What is osteosarcoma?
Osteosarcoma is a malignant tumor of the bone. Characteristic of this tumor is that cancer cells produce bone (botschors). "Osteo" means bone and "sarcoma" is a term for a group of malignant (cancerous) tumors. It is the most common bone tumor (so no metastasis of a different type of cancer), and is found particularly in adolescents, the peak is located between the age 10-25 years. A second peak is after age 60 years. A osteosarcoma can occur in principle in the whole body, but occurs in the majority of cases in the upper arm or knee region (60%) in the vicinity of the growth plate. In the Netherlands is about 35-45 cases per year. Osteosarcoma is slightly more common in boys / men than girls / women.
Osteosarcoma causes
It is not known how a osteosarcoma. There is probably damaged a vital piece of DNA in the cell nucleus. People with rare genetic defects have a greater chance of developing an osteosarcoma. This includes those with the Rothmund-Thomson syndrome, Bloom syndrome, Li-Fraumeni syndrome and the familial form of the retinoblastoma. But usually it comes sporadically osteosarcoma, that is to say in a non-hereditary form. Furthermore, it is more often than osteosarcoma described average in the Paget's disease, after the chemotherapeutic treatment and after irradiation.
Osteosarcoma symptoms
One of the first symptoms is pain usually at night and at rest. The pain is usually in or around the bone. Sometimes there is a swelling on. If these are close to the joint, there may arise a disability. Also a spontaneous fracture can occur by involving bone (= pathological fracture). The duration of symptoms is variable, from a few weeks to a few months.
Osteosarcoma diagnosis
Based on the clinical history and physical examination, a number of diagnostic examinations. Namely an x-ray of the affected area and an MRI. This image looks exactly where the tumor is in the bone, how big the tumor is, how far the tumor extends into the surrounding tissues and how the relationship is with the nerves and blood vessels. Metastases are mainly made in the lungs and bones, therefore, for a CT scan of the lungs and a bone scan to detect metastases in the skeleton. In order to establish with certainty that it is a malignant tumor tissue is always required of the tumor. This is eliminated by means of a biopsy. The location of the biopsy is chosen so that the biopsy route later, in the removal of the tumor (resection) may be meeverwijderd to lag behind to prevent malignant cells in the biopsy process. Blood tests are not very specific, but sometimes it is found an increase in alkaline phosphatase and / or LDH.
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| Osteosarcoma pictures |
Osteosarcoma treatment
The treatment of an osteosarcoma consists of chemotherapy and surgery. The goal of chemotherapy is to reduce the risk of metastasis and to kill the tumor cells at the same time as much as possible so that the operation can be easier. The total treatment takes about ½ to 1 year. In brief, the treatment of several weeks of chemotherapy prior to surgery (mean 10 weeks), the operation in which the tumor from the bone is removed. After the operation is followed by a chemotherapy period of 5-8 months, depending on the response to the chemotherapy. Radiotherapy has no place in the initial treatment of osteosarcoma, because the tumor is relatively not very sensitive to radiation. Radiotherapy is sometimes used in specific cases.
Healing chance
The chance of cure of all patients with osteosarcoma is around 60%. The chance of recovery depends on the place where the tumor is located, (how to properly operate, the tumor), the degree of aggressiveness, the response of the tumor to chemotherapy and the existence or non-existence of metastases. No metastases means a greater chance of cure. Because every situation and every person is unique, it remains difficult to make predictions.
Chemotherapy
For 1970, the period that no chemotherapy was still used for osteosarcoma, treatment consisted only of surgery in patients with no metastases (metastases) were found. Surgery consisted in this case often an amputation of the leg or the arm in which it grew osteosarcoma. These patients deceased almost all with recurrent disease. The survival of a patient with an osteosarcoma was 15% in the long term. This means, if one, reasoned that the osteosarcoma at diagnosis was already metastasized, but without that there metastases in the lungs were seen. This is also known as micro-metastases. For this reason, one has started to treat a patient with an osteosarcoma with chemotherapy. Many doctors have been engaged to seek out the best combination of chemotherapeutic agents. Nowadays there are four chemotherapeutic agents (cytostatic drugs) are used which have a good activity against an osteosarcoma. These are:
- methotrexate
- adriamycin
- cisplatin
- ifosfamide
These substances are administered in a combination, which has been found in high-dose Methotrexate, together with the combination of Adriamycin-Cisplatin, a so-called MAP-cure, it is very effective. In elderly patients, may be selected for a Adriamycin / Cisplatin treatment (AP). With such a combination, wherein a portion of the total for chemotherapy, and another part is given after surgery (neo-adjuvant), heals approximately 60% of the patients. Thus, by the addition of chemotherapy increased the survival rate by about 50%. Because osteosarcoma is a rare disease, it is important to combine the treatment in hospitals worked with (inter) national protocols (TRIALS) for osteosarcoma. This collaboration is reflected in clinical behandelprotococollen, such as the EURAMOS-1 protocol.
Fertility and sexuality
This refers to what is done to the late effects of chemotherapy on the reproductive organs to reduce as much as possible. An important late effect of treatment with chemotherapy is damage to the gonads, with a risk of permanent infertility. Ifosfamide and Cisplatin are 2 cytostatic drugs that are used in an osteosarcoma, and such damage can make. In boys sperm can be frozen, girls can ovarian tissues (ovaries) are frozen.
Surgery
In principle, there has been a period of time before surgery with chemotherapy. Two weeks before the scheduled surgery MRI is made to see how the tumor has responded radiologically on chemotherapy. The goal of surgery is to remove all of the tumor tissue (resection) with covering of the healthy surrounding tissue, after which follows the second part of the operation, the recovery (reconstruction) of the affected body part in order to maintain an optimal function. The type of surgery depends on the size, the location and extent of the tumor in the surrounding tissues such as muscle, joint, nerve and blood vessels.
Limb sparing surgery; usually the leg or the arm can be maintained and will be the part that will be removed to be replaced by the following:
- donor bone (allograft)
- own bone (autograft)
- tumor prosthesis
If the joint can be maintained, the defect bridged with a healthy piece of bone from yourself or with donor bone from the bone bank (allograft). In the event of the use of donor bone is growing needs to each of the own and new bone parts lot of time and patience. If the defect is closed near a joint (usually around the knee, sometimes around the shoulder or hip joint) inserted an artificial joint is. The advantage of limb sparing surgery is the maintenance of a functional, leg or arm. The use of a tumor prosthesis allows for faster loading.
- Reversible Plastic: when the tumor is located just above the knee and lower leg and nerve tumor are quite a reversible plastic can be used. After the knee and a part of the upper leg amputated, the lower leg is reversed so with the heel and the toes to the front towards the rear, fixed put to the remaining portion of the thigh. The ankle can then serve as a knee joint, to which a properly functioning prosthesis is being measured.
- Amputation: when the tumor grows through the nerves and blood vessels, and the surgeon's arm or leg can not restore functional amputation is the only option.
Psychosocial
The diagnosis of cancer has a major impact in the lives of people. Even the often invasive treatments have a major impact on the psychological and social wellbeing. Cancer affects not only the body, but affects all areas of life. The disease requires a lot of adaptability of people. The way in which people deal with the effects of cancer diagnosis and treatment has a major impact on their quality of life. Every cancer patient faces the challenge to find a new balance in life. Psychosocial care focuses on the recovery of the quality of life
Medical Follow-up
More and more patients survive cancer through improved diagnosis and treatment. This is good progress, but it also has a downside. In some of the patients, there appear to occur late effects of the treatment. These effects can range from specific physical to psychosocial. Of the drug adriamycin example, we know that this can cause heart damage later in life. But there may be learning and attention problems or issues in the field of fertility and growth. These "late" effects may influence the quality of life, and sometimes make it necessary to further medical care. For these reasons, it remains under control for years. The first years of intensive and later annually.