Fibrosarcoma is a rare and highly aggressive type of sarcoma (bone and soft tissue cancerA disease where abnormal cells split without control and spread to other nearby body tissue and/or organs. Cancer cells can also spread to other parts of the body through the bloodstream and lymph systems.). It originates from fibroblasts, which are the most common type of cell in connective tissues such as bone, cartilage, and fat. They are most commonly found at the ends of bones in the arms and legs, however it can also develop in the breast, chest wall, lungs, and/or ovaries.
Fibrosarcoma has a slight prevalence in males and is most common in children under 10 and adults between the ages of 20-60; however, anyone can develop this disease.
Types of Fibrosarcoma
There are several types of fibrosarcomas, which can be categorised by their location within the body, as well as the age group they affect.
Fibrosarcoma Subtypes Based on Location
Fibrosarcomas can be classified by their location within the body.
Fibrosarcoma of the Bone
Fibrosarcoma of the bone is the most common subtype of fibrosarcomas, that generally develops in long bones such as the femur (thigh bone) and humerus (upper arm bone). They most commonly develop in males, and generally between the ages of 30-60. There are several types of fibrosarcoma of the bone:
- Primary medullary fibrosarcoma (generally develops around the long bones surrounding the knee – often intermediate to high grade
• A score that tells how quickly a tumour might spread and grow by looking at how the abnormal cells and tissue look under a microscope.
• Grade is not the same as stage.
• Grading is different depending on the type of cancer.
). - Primary surface fibrosarcoma (develops on the surface of bones and soft tissue).
- Secondary fibrosarcoma (originate in another portion of the body and metastasiseFor cancer to spread or grow out of its original place. (spread) into a fibrosarcoma).
- Multi-centric fibrosarcoma (rare – more than one fibrosarcoma present).
- Congenital fibrosarcoma (very rare – fibrosarcoma that runs in families).
The aggressiveness and prognosisTo predict how a disease/condition may progress and what the outcome might be. of fibrosarcomas found in the bone vary based on the subtype diagnosed and the age of the patient.
Fibrosarcoma of the Breast
Fibrosarcomas of the breast are a very rare type of fibrosarcoma that develops in the breast. It is also an incredibly rare form of breast cancer. Because of how rare this subtype is, there has been limited research done into the aggressiveness and prognosis of this disease.
Fibrosarcoma of the Chest Wall
Fibrosarcomas of the chest wall are a very rare type of fibrosarcoma that develop on the wall of the chest. It most commonly forms in adults, and is very rarely found in infants in children. Because of how rare this subtype is, there has been limited research done into the aggressiveness and prognosis of this disease.
Fibrosarcoma of the Lung
Fibrosarcoma of the lung, also known as primary bronchopulmonary fibrosarcoma, is a very rare type of fibrosarcoma that develops in the lungs. In most cases, these tumours develop before the age of one, and are a type of infantile fibrosarcoma. Because of how rare this subtype is, there has been limited research done into the aggressiveness and prognosis of this disease.
Ovarian Fibrosarcoma
Ovarian fibrosarcoma is a very rare form of fibrosarcoma that develops in one or both ovaries. It is also a very rare form of ovarian cancer, and is often found in women over the age of 40. This subtype is known to be aggressive, and may not have as good of a prognosis as other types of fibrosarcoma.
Fibrosarcomas Based on Age
In addition to their location, fibrosarcomas can also be categorised based on the age group that they affect.
Infantile/Congenital Fibrosarcoma
Infantile fibrosarcoma, also known as congenital fibrosarcoma, is a common type of fibrosarcoma that develops in patients under the age of two. In most cases they are found within the first year of life, however they can also be found later or in utero. Infantile fibrosarcoma tends to be less aggressive than adult-type fibrosarcoma, and can have a good prognosis.
Adult-type Fibrosarcoma
Adult-type fibrosarcoma is a rarer type of fibrosarcoma that is most common in adults between the ages of 40-60. These tumours are often found in the lower extremities, such as the thighs, knees, arms, and trunk. This subtype is often aggressive, and may not have as good of a prognosis as other types of fibrosarcoma.
Other Types of Fibrosarcoma
These types of fibrosarcoma aren’t classified by location or age.
Ameloblastic Fibrosarcoma
Ameloblastic fibrosarcoma is a rare form of fibrosarcoma that develops within the teeth. They are generally found in people over the age of 60, however they can occur at any age. These tumours are often aggressive, and may not have as good of a prognosis as other types of fibrosarcoma.
Myxofibrosarcoma
Myxofibrosarcoma is a common type of fibrosarcoma in elderly patients, most commonly between the ages of 60-80. They are often found close to the surface of the skin in the arms and legs, and are rarely found in deeper soft tissues. The aggressiveness and prognosis of myxofibrosarcoma varies with tumour stage and grade.
Treatment
If fibrosarcoma is detected, it will be staged and graded based on size, metastasisKnown as secondary cancer, it grows/spreads from the original/ primary cancer. (whether the cancer has spread to other parts of the body) and how the cancer cells
• the smallest, living parts of the body. Cells work together to form or build the body
• a human is made up of millions of Cells
• Cells reproduce themselves to make sure a body stays working
• sometimes Cells can be abnormal or damaged and these can be cancer cells
look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymphA clear fluid that moves through the body through the lymphatic system, carrying cells that fight infection. nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localisedOnly to one area/place of body. cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advancedAt a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for fibrosarcoma may include:
- Surgery, potentially including:
- Wide en bloc resection.
- Curettage (cancer is scraped out with a small, sharp instrument called a curette).
- Limb-sparing surgery (surgery to remove the cancer only and salvage the limb).
- Amputation (removal of all or part of the affected limb).
- Bone graft (a portion of bone from another part of the body or another person is used to replace the bone lost to cancer).
- Radiation therapy.
- Chemotherapy
• a chemical drug treatment to kill or slow-growing cancer Cells
• these drugs are called cytotoxic drugs
. - Clinical trials.
- Palliative careLessening pain without curing the disease..
For more information on the treatment options, please refer to the Rare Cancers Australia treatment options page.
Risk factors
While the exact cause of fibrosarcoma’s remain unknown, the following factors may increase the risk of developing the disease:
- Increasing age.
- Pre-existing conditions, such as enchondroma, chronicA long-lasting disease that changes slowly. osteomyelitis, Paget’s disease and/or fibrous dysplasia
• A change in the size, shape and the way normal Cells are organised.
• Dysplastic Cells are abnormal but are not cancerous.
. - Previous radiation treatment.
- Irritated tissues from surgery and/or prosthetic implantations.
- Prior bone damage.
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Symptoms
Fibrosarcomas are difficult to diagnose, and often misdiagnosed. Common symptoms of this disease include:
- Pain, tenderness and swelling at infectionWhere germs, bacteria or viruses that are not usually in the body, invade the body and make a person sick. site.
- Loss of range of motion.
- Unexplained/easily broken bone.
Not everyone with the symptoms above will have cancer but see your GP if you are concerned.
Diagnosis/diagnosing
If your doctor suspects you have a fibrosarcoma, they may order the following tests to confirm the diagnosisUsing medical test results, identify and name a disease and/or condition. and refer you to a specialist for treatment.
Physical examination
Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities.
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imagingTaking images/photos of inside body parts using magnet rather than x-ray. (MRITaking images/photos of inside body parts using magnet rather than x-ray.), a computed tomography scan (CT scanA 3-D (three dimensional) x-ray pictures that gives more information than a normal x-ray.), x-rays, bone scan and/or positron emission tomography (PET scanA test that uses a radioactive drug to show a picture of how your tissues and organs are working. Also known as a positron emission tomography scan.), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a bloodhello test may be taken to assessTo measure, look at and learn from. your overall health and help guide treatment decisions.
Biopsy
Once the location of the cancer has been identified, the doctor will perform a biopsyTo take a small piece of body tissue and test it in a laboratory. to remove a section of tissue using a needle. In most cases, this will be done by a core-needle biopsy, which involves a larger, hollow needle being inserted into a small incision above the area to be biopsied. Once a sample has been removed, it will be sent to a lab and analysed for cancer cells.
