Malignant rhabdoid tumor is a rare childhood tumor that commonly
starts in the kidneys but also can occur in other soft tissues or in the brain,
where it is referred to as atypical teratoid rhabdoid tumor (ATRT).
Malignant rhabdoid tumor occurs most commonly in infants and
toddlers; the average age of diagnosis is 15 months old. There are about 20 to
25 new cases of malignant rhabdoid tumor diagnosed each year in the United
States. Cells from malignant rhabdoid tumors in children can spread
(metastasize) to other areas of the body.
Dana-Farber/Boston Children’s is a world-leading center in
the research and treatment of rhabdoid tumors. Our vibrant program of basic and translational
research into rhabdoid tumors is uncovering new opportunities to improve the
care of children with malignant rhabdoid tumor. Our multidisciplinary treatment approach ensures in-depth discussion of each child and personalized treatment plans for every patient.
Continue reading to learn more about malignant rhabdoid
tumor or visit the Kidney Tumor Program page to learn about our treatment program.
Diagnostic procedures for malignant rhabdoid tumor are used
to determine the exact type of tumor your child has and whether the tumor has
spread. Your child’s doctor will perform a complete medical and physical
addition, your child’s physician may order some of the following diagnostic
tests for malignant rhabdoid tumor:
If a biopsy is taken, the sample will be tested for
mutations in a gene called SMARCB1
(this gene also goes by the names INI1,
SNF5, and BAF47), which is turned off in nearly all rhabdoid tumors.
If a mutation is found, your child's doctor may recommend additional
tests to see if the mutation is what is called a germline mutation—the kind
that is present throughout the body and could potentially be passed within families.
Should those results be positive, your child's doctor may then recommend
testing you and other members of your family for the mutation, along with
The treatment of malignant rhabdoid tumor involves a
combination of therapies including surgery, radiation, and chemotherapy.
However, because this tumor is rare and aggressive, there is no defined
standard of care, and treatment options may be tailored to your child's
situation. Your child's doctor and other members of your care team will discuss
the options with you in depth. Prompt medical attention and aggressive therapy
are important for the best prognosis.
At Dana-Farber/Boston Children's, we treat children with malignant rhabdoid tumor through
the Kidney Tumor Program within our Solid Tumor Center.
Surgery for malignant
In most cases, the first step in treating malignant rhabdoid
tumor is surgery. Often a surgeon will try to remove the whole tumor at the
time of biopsy. Depending on the size and location of the tumor, the surgeon
may remove only part of the kidney or the whole kidney.
Chemotherapy for malignant
After surgery, children with malignant rhabdoid tumor are
treated with a very aggressive course of chemotherapy,
a group of drugs that interfere with the cancer cell’s ability to grow or
Does chemotherapy cause side effects?
While chemotherapy can be quite effective in treating certain
cancers, the drugs cannot tell the difference between normal, healthy cells and
cancer cells. Because of this, your child may experience adverse side effects
during treatment. Understanding these side effects can help you, your child, and
your care team prepare for, and, in some cases, prevent these symptoms from
Chemotherapy is systemic treatment, meaning it is introduced
to the bloodstream and travels throughout the body to kill cancer cells. Children
with malignant rhabdoid tumor receive chemotherapy:
Radiation therapy for malignant
Children with malignant rhabdoid tumor who are more than six
months old also may receive radiation therapy as part of their treatment.
transplantation for malignant rhabdoid tumor
The treatment of malignant rhabdoid tumor can be so intense
as to affect the body's ability to produce normal blood and immune cells. For
that reason, your child's doctor may recommend that your child undergo an
autologous stem cell transplant. This involves temporarily removing some of a child’s healthy
blood cells and giving her very high doses of chemotherapy and, in some cases,
radiation therapy to overwhelm and destroy the malignant rhabdoid tumor. The
child is then given back her own stored healthy blood cells to promote normal
body and organ function once treatment is done.
Supportive care is treatment to prevent and treat
infections, side effects of treatments, and complications. The goal is to keep
your child comfortable during treatment. It also is an important part of
preventing short- and long-term complications of the disease and treatment.
Your child’s prognosis (chance of recovery) and treatment
options depend on a number of different factors:
In general, malignant rhabdoid tumors are very aggressive
and difficult to treat. Patients often initially respond well to treatment, but
relapse either during or shortly after treatment remains a risk. Children with
germline mutations to SMARCB1 also
have a high risk of developing second tumors. Prompt medical attention and
appropriate therapy are important for the best prognosis.
Survivorship comes with numerous complex issues: the
long-term effects of treatment and the risk of second cancers, as well as
social and psychological concerns. Children treated for malignant rhabdoid
tumor should visit a survivorship clinic every year to:
A typical follow-up visit may include some or all of the
Since 1993, physicians, nurses, researchers, and
psychologists at the David B. Perini Jr. Quality of Life Clinic at Dana-Farber Cancer Institute have helped thousands of survivors
of pediatric cancers, treated at the institute and at other hospitals, to manage
these long-term consequences. Learn more about our cancer survivorship programs.
Researchers at Dana-Farber/Boston Children's are global leaders
in the basic and translational study of rhabdoid tumors, including malignant
rhabdoid tumor and atypical teratoid/rhabdoid tumor. Their goal: to understand
the biological basis of rhabdoid tumors, including the role(s) of mutations in
the gene SMARCB1, and to develop more
effective treatment methods for children with these tumors.
In addition, we have a phase I clinical trial open
for children with refractory or relapsed malignant rhabdoid tumor, as well as
access to any malignant rhabdoid tumor trials available through the Children's
Learn more about clinical trials
For many children with rare or hard-to-treat conditions,
clinical trials provide new options.
Phone: 617-632-5508Online form: Request an appointment