Sarcoma (bone and soft tissue cancer) is a composite and complex group of 50 different histological types of malignant tumors occurring in mesenchymal cells in the bone or soft tissue (long bones, musculature, peripheral nerves, fat tissue, and connective tissue).
The international consensus is that sarcoma treatment should take place at highly specialized centers. In Norway, diagnostics and treatment is formally centralized at the regional hospital level.
There exist Nordic guidelines for diagnosis and treatment by the Scandinavian Sarcoma Group. A new database has recently been established by the National Competence Centerfor registering all sarcoma patients in Norway in addition to registration of the treatment given and their results.
Successful treatment requires that patients are treated by a multidisciplinary team for diagnosis and treatment within radiology, pathology/cytology, nuclear medicine, cytogenetics and molecular genetics, surgery and orthopedics, and oncology. It is very important that patients are referred to a sarcoma center before surgery takes place, because surgery performed in hospitals that are not highly specialized centers often causes tumor cell contamination of neighboring structures and poorer local tumor control.
Sarcoma is a rare type of cancer constituting about 1% of all malignant tumors. In Norway, there are about 160 soft tissue sarcomas and 40 bone sarcomas diagnosed annually.
Of all of the soft tissue sarcomas, 60% are localized in the extremities, pelvic wall, and thoracic wall. Twenty percent are found in the abdominal/retroperitoneal area and 20% in the head/neck or the female genitalia.
General Information on Sarcoma
|
|
- What is sarcoma?
- Who is at risk?
- What are the signs and symptoms?
- What can you do to prevent sarcoma?
- How is sarcoma diagnosed?
- What are the treatment options?
What is Sarcoma؟؟
Sarcoma is a rare type of cancer that arises in the bone and soft tissue, which includes fat, muscles, blood vessels, nerves and fibrous tissue. Sarcomas can develop in any part of the body, though they are most commonly seen in the arms and legs. |
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Who is at risk?
There are very few known risk factors for sarcomas. These include:
- Previous radiotherapy treatment
- Family cancer syndromes
- A damaged lymphatic system
Some chemicals are also thought to be associated with sarcoma, though this has not been proven. Smoking, diet and exercise are not linked to developing sarcomas.
*People who think they may be at risk should discuss this with their doctor.
What are the signs and symptoms?
The signs of sarcoma depend on the site where they arise. Patients with bone sarcoma present with the following symptoms.
- Bone swelling and pain which tends to worsen at night
- Develop fractures after minor trauma as the bone is weakened by the tumour
- A new lump that may not necessarily be painful located anywhere in the body that increases in size, and may grow to a large size over time (patients experiencing soft tissue sarcomas)
*A doctor should be consulted should the above symptoms occur.
What can you do to prevent Sarcoma?
Given the limited risk factors, it is difficult to prevent sarcomas. There are no screening tests, and the best approach is for patients with new lumps, or lumps that are increasing in size, to seek medical attention early. This is especially so for patients who have a strong family history of cancer, or patients with previous radiation therapy treatment.
How is Sarcoma diagnosed?
If there are signs and symptoms of sarcoma, your doctor may order some imaging tests. These include, plain x-rays, CT scans, ultrasound scans, MRI scans or even PET or bone scans. The test selected depends on the location of the lump.
After the imaging tests are done, a biopsy is needed to confirm the diagnosis of sarcoma. This can either be done as a needle biopsy, or a surgical biopsy.
Needle biopsies are done using a core biopsy needle. These remove a 1mm core of tissue from the tumour, and can be done in the clinic or under by a radiologist in the radiology centre using imaging guidance. Multiple cores are often needed in order to obtain sufficient tissue for a diagnosis. Fine Needle Aspiration (FNA) biopsies are not used frequently for sarcomas because insufficient tissue is obtained.
Surgical biopsiesmay be required for some tumours, especially bone sarcomas and large soft tissue sarcomas.
Biopsies have to properly planned and should be performed by teams with experience in sarcoma treatment. An improperly performed biopsy may make subsequent removal difficult and may compromise outcomes of treatment.
The specimen taken during the biopsy is processed and reviewed by the pathologist. At times, further these using molecular tests may be required in order to establish the diagnosis.
Once a diagnosis of sarcoma is made, further imaging investigations may be required to complete the staging of the cancer. This helps to assess the prognosis for the patient.
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