Adamantinoma
June 11th, 2008 Posted by: admin
Definition
An adamantinoma is a slow growing cancerous bone tumor that is most often found in the shin bone (tibia) or the jaw bone (mandible). Sometimes, these tumors might be found in the forearm, hands or feet. It is a rare tumor, occurring primarily in males between the ages of 10-30, usually after skeletal maturity is reached.
In about 20 percent of cases, adamantinoma cancer cells spread to other parts of the body (metastasize), usually to the lungs or nearby lymph nodes.
Causes
The cause of adamantinoma is yet unknown. Patients with adamantinoma usually have a history of trauma to the affected bone, but it is unclear whether trauma or the tumor appeared first and then produced the other one.
Symptoms
Adamantinoma is characterized by a group of several symptoms. However, each child may experience symptoms differently. Symptoms may include, but are not limited to, the following:
- pain (sharp or dull) at the site of the tumor
- swelling and/or redness at the site of the tumor
- increased pain with activity or lifting
- limping
- decreased movement of the affected limb
The period of having the symptoms might vary between 2-4 weeks to minimum 6 months Often, an injury brings a child into a medical facility, where an x-ray may indicate suspicious bone lesions.
Diagnose
In addition to a complete medical history and physical examination, diagnostic procedures for adamantinoma include (not necessarily in this order):
- biopsy of the
- Complete blood count (CBC)
- Blood tests - (including blood chemistries)
- Multiple imagine studies of the tumor and sites of possible metastasis, such as:
- x-rays
- Magnetic Resonance Imaging (MRI)
- Computerized Tomography scan (also called CT or CAT scan)
- Bone scans
Staging
Once adamantinoma is diagnosed, the next step is to find out how far the tumor has progressed. Staging the tumor indicates how far the tumor has spread from its original location. This is very important because it give clues about which form of treatment is most appropriate, and which could be the prognosis of the patient (especially if is a child).
More often, adamantinoma does not spread beyond the bone where it arose or beyond nearby tissues. Although, in 20 percent of cases, the tumor becomes metastatic, meaning it spreads to lungs, lymph nodes, bones other than the bone that the tumor originated in, or to other organs or structures of the body.
Treatment
Treatment options will vary greatly, depending on your child’s individual situation.
Specific treatment for adamantinoma will be determined by your child’s physician based on:
- Your child’s age
- Extent of the disease
- Your child’s tolerance for specific medications, procedures, or therapies
- How your child’s doctor expects the disease may progress
- Your opinion or preference
Treatment for most bone tumors can include a combination of surgery, chemotherapy and/or radiation. However, adamantinomas have proven to be highly resistant to both chemotherapy and radiation, making surgery alone the option of choice.
Surgery
Surgery for adamantinoma involves the biopsy, surgical removal of the tumor, bone/skin grafts, limb salvage procedures, amputation, and/or reconstruction, all performed by an orthopaedic surgeon. The type of surgery will depend on the size and location of the tumor, and whether the cancer has spread.
Types of Surgery - Limb-salvage surgery
- Amputation
Supportive care
Supportive care refers to any type of treatment to prevent and treat infections, side effects of treatments and complications and to keep your child comfortable during treatment.
Continuous follow-up care
A schedule of follow-up care determined by your child’s physician and other members of yoru care team to monitor ongoing response to treatment and possible late effects of treatment. Your child’s physician and other members of your care team will discuss these with you in-depth.
What is the long-term outlook for patients with adamantinoma? Prognosis for adamantinoma greatly depends on:
- The extent of the disease
- The size and location of the tumor
- Presence or absence of metastasis
- The tumor’s response to therapy
- The age and overall health of the child
- Your child’s tolerance of specific medications, procedures or therapies
- New developments in treatment
As with any cancer, prognosis and long term survival can vary greatly from child to child. Every child is unique and treatment and prognosis is structures around the child’s needs. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with adamantinoma. Second malignancies can occur in some survivors of this disease. It should be neglected the horizon pffered by the new methods that are continually being discovered to improve treatment.