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, it 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. It is more difficult to treat patients with metastatic Ewing
Fortunately, treatments for Ewing sarcoma have improved dramatically in
recent years, and most children who develop this disease 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.
Continue reading to learn more about Ewing sarcoma, visit the Bone &
Soft Tissue Program to learn more about our treatment options, or meet our solid tumor treatment team.
The system used
for staging Ewing sarcoma is simpler than the one used for a lot of other
cancers. Ewing sarcoma is classified as either localized or metastatic.
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.
The symptoms of Ewing sarcoma can
be non-specific, and might mimic other, more common ailments. While symptoms
vary from child to child, the most common include:
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:
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 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):
Your child may need physical
therapy after surgery to help him restore strength and
function in the affected limb. If his leg was affected, he may need crutches
for some part of the two to three months of healing time. Bones in the area where the tumor was may
break easily, after only mild bumps or falls, because they have been weakened.
This is called a pathologic fracture.
we also offer complementary or alternative medicines as additions to (but not
replacements for) standard medical and surgical treatment. These therapies,
which may help control pain and side effects from surgery, chemotherapy and
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.
Clinical 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”).
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.
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
neurologists, neuro-psychologists or alternative/complementary therapy
also offer patient and family education, psychosocial assessment, genetic
counseling, reproductive counseling and opportunities to speak with other childhood cancer survivors.
Our orthopedic surgeons, clinicians, and families discuss rotationplasty, a surgical option for treating certain bone tumors. It allows a child to avoid full amputation of the leg.
Through this innovative treatment, some patients are able to spend more time at home during chemotherapy treatments.