Adamantinomas are a rare type of bone cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs that occurs when epithelial tissuea group of cells that work together to perform a function (protective tissue that lines the skin and other organs of the body) grows within osseous (bone) tissue. It is most commonly found in in the bones of the lower legs (tibia and fibula), but can also be found in other bones such as the radius (forearm), ulna (forearm), mandible (jaw), or in the bones of the hands and feet. Adamantinomas found in the bones of the lower legs may also be referred to as extragnathic adamantinomas.
Adamantinomas are slightly more common in males, and is most commonly diagnosed between the ages of 10-30. 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 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. However, because of how rare adamantinomas are, there is currently no standard staging and grading system for this disease. Instead, your doctor will recommend a treatment plan based on the following factors:
- Cancer location.
- Whether or not the cancer has metastasised.
- Your age.
- General health.
- Your treatment preferences.
In general, most adamantinomas are considered to be low-grade (cancer cells present as slightly abnormal and are usually slow growing), and are unlikely to metastasise.
Once your tumoura tissue mass that forms from groups of unhealthy cells 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 adamantinomas may include:
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, potentially including:
- Wide local excisionremoval of cancerous tissue and a margin of healthy tissue around it to prevent cancer recurrence. This option may also be referred to as a limb-sparing surgerysurgery to remove the cancer only and salvage the affected limb.
- Amputationcomplete or partial removal of a limb.
- 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.
Unfortunately, this type of cancer does not often respond to chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells and radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
Risk factors
Because of how rare adamantinomas are, there has been limited research done into the riskthe possibility that something bad will happen factors of this disease.
Symptoms
Many patients with adamantinomas appear asymptomatic in the early stages of disease. As the tumour progresses, some of the following symptoms may appear:
- Sharp or dull pain in affected area.
- Swelling of affected area.
- Redness in affected area.
- Increased pain with activity or lifting.
- Limping (if in the leg).
- Decreased movement of affected area.
In rare cases, the tumour may metastasise to another part of the body, most commonly the lungs. This may cause additional symptoms.
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 a PDS, 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 .
- Bone scana type of medical imaging that uses a radioactive tracer to detect bone conditions or abnormalities.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology 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.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Biopsyremoval of a section of tissue to analyse for cancer cells.