Wilms tumor (also called nephroblastoma) is a cancerous tumor in the cells of the kidney.
Fortunately, with the right treatment, Wilms tumor is highly treatable.
Children with Wilms Tumor receive highly
coordinated multidisciplinary care through the Kidney Tumor Program at Dana-Farber/Boston Children’s.
As a regional, national and international referral center, our surgeons and solid tumor oncologists have extensive expertise in the
care of children with Wilms tumor, particularly those with bilateral tumors
(tumors on both sides of the abdomen), large tumors, or "unfavorable"
tumor subtypes rarely seen elsewhere. Our multidisciplinary team, which draws
on specialists from across Dana-Farber Cancer Institute and Boston Children's
Hospital, works together to develop thoughtful and precise treatment plans.
Wilms tumor specialists at Dana-Farber/Boston Children’s have pioneered important new surgical and medical approaches to the treatment of Wilms. They also play key roles in large cooperative studies through the National Wilms Tumor Study Group and Children's Oncology Group. These studies have rigorously compared treatment plans to determine the most effective and safest treatments and have helped to greatly advance Wilms tumor treatment over the last three decades.
Most cases of Wilms tumor occur with no family
history of the disease or other known cause. In a small number of cases, children are born
with a genetic predisposition to develop Wilms Tumor. These children have rare
genetic syndromes such as:
The most common symptom of Wilms tumor is the
sudden appearance of a solid mass in the abdomen. Though each child may
experience symptoms differently, other symptoms may include:
Keep in mind that the
symptoms of Wilms tumor may resemble other more common conditions or medical
problems. Always consult your child's physician for help with getting to a
The first step in treating your child is forming an
accurate and complete diagnosis. Your child’s physician may recommend a number
of different tests to determine the cause of the symptoms. In addition to a
complete medical history and physical examination, your child’s doctor may
order one or more of the following:
After we complete all
necessary tests, our experts meet to review and discuss what they have learned
about your child's condition. Then we will meet with you and your family to
discuss the results and outline the best treatment options.
An important part of diagnosing Wilms tumor
involves staging and classifying the disease, which will help your child’s
doctor determine treatment options and prognosis. Staging is the process of determining
whether the cancer has spread and, if so, how far.
Kidney tumors are typically categorized into five
stages. Wilms tumor is staged as follows according to guidelines from the
Children’s Oncology Group (COG), a national consortium of pediatric cancer
Your child’s doctor
can provide additional information on the classification of Wilms tumors.
Your child’s physician will determine a specific
course of treatment based on several factors, including:
The first step in most treatments is surgery to
remove the tumor. The most common operation is a radical nephrectomy, which removes the whole kidney,
the tissue around the kidney, the ureter (the tube that drains urine from the
kidney to the bladder) and the adrenal gland (which sits on top of the kidney).
This operation is performed if the tumor has occurred in only one kidney. Some
cases of low-stage Wilms tumor can be treated with surgery alone.
If your child has tumors in both kidneys or has a
known predisposition to kidney cancer, your child’s physician may recommend a
partial nephrectomy or kidney-sparing surgery which aims to remove the tumor
while leaving the maximum amount of healthy kidney tissue.
In rare cases—when the tumor is very large—your
child’s doctor may recommend chemotherapy to try to shrink the tumor before
surgery. In some cases, a biopsy may be the best first
surgical procedure for your child, to establish the diagnosis before treatment.
Surgical procedures for Wilms tumor – especially
bilateral tumors – should be performed in specialized centers where experienced
surgeons, working in the most technologically advanced settings, can provide
the most appropriate kidney surgery. Surgeons at Dana-Farber/Boston Children’s
have extensive, specialized experience in treating Wilms tumor.
Surgery is usually followed by chemotherapy and
sometimes radiation therapy.
The type of chemotherapy and need for radiation are determined by tumor stage (extent
of spread of the tumor) and tumor type. Radiation therapy may be indicated for
some tumors, including any that have spread to the lymph nodes in the abdomen
or the lungs.
In very rare cases, a stem cell transplant,
involving a transplant of your child’s own stem cells, may be used. This allows
your child’s physician to give high-intensity chemotherapy in difficult to
treat cases of Wilms tumor. Stem cell transplantation and the treatment needed
to manage its effects are complex and involve some risk. Your physician will
give you more detailed information on what to expect.
Treating other kidney tumors Malignant
rhabdoid tumor of the kidney was once considered a subset of Wilms tumor,
but is now classified as a separate tumor type in its own right. Other types of
kidney tumors include renal cell carcinoma, clear cell sarcoma of the kidney, cystic
nephroma and congenital mesoblastic nephroma.
Side effects in the treatment of Wilms tumor can
arise from surgery, radiation or chemotherapy.
Our goal is to keep your child as healthy and
strong as possible throughout treatment. Some side effects can be managed with
standard medical approaches. However, Dana-Farber/Boston Children’s also has
specialists who deliver complementary or alternative medicines. These
treatments, which may help control pain and side effects of therapy, include
We also have
nutritionists who can help manage nausea or other side effects, child life
specialists who offer play therapy, psychosocial oncologists and social workers
who give psychosocial support, and many other support specialists to help with
all aspects of cancer treatment.
Researchers at the Dana-Farber/Boston
Children’s Kidney Tumor Program conduct laboratory and
clinical research designed to improve understanding and treatment of pediatric
kidney tumors. We also offer clinical trials through the Children’s Oncology
Group (COG), and three of our specialists – Robert
Shamberger, MD; Elizabeth
Mullen, MD, FAAP; and Tom
Hamilton, MD; are on the COG renal tumor committee.
Here are some of our latest research studies:
For many children with rare or hard-to-treat
conditions, clinical trials provide new options.
As with any cancer, prognosis and long-term
survival can vary greatly from child to child depending primarily on the stage
of the disease and the tumor cells' appearance under a microscope. Prompt
medical attention, aggressive therapy and continued follow-up care are
important for the best possible prognosis.
With prompt, aggressive
treatment, most children with Wilms tumor are successfully treated and
experience relatively few side effects. More aggressive tumors that have spread
significantly may be harder to cure. Should a tumor come back after treatment,
your doctor may recommend additional surgery, chemotherapy and/or radiation
The goal of treatment
is both to cure the cancer and minimize the long-term effects of therapy. Children,
however, are at some risk of late-effects of the cancer and its treatment,
including high blood pressure, heart and
lung problems after chemotherapy or radiation therapy, and slowed or decreased
growth and development. Reduced kidney function may appear primarily in
children with tumors in both kidneys (bilateral involvement), or with a
predisposition syndrome that causes kidney abnormalities (nephritis) distinct from those
related to the tumor or treatment.
Factors that influence the outlook for a child with
Wilms tumor include: