A fibrosarcoma is a malignant (cancerous) tumor that originates in the
connective fibrous tissue found at the ends of bones of the arm or legs, and
then spreads to other surrounding soft tissues. It is a type of soft tissue tumor; soft tissues include fat, muscles, tendons (bands of fiber that connect
bones to muscle), nerves, joint tissue, blood vessels and other fibrous tissue.
Fibrosarcoma most commonly affects either a lower leg or arm.
Children with fibrosarcoma are treated
at Dana-Farber/Boston Children's through our Bone and Soft Tissue Program. Our integrated pediatric oncology program offers the
combined expertise of a leading cancer center and a world-renown children’s
hospital.
Fibrosarcoma causes
The exact cause of fibrosarcoma and other soft tissue tumors is not
entirely understood. However, studies have indicated that genetic alterations
may play a role. Researchers have studied a small number of families that
contain several members from 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. A chromosomal
rearrangement has been found in some fibrosarcomas.
Fibrosarcoma symptoms
Each child may experience fibrosarcoma symptoms differently, depending
on the size, location and spread of the tumor.
Fibrosarcoma symptoms may include:
- A painless or tender mass in an extremity or trunk
- Pain or soreness caused by suppressed nerves and
muscles
- Limping or other difficulty using legs, feet, arms
hands
Fibrosarcoma symptoms
may resemble other conditions or medical problems. Always consult a physician
for a diagnosis.
In addition to a complete medical history and physical examination of
your child, diagnostic procedures for fibrosarcoma may include:
- Multiple imaging studies of the tumor and sites of
possible metastasis, such as:
- x-rays – a diagnostic test which uses invisible electromagnetic energy beams to
produce images of internal tissues, bones, and organs onto filmX-rays are very
useful in the diagnosis of bone tumors and frequently x-rays allow the
physician to distinguish between benign and malignant tumors. An x-ray is
usually the first diagnostic study and it often gives the doctor information
regarding the need for further testing.
- magnetic
resonance imaging (MRI) – a diagnostic procedure that uses a combination of
large magnets, radiofrequencies, and a computer to produce detailed images of
organs and structures within the body. This test is used to assess the size and
extent of the mass and its relationship to surrounding muscle, bone, nerves and
blood vessels.
- computerized
tomography scan (also called a CT or CAT scan) – a diagnostic imaging procedure
that uses a combination of x-rays and computer technology to produce
cross-sectional images (often called slices), both horizontally and vertically,
of the body. A CT scan shows detailed images of any part of the body, including
the bones, muscles, fat, and organs. CT scans are more detailed than general
x-rays. They are used primarily to assess the chest and lung for metastatic
tumors.
- bone
scans – a nuclear imaging method used to detect bone and metastatic tumors. It
can determine if there are abnormalities in other bones. This test does not
distinguish between tumor,infection or fractures.
- Complete blood count (CBC) – a measurement of size, number, and
maturity of different blood cells in a specific volume of blood
- Blood tests (including blood chemistries)
- Biopsy of the tumor – a small tissue sample of the
tumor is surgically removed. The appearance of the tumor under a microscope
establishes the diagnosis and distinguishes a fibrosarcoma from other sarcomas.
Biopsy also helps doctors determine how aggressive the tumor is.
How is fibrosarcoma staged?
Once fibrosarcoma 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.
A fibrosarcoma may be localized, meaning it has not spread beyond the bone
where it arose or beyond nearby tissues, or 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. Tumors found during infancy are usually not
metastatic.
Specific fibrosarcoma treatment options will be determined by your
child's physician based on:
- your child's age, overall health, and medical
history
- extent of the disease
- your child's tolerance for specific medications,
procedures, or therapies
- how your child's physician expects the disease may
progress
- your opinion or preference
Fibrosarcoma treatment for children may include:
- Surgery: Surgery is often a first step, allowing doctors to
form a complete diagnosis of the tumor type and providing information on
the stage of the disease. If your child’s leg or arm is affected, your
child may receive:
- limb-salvage surgery, to help preserve the limb by removing
the tumor and wide margins of healthy tissue surrounding the tumor
- rotationplasty, a partial amputation that preserves a
cancer-free lower leg, attaches it to the thighbone and uses the ankle as
a knee joint
- amputation surgery, which in rare cases may be necessary (for
example, if it involves the nerves and blood vessels)
- Head or neck surgery, if the fibrosarcoma is in the head or neck; this involves surgical removal of the tumor and bone/skin grafts.
The type of surgery will depend on the size and location of the tumor, and whether the cancer has spread. At Dana-Farber/Boston Children's, these procedures are performed in the hospital's new state-of-the-art pediatric MR-OR. It allows specialists to take an MRI scan immediately following surgery to ensure the tumor is completely removed and decreases the need for additional surgery.
- Radiation therapy: This treatment can help stop the growth of
abnormal cells in specific areas of the body. Radiation therapy uses
high-energy rays from a specialized machine to damage or kill abnormal
cells. Our doctors use precisely targeted and dosed radiation to kill
cancer cells left behind after your child's surgery.
- Chemotherapy: These medicines can help stop the growth of
abnormal cells throughout the body. Chemotherapy is systemic treatment,
meaning it is introduced to the bloodstream and travels throughout the
body to kill or slow the growth of targeted cells. Different groups of
chemotherapy drugs work in different ways, and can be taken in a variety
of ways including orally or intravenously.
- Rehabilitation: Rehabilitation includes physical and occupational therapy and
psychosocial adapting.
- Supportive care: Supportive care refers to any type of treatment to prevent or treat
infections, side effects of treatments, and complications, and to keep your
child comfortable during treatment.
Prognosis for fibrosarcoma greatly depends on:
- the extent of the disease
- the size and location of the tumor
- presence or absence of metastasis
- the tumor's response to therapy
- the age and overall health of the child
- your child's tolerance of specific medications,
procedures, or therapies
- new developments in treatment