A sarcoma is a cancer that arises from transformed cells of mesenchymal (connective tissue) origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or hematopoietic tissues, and sarcomas can arise in any of these types of tissues.
Angiosarcoma,,Chondrosarcoma, Dermatofibrosarcoma protuberans, Desmoplastic small round cell tumors, Epithelioid sarcoma, Ewing sarcoma, Gastrointestinal stromal tumor (GIST), Kaposi’s sarcoma, Leiomyosarcoma, Liposarcoma, Malignant peripheral nerve sheath tumors, Myxofibrosarcoma, Osteosarcoma, Rhabdomyosarcoma, Soft tissue sarcoma, Solitary fibrous tumor, Synovial sarcoma, Undifferentiated pleomorphic sarcoma.
A 61-year-old female with a previous history of recurrent dental cavities presented with a 4 week history of a rapidly growing lesion in the left jaw and was referred to a dentist.
On examination, she was found to have a medial jaw mass causing protrusion of the upper jawline.
A CT scan of the paranasal sinuses showed a soft tissue (27mmby 17mm by 12mm) overlying the inferior aspect of the left maxillary bone anteriorly.
The mass stranded the overlying fat and had destroyed the apex of the left antral sinus with extension to the interior aspect of the sinus and associated mucosal thickening.
Left submental nodes (5mm), submandibular nodes (7mm) were noted.
The rest of the paranasal sinuses were well pneumatized. Hypertrophy of the bilateral inferior turbinates was noted.
The nasopharynx, parotid, submandibular glands were normal. FNAC was advised.
Subsequent biopsy report showed Conventional osteosarcoma.
She was immediately commenced on chemotherapy and has done 5 cycles so far.
She is tolerating the chemotherapy with minimal side effects.
Symptoms depend on tumour type and location. symptoms of sarcoma include:
A lump that can be felt through the skin that may or may not be painful
A broken bone that happens unexpectedly, such as with a minor injury or no injury at all
It’s not clear what causes most sarcomas.
Factors that can increase the risk of sarcoma include:
Inherited syndromes. Some syndromes that increase the risk of cancer can be passed from parents to children. Examples of syndromes that increase the risk of sarcoma include familial retinoblastoma and neurofibromatosis type 1.
Radiation therapy for cancer. Radiation treatment for cancer increases the risk of developing a sarcoma later.
Chronic swelling (lymphedema). Lymphedema is swelling caused by a backup of lymph fluid that occurs when the lymphatic system is blocked or damaged. It increases the risk of a type of sarcoma called angiosarcoma.
Exposure to chemicals. Certain chemicals, such as some industrial chemicals and herbicides, can increase the risk of sarcoma that affects the liver.
Exposure to viruses. The virus called human herpesvirus 8 can increase the risk of a type of sarcoma called Kaposi’s sarcoma in people with weakened immune systems.
Tests and procedures used to diagnose sarcoma and determine its extent (stage) include:
A physical exam. Your doctor will likely do a physical exam to better understand your symptoms and look for other clues that will help with your diagnosis.
Imaging tests. Which imaging tests are right for you will depend on your situation. Some tests, such as X-rays, are better for seeing bone problems. Other tests, such as MRI, are better for seeing connective tissue problems. Other imaging tests might include ultrasound, CT, bone scans and positron emission tomography (PET) scans.
Removing a sample of tissue for testing (biopsy). A biopsy is a procedure to remove a piece of suspicious tissue for lab testing. Sophisticated lab tests can determine whether the cells are cancerous and what kind of cancer they represent. Tests can also reveal information that’s helpful for choosing the best treatments.
Once your doctor determines you have sarcoma, he or she might recommend additional tests to look for signs that the cancer has spread.
Sarcoma is usually treated with surgery to remove the cancer. Other treatments might be used before or after surgery. Which treatments are best for you will depend on the type of sarcoma, its location, how aggressive the cells are and whether cancer has spread to other parts of your body.
Treatment for sarcoma varies depending on sarcoma type, location and other factors.
Treatment for sarcoma might involve:
Surgery. The goal of surgery for sarcoma is to remove all of the cancer cells.
Radiation therapy. Radiation therapy uses high-powered energy beams, such as X-rays and protons, to kill cancer cells. The radiation can come from a machine that moves around your body directing the beams of energy (external beam radiation). Or the radiation might be placed in your body temporarily (brachytherapy). Sometimes radiation is done during an operation to remove the cancer (intraoperative radiation).
Chemotherapy. Chemotherapy is a drug treatment that uses chemicals to kill cancer cells. Some types of sarcoma are more likely to respond to chemotherapy treatment than others.
Targeted therapy. Targeted therapy is a drug treatment that uses medicines that attack specific weaknesses in cancer cells. Your doctor may have your sarcoma cells tested to see if they are likely to respond to targeted therapy drugs.
Immunotherapy. Immunotherapy is a drug treatment that uses your immune system to fight cancer. Your body’s disease-fighting immune system may not attack your cancer because the cancer cells produce proteins that blind the immune system cells. Immunotherapy drugs work by interfering with that process.
Ablation therapy. Ablation therapy treatments destroy cancer cells by applying electricity to heat the cells, very cold liquid to freeze the cells or high-frequency ultrasound waves to damage the cells.
FACTORS THAT AFFECT PROGNOSIS
The AJCC has identified several factors that affect prognosis of bone sarcomas:
Size of the tumor: larger tumors tend to have a worse prognosis compared to smaller tumors.
Spread of tumor to surrounding tissues: tumors that have spread locally to surrounding tissues tend to have a worse prognosis compared to tumors that have not spread beyond their place of origin.
Stage and presence of metastases: tumors that have spread (“metastasized”) to the lymph nodes (which is rare for bone sarcomas) or other organs or tissues (for example, to the lungs) have a worse prognosis compared to tumors that have not metastasized.
Tumor grade: higher grade tumors (grades 2 and 3) tend to have a worse prognosis compared to low grade (grade 1) tumors.
Skeletal location: tumors originating in the spine or pelvic bones tend to have a worse prognosis compared to tumors originating in arm or leg bones.
For soft tissue sarcomas other than GISTs, factors that affect prognosis include:
Stage: as with bone sarcomas, tumors that have metastasized have a worse prognosis compared to tumors that have not metastasized.
Grade: the AJCC recommends using a grading system called the French Federation of Cancer Centers Sarcoma Group (FNCLCC) Grade for soft tissue sarcomas, with high-grade tumors having a worse prognosis compared to low-grade tumors.
For GISTs, the key factor that affects prognosis is:
Mitotic rate: mitotic rate refers to the fraction of cells that are actively dividing within the tumor; GISTs that have a high mitotic rate have a worse prognosis compared to GISTs that have a low mitotic rate.