sarcoma is a malignant tumor of the soft tissues, usually around joints. Under
the microscope the tumor resembles synovial tissue (the lining tissue of
joints). Synovial tissue is found around the tendons (bands of fiber that connect
muscle to bone), and can form bursa (fluid filled cushioning pouches or sacs
found in spaces between tendons, ligaments and bones) found in the area of
Synovial sarcoma is a rare tumor. It is one of many types of cancer classified as a soft tissue sarcoma, cancer that originates in soft tissue, which includes fat,
muscles, tendons, nerves, synovial tissue, blood vessels and other fibrous
tissue. As a group, soft tissue sarcomas account for less than 1 percent of all
new cancer cases each year. In the United States, approximately 900 children
and adolescents are diagnosed with soft tissue sarcomas each year.
Children diagnosed with synovial sarcoma are treated
at Dana-Farber/Boston Children's through our Bone
and Soft Tissue Tumors 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.
exact cause of synovial sarcoma is not entirely understood; however, studies have
indicated that genetic alterations may play a role in the formation of soft
tissue sarcomas. Researchers have studied a small number of families that
contain several members of one generation who have developed soft tissue
sarcomas. In addition, limited studies have shown a possible link between soft
tissue sarcomas and the development of other types of cancer.
synovial sarcoma, a rearrangement in the chromosome material between
chromosomes X and #18 is usually present. This rearrangement changes the
position and function of genes, causing a fusion of genes referred to as a
"fusion transcript." Patients have an abnormal fusion transcript
involving two genes which creates a novel (new) gene. This important discovery
has led to improvements in diagnosing soft tissue sarcomas, and may lead to newer
treatments in the future.
Certain inherited diseases are also associated with an increased risk of
developing soft tissue sarcomas. These include people with Li-Fraumeni syndrome
(which involves alterations in the p53 gene) or neurofibromatosis (which
involves alterations in the NF1 gene). For some soft tissue tumors, there seems
to an association with an Epstein-Barr virus infection.
following are the most common symptoms of synovial sarcoma. However, each child
may experience symptoms differently. Symptoms can depend on the size and
location of the tumor. Sometimes the symptoms of synovial sarcoma can resemble
those of arthritis, bursitis or synovitis. Symptoms may include:
symptoms of synovial sarcoma may resemble other conditions. Always consult your
child's physician for a diagnosis.
addition to a complete medical history and physical examination, the most
conclusive diagnostic procedure for synovial sarcomas is a biopsy, a single
tissue sample taken from the tumor through a simple surgical procedure. The
tumor's cellular appearance under a microscope enables doctors to distinguish
it from other types of cancer. Your child likely also will undergo various imaging studies that will include one or
more of the following:
synovial sarcoma has been diagnosed, the tumor is staged. This process
indicates how far the tumor has spread from its original location. The stage of
a tumor suggests which form of treatment is most appropriate, and gives some
indication of prognosis for the condition.
A synovial sarcoma may be localized, meaning it has not spread beyond the joint
where it arose or beyond nearby tissues; or it can be metastatic, meaning it has spread
to lungs, bones other than the bone that the tumor originated in, or to other
organs or structures of the body.
treatment for synovial sarcoma will be determined by your child's
treatment team based on:
includes biopsy and surgical removal of the entire tumors, nearby muscle and
lymph nodes. Depending on the location and size of the tumor, it may be
necessary to remove all or part of the limb. In most cases limb-sparing surgery
is used to avoid amputation. The following is a description of both procedures:
Limb-salvage surgery –
Limb-sparing surgery is indicated only if your child's orthopedic surgeon
determines that it is possible that the tumor, and wide margins of healthy
tissue surrounding the tumor, can be removed. Through limb-sparing surgery, all
of the tissue involved with the tumor, including some degree of muscle
surrounding it and nearby lymph nodes, are removed; unaffected tendons, nerves
and vessels are saved.
If bone is also removed, it is usually replaced with a bone graft or with a
metal rod. Subsequent surgery may be needed to repair or replace rods, which
can become loose or break.
Patients who have undergone limb-sparing surgery need intensive rehabilitation.
It may take as long as a year for a patient to regain full use of a leg
following limb-salvage surgery.
Some patients who have had limb sparing procedures may eventually have to
undergo amputation. Radiation therapy and/or chemotherapy are given either
before surgery to shrink the tumor, or after surgery to kill remaining cancer
Rotationplasty – Rotationplasty can be used to treat malignant bone or soft tissue tumors that occur near a child’s knee (in the lower part of the femur or upper tibia bone). This procedure preserves the lower leg, attaches it to the thighbone, then uses the ankle as a knee joint.
Amputation – In certain cases, if
your child's orthopedic surgeon determines that the tumor cannot be removed
because, for example, it involves the nerves and blood vessels, amputation is
the only option.
During the operation, doctors ensure that muscles and skin form a cuff around
the amputated bone. A cast is applied in the operating room which permits a
temporary artificial leg (prosthesis) to be applied during the first few
post-operative days for walking. Crutches are used for several weeks. As the
swelling decreases (10 to 14 days) the patient is fitted for a plastic,
temporary socket and prosthesis, which is used for 3 to 4 months until the
stump is healed sufficiently to accept a permanent artificial leg.
The advantages of an amputation are that it is a simple operation with minimal
chances of surgical complication and it definitively removes the local tumor.
The functional outcome is good with the modern prostheses available today and
with "immediate-fit" prostheses applied in the operating room.
Although the patient will probably have a limp with above-the-knee amputations,
the procedure is functional and stable. He/she will be able to walk, climb
stairs, swim (with the prosthesis on or off) and participate in many sports
such as skiing, basketball, baseball, and tennis although running will be
limited. The functional limitations are left to the imagination and
determination of the patient.
therapy is a treatment that uses high energy rays from a specialized
machine to damage or kill cancer cells and shrink tumors. This is sometimes
used in conjunction with surgery for synovial sarcoma, either before or after
resection of the tumor. On rare occasions radiation alone is used for treatment
of the primary tumor.
is a drug treatment that works by interfering with the cancer cell's ability to
grow or reproduce. Different groups of drugs work in different ways to fight
cancer cells and shrink tumors. Chemotherapy may be used alone for some types
of cancer or in conjunction with other therapy such as radiation or surgery.
Often, a combination of chemotherapy drugs is used to fight a specific cancer.
Certain chemotherapy drugs may be given in a specific order depending on the
type of cancer it is being used to treat.
While chemotherapy can be quite effective in treating certain cancers, the
agents do not differentiate normal healthy cells from cancer cells. Because of
this, there can be many adverse side effects during treatment. Being able to
anticipate these side effects can help the care team, parents, and child
prepare and, in some cases, prevent these symptoms from occurring, if possible.
Chemotherapy is a systemic treatment, meaning it is introduced into the
bloodstream and travels throughout the body to kill cancer cells.
Chemotherapy may be given:
care refers to any type of treatment to prevent and treat infections, side
effects of treatments and complications and to keep your child comfortable
at Dana-Farber/Boston Children's are conducting numerous research studies
that will help clinicians better understand and treat soft tissue sarcomas.
Other types of treatment currently being studied include:
greatly depends on:
with any cancer, prognosis and long-term survival can vary greatly from
individual to individual. Prompt medical attention and aggressive therapy are
important for the best prognosis.
Cancer survivorship care
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.
Lindsay Frazier, MD, explains how a multidisciplinary team of specialists come together to deliver care for solid tumors.