Osteofibrous dysplasiathe presence of abnormal cells in a tissue or organ (OFD), also known as ossifying fibroma of the long bones, is a very rare and benignnot cancerous, can grow but will not spread to other body parts type of bone tumoura tissue mass that forms from groups of unhealthy cells that occurs when osseous tissuea group of cells that work together to perform a function (bone tissue) is replaced by fibrous tissue (tissue that makes up tendonsa type of connective tissue that connects muscle to bone and ligaments) in a bone. OFD is most commonly found in the bones of the lower legs (tibia and fibula), but can also be found in other bones such as the humerus (upper arm), radius (forearm), ulna (forearm), clavicle (collarbone), or mandible (jaw).
OFD is generally diagnosed equally among the sexes, and is most commonly found in children under 10 years old. However, anyone can develop this disease.
Treatment
When cancers are detected, they are staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs 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. However, as most OFDs are considered benign, these tumours are generally considered low-grade and not staged.
Once your tumour has been diagnosed, 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, age, stage of disease and overall health.
Treatment options for OFD may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- Surgical excisionto surgically remove/cut out.
- Curettagea procedure where the cancer is scraped out with a small, sharp instrument (curette).
- 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..
- Extraperiosteal excisionremoval of affected bone or bone lesion, as well as surrounding membrane (periosteum) (this is then treated with a bone graft or a prosthesis to fill the hole).
- Bone bracea stabilising device used to prevent limb bowing and further bone damage.
- 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
Because of how rare OFD is, there has been limited research done into the riskthe possibility that something bad will happen factors of this disease.
Symptoms
Many patients with OFD appear asymptomatic in the early stages of disease. As the tumour progresses, some of the following symptoms may appear:
- Pain and/or swelling in affected area.
- Easily broken bones.
- Limb bowing (when the legs curve outwards at the knees while the feet and ankles touch).
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have an OFD, 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:
- X-raya type of medical imaging that uses x-ray beams to create detailed images of the body .
- 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.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.