilms tumor?

Treatment for stage 4 Wilms tumour is usually chemotherapy for 6 weeks, then surgery to remove the kidney. After surgery, your child is likely to have more chemotherapy and may have radiotherapy. The exact treatment schedule depends on: your child's risk group.

Which drug is used in the treatment of Wilms tumor in children?

A combination of chemo drugs is used to treat children with Wilms tumors. The chemo drugs used most often are: Actinomycin D (dactinomycin) Vincristine.

What is an important intervention to take for a child with Nephroblastoma Wilms tumor?

Your child's care plan also includes treatment for symptoms and side effects, an important part of cancer care. The main treatments for a Wilms tumor are surgery, chemotherapy, and radiation therapy. Treatment options and recommendations depend on several factors, including: The type, stage, and histology of the tumor.

How long is chemotherapy for Wilms tumor?

Chemo is given for about 6 months. Anaplastic histology, with focal (only a little) anaplasia: Treatment starts with surgery if it can be done, followed by radiation therapy over several weeks. This is followed by chemo, usually with 3 drugs (actinomycin D, vincristine, and doxorubicin) for about 6 months.

Is Wilms tumor treatable?

Wilms tumor is a rare kidney cancer that is highly treatable. Most kids with Wilms tumor survive and go on to live normal, healthy lives. Also known as nephroblastoma, Wilms tumor can affect both kidneys, but usually develops in just one.

What cyclophosphamide is used for?

Cyclophosphamide is used to treat cancer of the ovaries, breast, blood and lymph system, and nerves (mainly in children). Cyclophosphamide is also used for retinoblastoma (a type of eye cancer mainly in children), multiple myeloma (cancer in the bone marrow), and mycosis fungoides (tumors on the skin).

What is SIOP in Wilms tumor?

SIOP (Societe Internationale D'oncologie Pediatrique) is another European Group that in 1971 started studies on Wilms' tumor. It differed from NWTS in the concept of giving preoperative chemotherapy to all patients.

What factor determines the initial treatment of a neuroblastoma?

For neuroblastoma, treatment depends on the patient's risk group. The stage of neuroblastoma is one factor used to determine risk group. Other factors are age, tumor histology, and tumor biology. There are three risk groups: low risk, intermediate risk, and high risk.

What is the nursing management of Wilms tumor?

The nursing care plan goals for a child with Wilms tumor include: prevention of injury, improved condition of oral mucous membranes, comprehension of the disease and its management, and absence of complications.

What is the serious potential complication of Wilms tumor?

Spread of the tumor to the lungs, lymph nodes, liver, bone, or brain is the most worrisome complication. High blood pressure and kidney damage may occur as the result of the tumor or its treatment. Removal of WT from both kidneys may affect kidney function.

Why would we perform a Wilms tumor excision?

The main goal of surgery is to remove the entire Wilms tumor in one piece, if possible. This is to keep the cancer cells from spreading in the abdomen (belly). Surgeons who operate on these tumors are careful to limit the chance of this type of cancer spread whenever possible.

What is the treatment for a Wilms tumor?

Most children with Wilms tumors will get more than one type of treatment. The main types of treatment are: Surgery for Wilms Tumors. Chemotherapy for Wilms Tumors. Radiation Therapy for Wilms Tumors.

What to do if your child has a Wilms tumor?

It’s important to weigh the benefits of each treatment option against the possible risks and side effects.

Why is it so hard to remove a kidney tumor?

Sometimes the tumor might be hard to remove because it's very large, it has spread into nearby blood vessels or other vital structures, or it’s in both kidneys. For children with these tumors, doctors might use chemotherapy, radiation therapy, or a combination of these to try to shrink the tumor (s) before surgery.

What are the services that help with cancer?

These might include nursing or social work services, financial aid, nutritional advice, rehab, or spiritual help. For children and teens with cancer and their families, other specialists can be an important part of care as well.

What to talk to your child's cancer care team about?

Be sure to talk to your child's cancer care team about any method you are thinking about using. They can help you learn what is known (or not known) about the method, which can help you make an informed decision. Complementary and Alternative Medicine.

How do I contact the American Cancer Society?

Call our National Cancer Information Center at 1-800-227-2345 and speak with one of our trained specialists. Finding Help and Support When Your Child Has Cancer.

What can I do to help my child with a tumor?

These methods can include vitamins, herbs, and special diets, or other methods such as acupuncture or massage, to name a few.

How to treat Wilms tumor?

Treatment for Wilms' tumor usually involves surgery and chemotherapy, and sometimes radiation therapy. Treatments may vary by the stage of the cancer. Because this type of cancer is rare, your child's doctor may recommend that you seek treatment at a children's cancer center that has experience treating this type of cancer.

How to diagnose Wilms tumor?

To diagnose Wilms' tumor, your child's doctor may recommend: A physical exam. The doctor will look for possible signs of Wilms' tumor. Blood and urine tests. These lab tests can't detect Wilms' tumor, but they can indicate how well the kidneys are working and uncover certain kidney problems or low blood counts. Imaging tests.

How does chemo help Wilms?

Chemotherapy uses powerful drugs to kill cancer cells throughout the body. Treatment for Wilms' tumor usually involves a combination of drugs, given through a vein, that work together to kill cancer cells.

What kind of scan is needed for Wilms tumor?

The doctor may recommend a chest X-ray or chest CT scan and bone scan to determine whether the cancer has spread beyond the kidneys.

What is the purpose of surgery for Wilms?

Surgery for Wilms' tumor may include: Removing part of the affected kidney.

What is the best treatment for cancer in children?

Radiation therapy. Depending on the stage of the tumor, radiation therapy may be recommended. Radiation therapy uses high-energy beams to kill cancer cells. During radiation therapy , your child is carefully positioned on a table and a large machine moves around your child, precisely aiming energy beams at the cancer.

Why is chemotherapy used after surgery?

Chemotherapy may be used before surgery to shrink tumors and make them easier to remove. It may be used after surgery to kill any cancer cells that may remain in the body . Chemotherapy may also be an option for children whose cancers are too advanced to be removed completely with surgery.

Stage I Wilms Tumor

Treatment of stage I Wilms tumor with favorable histology may include:

Stage II Wilms Tumor

Treatment of stage II Wilms tumor with favorable histology may include:

Stage III Wilms Tumor

Treatment of stage III Wilms tumor with favorable histology may include:

Stage IV Wilms Tumor

Treatment of stage IV Wilms tumor with favorable histology may include:

Stage V Wilms Tumor and patients at high risk of developing bilateral Wilms tumor

Treatment of stage V Wilms tumor (bilateral) may be different for each patient and may include:

What is the treatment for a wilm tumor?

Chemotherapy for Wilms Tumors. Chemotherapy (chemo) is the use of drugs to treat cancer. These drugs enter the blood and reach all areas of the body, which makes this treatment useful for cancer that has spread or might have spread beyond the kidney.

How often do you get chemo for Wilms?

Most often, the drugs are given once a week for at least several months. Chemo is usually given by a nurse in the doctor’s office or in the outpatient section of the hospital. Some children with Wilms tumors might need to stay in the hospital while they are getting chemo, but usually this is not needed.

What is chemo used for?

A combination of chemo drugs is used to treat children with Wilms tumors. The chemo drugs used most often are: For tumors at more advanced stages, those with anaplastic histology, or tumors that recur (come back) after treatment, other drugs might also be used, such as:

Do children with Wilms tumors need chemo?

Most children with Wilms tumors will get chemo at some point during their treatment. (Some children with very low risk tumors might not need it.) In the United States, chemo is usually given after surgery. Sometimes it may be needed before surgery to shrink a tumor to make the operation possible.

Does chemo help with Wilms?

But this small increase in risk has to be weighed against the importance of chemo in treating Wilms tumor. Some drugs might also affect fertility (the ability to have children) years later. See Living as a Wilms Tumor Survivor for more on the possible long-term effects of treatment.

How many Wilms tumors are there?

Approximately 5% to 10% of individuals with Wilms tumor have bilateral or multicentric tumors. The prevalence of bilateral involvement is higher in individuals with genetic predisposition syndromes than in those without predisposition syndromes. For example, in 545 cases of bilateral Wilms tumors, bona fide pathogenic germline variants were found in 22% of patients. [ 124] The most common predisposition variants are mutations of WT1 and 11p15 loss of imprinting. [ 22, 72]

Where does Wilms tumor occur?

Wilms tumor may arise during embryogenesis on the background of an otherwise genomically normal kidney, or it may arise from nongermline somatic genetic precursor lesions residing in histologically and functionally normal kidney tissue. Hypermethylation of H19, a known component of a subset of Wilms tumors, is a very common genetic abnormality found in these normal-appearing areas of precursor lesions. [ 62]

How long does radiation therapy take for a Wilms tumor?

In a review of 1,488 patients with Wilms tumor who underwent surgery and radiation therapy, a surgery-to-radiation therapy interval of greater than 14 days was associated with an increased risk of mortality (hazard ratio, 2.13; P = .013). This underscores the importance of initiating radiation therapy within 14 days of surgery, which is specified in Wilms tumor treatment protocols. [ 225 ] [ Level of evidence: 3iiiA]

What percentage of Wilms tumors are anaplastic?

Tumors occurring in older patients (aged 10–16 years) have a higher incidence of anaplastic histology. [ 166] In bilateral tumors, 12% to 14% have been reported to have anaplastic histology in one kidney. [ 167, 168]

Why is radiation therapy used?

Radiation therapy is used to improve local control and treat sites of metastatic disease. Radiation therapy has historically been dependent on stage and histology, but more recently is also guided by the tumor molecular signature. [ 228]

Can a renal mass be biopsy?

Biopsy of a renal mass may be indicated if the mass is atypical by radiographic appearance for Wilms tumor, and the patient is not going to undergo immediate nephrectomy. If a primary nephrectomy cannot be performed, a biopsy, either open or with multiple cores, is required.

Can a Wilms tumor be found in a child?

Most Wilms tumor patients present asy mptomatically with an abdominal mass noticed by a parent or pediatrician on a well-child visit. In children with known predisposing clinical syndromes, renal tumors can be found during routine screening. Clinical findings may include the following:

Management of lung metastasis

Patients with negative findings on chest radiography and positive findings on CT scanning of the lungs require tissue diagnosis of the lung nodules because several conditions (eg, histoplasmosis, atelectasis, pseudotumor, intrapulmonary lymph node, pneumonia) can mimic pulmonary metastases.

Management of bilateral Wilms tumor

One report of patients with bilateral Wilms tumor treated according to the National Wilms Tumor Study-4 demonstrated that preservation of renal parenchyma is possible following initial preoperative chemotherapy.

Additional treatment considerations

Whether patients younger than age 6 months who have stage III disease should receive radiation therapy is controversial. In the NWTS-5 protocol, physicians were required to call an NWTS-5 radiation oncologist to discuss guidelines.

Activity

No precautions regarding activity are advised, although the patient and his or her parents should be aware that the patient will have only 1 kidney after therapy. Activities that carry an inherent risk of kidney injury, such as boxing and hockey, should be avoided.

Consultations

The patient should be referred to a pediatric surgeon, a pediatric oncologist, and, in some cases, a radiation oncologist.

What is the best treatment for Wilms tumors?

Surgery is the main treatment for nearly all children with Wilms tumors. It's importantthat it is done by a surgeon who specializes in operating on children and has experiencein treating these cancers.

What is the goal of a Wilms tumor removal surgery?

The main goal of surgery is to remove the entire Wilms tumor in one piece , if possible.This isto keep the cancer cells from spreading in the abdomen (belly). Surgeons whooperate on these tumors are careful to limit the chance of this type of cancer spreadwhenever possible. If the surgeon finds (either with imaging tests1 done before surgery,or when starting the operation) that the entire tumor can’t be removed safely, othertreatments may be used first. If these treatments shrink the tumor enough, surgery canthen be done more safely.

What to do before chemo for kids?

Before each chemo session, your child’s doctor will get blood tests4 to check blood celllevels and to see how well the liver and kidneys are working. If there are problems,chemo might need to be delayed or the doses reduced.

What is the treatment for cancer?

Chemotherapy (chemo) is the use of drugs to treat cancer. These drugs enter the bloodand reach all areas of the body, which makes this treatment useful for cancer that hasspread or might have spread beyond the kidney.

What is the most common surgery for a Wilms tumor that is only in one kidney?

radical nephrectomy removes the entire kidney and some nearby structures. This isthe most common surgery for a Wilms tumor that’s only in one kidney, as it provides thebest chance of making sure all of the tumor is removed.

What to do if your child has a Wilms tumor?

If your child has been diagnosed with a Wilms tumor, your child's treatment team willdiscuss the options with you. It’s important to weigh the benefits of each treatmentoption against the possible risks and side effects.

Can surgery remove a tumor?

Surgery cannot remove these tumors completely because of their size or location or forother reasons. In some cases, surgery may be postponed until other treatments areable to shrink the tumor first (see below).

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