Ewing sarcoma is a kind of cancer that grows in bones or soft
tissues. The tumor was first described by a pathologist, Dr. James Ewing, in
the 1920’s. Most often, Ewing sarcoma is found in the bones of the pelvis or thigh, though
it can arise throughout the body.
Most people with Ewing sarcoma have localized disease,
meaning tests do not find signs of the tumor outside of the main tumor. About a
quarter of people with Ewing sarcoma have metastatic disease, meaning tests show
that the tumor has spread to parts of the body outside of the main tumor. When
Ewing sarcoma spreads, it usually spreads to the lungs, other bones, or the
bone marrow. Metastatic Ewing sarcoma is more difficult to treat.
Fortunately, Ewing sarcoma treatments have improved dramatically in
recent years, and most children who develop Ewing sarcoma have a good chance for
recovery with proper treatment.
Children diagnosed with Ewing sarcoma are treated
at Dana-Farber/Boston Children's through our Bone & Soft Tissue Tumor Program.
Our integrated pediatric oncology
service offers – in one specialized program – the combined expertise of a leading
cancer center and a world-renown children’s hospital.
The system used
for staging Ewing sarcoma is simpler than the one used for most other
cancers. Ewing sarcoma is classified as either localized or metastatic.
- Localized means
the tumor has not spread beyond the initial location where it began, or beyond
the closest surrounding tissues.
- Metastatic means
the tumor has spread to the lungs, bones, bone marrow, or to other organs or structures in
the child’s body.
The classification (or “stage”), which is
determined based on the results of imaging tests and biopsies, helps your
doctors decide on treatment options and prognosis.
Ewing sarcoma occurs because a
certain type of stem cell starts to grow abnormally, and these cells then form
a tumor. We understand now that a specific chromosomal change (called a
“translocation”) in a cell's DNA – the “building blocks” that make up all
living organisms – is one of the first events that turns a normal cell into a Ewing
sarcoma cell. This translocation is not inherited, but instead develops in cells
after the child is born. It is not known why some people are more likely to
have cells that develop this translocation and therefore go on to develop Ewing
sarcoma. It is important to understand that there’s nothing you could have done
or avoided doing that would have prevented your child’s cancer from developing.
Ewing sarcoma symptoms can
be non-specific, and might mimic other, more common ailments. While symptoms
vary from child to child, the most common include:
- sporadic bone pain of varying intensity
- localized pain around the site of the tumor
- limp or limited range of motion in the affected
area
- swelling and/or redness around the site of the tumor
- fever of unknown cause
- decreased appetite
- weight loss
- fatigue
- symptoms related to nerve compression caused by
the tumor (such as numbness, tingling, or weakness)
- loss of bowel and bladder function (if the tumor
is in the spinal region)
Because many of these symptoms
can also point to other conditions, it’s important to have your child evaluated
by a qualified medical professional right away.
The first step in treating your child is forming an accurate
and complete diagnosis. Ewing sarcoma is typically diagnosed using a
combination of imaging and biopsy. Your child’s physician may order a number of
different tests including:
- A physical exam and complete medical history.
- Magnetic resonance imaging (MRI), a diagnostic
procedure that produces detailed images. An MRI uses a combination of large
magnets, radiofrequencies and a computer to analyze organs and structures
within the body.
- CT/CAT scan, an imaging technique that provides
more detailed pictures than x-rays.
- Positron emission tomography
(PET) scan, a test commonly used to
detect cancers and help doctors see how the organs
and tissues inside the body are functioning.
- Bone scan,
used to detect bone diseases and tumors, as well as to determine
the cause of bone pain or inflammation.
- Bone marrow aspiration and biopsy, a
procedure that involves taking
a small amount of bone marrow fluid and tissue (usually from part of both hip
bones) to look for signs of Ewing sarcoma cells.
- Tumor biopsy, a procedure that can involve a
surgeon removing a small piece of the tumor or a specialized radiologist using
a needle to obtain a part of the tumor. Doctors then look at the tumor cells to establish a diagnosis. This might include
testing to look for the translocation
seen in Ewing sarcoma (see "Causes and Symptoms of Ewing sarcoma" section above).
There may be other diagnostic tests that your doctor will discuss with
you depending on your child's individual situation. 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 possible treatment options.
Ewing sarcoma treatments have improved dramatically in recent years, particularly for
patients with localized disease.
Most of the time, there are two components in
treating children with newly diagnosed Ewing sarcoma: local
control, which involves treating
the tumor itself, usually through surgery, radiation or both; and systemic
therapy, which treats any tumor cells throughout the body,
usually through chemotherapy. Ewing sarcoma can usually only be cured by using
local control together with systemic therapy. Your child's treatment may
include (alone or in combination):
- Chemotherapy: Treatment for Ewing sarcoma almost always begins
with chemotherapy, which aims to destroy or shrink cancer cells and
prevent them from spreading. Most
children with Ewing sarcoma respond very well to chemotherapy.
Chemotherapy is usually given over a period of a few days every two weeks for
about 12 weeks before it is time for local control of the main tumor. After surgery and/or radiation for local
control, chemotherapy is continued for another 4-6 months in order to eliminate
all the cancerous cells in the body. We give chemotherapy cycles every two weeks in Ewing sarcoma because studies
show that this scheduled (called “interval compression”) improves outcomes for
children with localized Ewing sarcoma.
The typical chemotherapy regimen for a child with newly
diagnosed Ewing sarcoma involves medicines given intravenously (or “IV”), as a
direct injection into the blood stream. This chemotherapy used to be given
during overnight stays in the hospital. Now through our home hydration program,
most children are able to receive this regimen as an outpatient.
While chemotherapy can be quite effective in treating
certain cancers, the drugs cannot differentiate normal healthy cells from
cancer cells. As a result, there can be adverse side effects during treatment.
Being able to anticipate these side effects can help the care team, child and
family prepare (and, in some cases, prevent) these complications from
occurring, if at all possible.
- Surgery:
Local control of Ewing sarcoma is
achieved with surgery and/or radiation therapy. Surgery may be performed
after the first 12 weeks of chemotherapy to remove any parts of the tumor that
remain. Several
forms of surgical intervention may be considered for your child depending on the size and location of the
tumor.
- Limb-salvage surgery helps preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor. The goal of limb-salvage surgery is to preserve limb function, as well as the pre-surgical appearance of the limb. However, limb-salvage surgery can leave an arm or leg fragile and increase the risk of fracture. As a result, patients will need to avoid high-stress physical activities, such as skiing, skate-boarding or bike riding.
- Amputation may be necessary if the tumor cannot be completely removed (for example, if it involves the nerves and blood vessels) or if limb function cannot be preserved through limb-salvage surgery. If amputation is necessary, your child may be fitted for a prosthesis following surgery.
- Rotationplasty is a partial amputation that preserves a cancer-free lower leg, attaches it to the thighbone and uses the ankle as a knee joint – especially useful in very young children where limb length can be an issue. One of the major benefits of rotationplasty compared to other surgical options is that it allows the child to maintain a very active lifestyle, including high-impact sports.
Surgery at Dana-Farber/Boston Children’s is personalized to help your child achieve the best functional and cosmetic outcome and to allow them to remain active and healthy. Depending on the type of surgery your child receives, your child may have to avoid certain physical activities following treatment.
Limb-salvage surgery and rotationplasty are complex procedures that require specialized expertise. Our bone tumor surgeons are among the few orthopedic surgeons in the nation trained in
pediatric tumor surgery and who have experience in these procedures. At Dana-Farber/Boston Children's, we also
have physical therapists and prostheticians who specialize in helping children who undergo these procedures.
- Radiation therapy: Children with Ewing sarcoma occurring in a
location where surgical removal would lead to unacceptable side effects will
receive radiation therapy instead
of surgery. Some children need both surgery and radiation therapy. Radiation is
used to either shrink the tumor or to destroy it completely. Ewing sarcoma is
very sensitive to radiation therapy.
- Proton beam radiation therapy: Depending on the location and size of the
tumor, our doctors may suggest proton beam radiation therapy, which targets a
more specific area than standard radiation and spares more of the surrounding
healthy tissue and organs.
- acupuncture/acupressure
- therapeutic touch
- massage
- herbs
- special dietary recommendations
Dana-Farber/Boston Children’s is one of the
top pediatric research centers in the world, with top researchers in Ewing
sarcoma. Our research program includes laboratory scientists and clinical
researchers from both Dana-Farber Cancer Institute and Boston Children’s
Hospital. We investigate Ewing sarcoma from every angle—from examining cells
under the microscope to tracking response to current drug regimens—so that we
can create better treatments for your child.
Our laboratory researchers have helped to define the gene
changes in Ewing sarcoma cells beyond the known translocation seen in these
cells. We are also developing new tools to detect low levels of Ewing sarcoma
cells in the blood and bone marrow. These tools may help us to improve how we
monitor response to treatment.
Ewing sarcoma clinical trialsClinical trials, or research studies
evaluating new treatment approaches, are a major offering at Dana-Farber/Boston
Children’s. We were one of the first centers to perform limb-salvage surgery for patients with bone cancers like Ewing sarcoma. Today, our clinical researchers continue to develop new approaches to the treatment of Ewing sarcoma.
We are now conducting numerous clinical trials, mainly focusing on patients with the hardest-to-treat forms of Ewing sarcoma: metastatic Ewing sarcoma and Ewing sarcoma that has come back after initial treatment (called “relapsed” or “recurrent” disease). For example, we are leading a clinical trial for children and young adults with newly diagnosed metastatic Ewing sarcoma that compares standard treatment to standard treatment with the addition a drug that blocks an important growth pathway in Ewing sarcoma (called the “IGF-1R pathway”).
Learn more
We offer
enrollment in both independent and cooperative treatment studies,
many of which are available only at our center.
For patients with localized
Ewing tumors, the five-year survival rate is close to 70 to 80 percent. For people
with metastatic disease, the five-year survival rate is 20 to 30 percent.
Patients
who have Ewing sarcoma that has
come back after initial treatment (called “relapsed” or “recurrent” disease)
have a poor prognosis. Children whose disease recurs more than two years after initial diagnosis
have a five-year survival rate of 30 percent, while very few patients whose
disease recurs less than two years from initial diagnosis will survive with our
current best therapies.
However, it’s important to understand that your
child’s short-term health and long-term health can vary greatly depending on
his specific circumstances. Prompt medical attention and intensive therapy are
very important, as is continuous follow-up care.
Because Ewing sarcoma does have a tendency to
recur later in life – even as many as 10 years after treatment – long-term
follow-up care is essential. We see patients with Ewing sarcoma for x-rays or
MRI of the primary tumor site and CT scan of the chest every three months after
treatment is completed. If all goes well, the frequency of the visits will decrease
for the next five years.
Some children who are treated for Ewing
sarcoma and other pediatric cancers can experience significant long-term
problems as a result of their treatment. All kids who have been treated for
cancer require ongoing, specialized cancer survivorship care.
Through the David B. Perini, Jr. Quality of Life Clinic, our cancer
survivorship clinic, childhood cancer survivors receive a comprehensive
follow-up evaluation from their cancer care team. In addition to meeting
with your pediatric oncologists, your child may see one of our
endocrinologists, cardiologists,
neurologists, neuro-psychologists or alternative/complementary therapy
specialists. We
also offer patient and family education, psychosocial assessment, genetic
counseling, reproductive counseling and opportunities to speak with other childhood cancer survivors.