Fibrosarcoma is a rare and highly aggressive type of sarcomacancer arising from bones and/or soft tissue that 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 gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells).
- Primary surface fibrosarcoma (develops on the surface of bones and soft tissuetissue/the material that joins, holds up or surrounds inside body parts such as fat, muscle, ligaments and lining around joints).
- Secondary fibrosarcoma (originate in another portion of the body and metastasise (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 cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs. 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 tumoura tissue mass that forms from groups of unhealthy cells stage and grade.
Treatment
If fibrosarcoma is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancer cellsthe basic structural and functional unit of all living things look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas 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 (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid.
- 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 tissuea group of cells that work together to perform a function.
- 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 localisedaffecting only one area 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 testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, 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:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- En bloc resectionremoval of the entire tumour in a single piece with a healthy margin of tissue surrounding it.
- Curettagea procedure where the cancer is scraped out with a small, sharp instrument (curette).
- Limb-sparing surgerysurgery to remove the cancer only and salvage the affected limb.
- Amputationcomplete or partial removal of a limb.
- Bone grafta surgical procedure that uses bone tissue from another bone in the body or from another person to repair or rebuild damaged or diseased bones..
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
While the exact cause of fibrosarcoma’s remain unknown, the following factors may increase the riskthe possibility that something bad will happen of developing the disease:
- Increasing age.
- Pre-existing conditions, such as enchondroma, chronica long-lasting disease that changes slowly over time osteomyelitis, Paget’s disease and/or fibrous dysplasiathe presence of abnormal cells in a tissue or organ.
- 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 infectiona condition where harmful pathogens, such as bacteria, viruses or parasites, have entered the body 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
If your doctor suspects you have a fibrosarcoma, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Imagingtests that create detailed images of areas inside the body tests, potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- Bone scana type of medical imaging that uses a radioactive tracer to detect bone conditions or abnormalities.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.
