Recurrent Osteosarcoma and Malignant Fibrous Histiocytoma of Bone and Treatment
Recurrent osteosarcoma and malignant fibrous histiocytoma (MFH) of bone are
cancers that have recurred (come back) after being treated. The cancer may come back in the
bone or in other parts of the body. Osteosarcoma and MFH most often recur in the
lung, bone, or both. When osteosarcoma recurs, it is usually within 18
months after treatment is completed.
There are different types of treatment for patients with osteosarcoma or
malignant fibrous histiocytoma (MFH) of bone.
Different types of treatment are available for children with
malignant fibrous histiocytoma (MFH) of bone. Some treatments are
standard (the currently used treatment), and some are being tested in
clinical trials. A treatment clinical trial is a
research study meant to help improve current treatments or obtain
information on new treatments for patients with
cancer. When clinical trials show that a new treatment is better than the
standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be
considered. Some clinical trials are open only to patients who have not started
Children with osteosarcoma or MFH should have their treatment planned by a
team of health care providers who are experts in treating cancer in children.
Treatment will be overseen by a
pediatric oncologist, a doctor who specializes in treating children with
cancer. The pediatric oncologist works with other
health care providers who are experts in treating osteosarcoma and MFH and
who specialize in certain areas of
medicine. These may include the following
- Orthopedic surgeon.
- Radiation oncologist.
- Rehabilitation specialist.
- Pediatric nurse specialist.
- Social worker.
Treatment for osteosarcoma or malignant fibrous histiocytoma may cause side
Side effects from cancer treatment that begin after treatment and continue for
months or years are called late
effects. Late effects of cancer treatmeeffects. Late effects of cancer treatment may include the following:
- Physical problems.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers (new types of cancer).
Some late effects may be treated or controlled. It is important to talk with
your child's doctors about the effects cancer treatment can have on your child.
Four types of standard treatment are used:
Surgery to remove the entire
tumor will be done when possible.
Chemotherapy may be given before surgery to make the tumor smaller. This is
neoadjuvant chemotherapy. Chemotherapy is given so less bone
tissue needs to be removed and there are fewer problems after surgery.
The following types of surgery may be doThe following types of surgery may be done:
Wide local excision: Surgery to remove the cancer and some healthy
tissue around it.
Limb-sparing surgery: Removal of the tumor in a limb (arm or leg)
amputation, so the use and appearance of the limb is saved. Most
patients with osteosarcoma in a limb can be treated with limb-sparing
surgery. The tumor is removed by wide local
excision. Tissue and bone that are removed may be replaced with a
graft using tissue and bone taken from another part of the patient's
body, or with an
implant such as artificial bone. If a fracture is found at
diagnosis oor during chemotherapy before surgery, limb-sparing surgery
may still be possible in some cases. If the surgeon is not able to remove
all of the tumor and enough healthy tissue around it, an amputation may be
- Amputation: Surgery to remove part or all of an arm or leg. This may be
done when it is not possible to remove all of the tumor in limb-sparing
surgery. The patient may be fitted with a
prosthesis (artificial limb) after amputation.
Rotationplasty: Surgery to remove the tumor and the knee
joint. The part of the leg that remains below the knee is then attached
to the part of the leg that remains above the knee, with the foot facing
backward and the ankle acting as a knee. A prosthesis may then be attached
to the foot.
Studies have shown that
survival done is a limb-sparing
surgery or an amputation.
Even if the doctor removes all the cancer that can be seen at the time of the
surgery, patients are also given chemotherapy after surgery to kill any cancer
cells that are left in the area where the tumor was removed or that have
spread to other parts of the body. Treatment given after the surgery, to lower
the risk that the cancer will come back, is called
Chemotherapy is a cancer treatment that uses
drugs to stop the growth of cancer cells, either by killing the cells or by
stopping them from dividing. When chemotherapy is taken by mouth or
injected into a
vein or muscle, the drugs enter the bloodstream and can reach cancer cells
throughout the body
(systemic chemotherapy). When chemotherapy is placed directly into the
cerebrospinal fluid, an
organ, or a body
cavity such as the
abdomen, the drugs mainly affect cancer cells in those areas
Combination chemotherapy is the use of more than one anticancer drug. The
way the chemotherapy is given depends on the type and
stage of the cancer being treated.
In the treatment of osteosarcoma and malignant fibrous histiocytosis of bone,
chemotherapy is usually given before and after surgery to remove the
Radiation therapy is a cancer treatment that uses high-energy
X-rays or other types of
radiation to kill cancer cells or keep them from growito kill cancer cells or keep them from growing. There are two
types of radiation therapy:
External radiation therapy uses a machine outside the body to send
radiation toward the cancer.
Internal radiation therapy uses a
radioactive substance sealed in needles,
seeds, wires, or
catheters that are placed directly into or near the cancer.
The way the radiation therapy is given depends on the type and stage of the
cancer being treated.
Osteosarcoma and MFH cells are not killed easily by external radiation
therapy. It may be used when a small amount of cancer is left after surgery or
used together with other treatments.
Samarium is a radioactive drug that targets areas where bone cells are growing, such as
tumor cells in bone. It helps relieve pain caused by cancer in the bone and it
blood cells in the
bone marrow. It also is used to treat osteosarcoma that has come back after
treatment in a different bone.
Treatment with samarium may be followed by
stem cell transplant. Before treatment with samarium,
stem cells (immature blood cells) are removed from the blood or
bone marrow of the patient and are frozen and stored. After treatment with
samarium is complete, the stored stem cells are thawed and given back to the
patient through an
infusion. These reinfused stem cells grow into (and restore) the body's
New types of treatment are being tested in clinical trials.
Targeted therapy is a treatment that uses drugs or other substances to find
and attack specific cancer cells without harming normal cells.
Kinase inhibitor therapy and
monoclonal antibody therapy are types of targeted therapy being studied in
clinical trials for osteosarcoma.
Kinase inhibitor therapy blocks a
protein needed for cancer cells to divide.
Sorafenib is a type of kinase inhibitor therapy being studied for the
Monoclonal antibody therapy is a cancer treatment that uses
antibodies made in the laboratory, from a single type of
immune system cell. These antibodies can identify substances on cancer cells
or normal substances that may help cancer cells grow. The antibodies attach to
the substances and kill the cancer cells, block their growth, or keep them from
spreading. Monoclonal antibodies are given by infusion. They may be used alone
or to carry drugs,
toxins, or radioactive material directly to cancer cells.
dinutuximab, and glembatumumab are monoclonal antibodies being studied for
the treatment of recurrent osteosarcoma.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a
clinical trial may be the best treatment choice. Clinical trials are part of
the cancer research process. Clinical trials are done to find out if new cancer
treatments are safe and effective or better than the
Many of today's standard treatments for cancer are based on earlier clinical
trials. Patients who take part in a clinical trial may receive the standard
treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer
will be treated in the future. Even when clinical trials do not lead to
effective new treatments, they often answer important questions and help move
Patients can enter clinical trials before, during, or after starting their
Some clinical trials only include patients who have not yet received
treatment. Other trials test treatments for patients whose cancer has not gotten
better. There are also clinical trials that test new ways to stop cancer from
recurring (coming back) or reduce the
side effects of cancer treatment.
Follow-up tests may be needed.
Some of the tests that were done to
diagnose the cancer or to find out the
stage of the cancer may be repeated. Some tests will be repeated in order to
see how well the treatment is working. Decisions about whether to continue,
change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment
has ended. The results of these tests can show if your child's
condition has changed or if the cancer has
recurred (come back). These tests are sometimes called
follow-up tests or check-ups.