What is synovial sarcoma?
Synovial sarcoma is one type of cancer that originates from soft tissues such as nerves, tendons, fats, muscles, and blood vessels. Soft tissue sarcomas are extremely rare and occur in only 1% of all cancer cases. Synovial sarcoma attacks around 900 people (mostly teens and young adults) in the United States each year. This cancer is a high-grade and aggressive tumor that spreads to other body parts or metastasize. Despite the name of synovial sarcoma, it is unrelated to the part of the joint called the synovial tissue. This cancer starts from primitive mesenchymal cells and usually appears as a deep and painless mass that grows rapidly. In some cases, the disease may limit the range of motion and cause pain, if the tumor presses on nearby nerves.
Synovial sarcoma generally develops in the lower limbs but can also occur in the torso, head, neck, and lungs. Patients generally complain of the appearance of small, painless, rapidly growing nodules. The prognosis for patients with synovial sarcoma is generally quite good. The 5-year survival rate is 50-60%, while for 10 years it is 40-50%. However, survival rates continue to improve with aggressive treatments that combine primary radical surgery, radiation, and chemotherapy.
What Causes Synovial Sarcoma?
The cause of synovial sarcoma is not fully understood. However, research shows that genetic alterations may play a role in the progression of the disease. In nearly 90% of cases, there is a rearrangement of chromosome materials between chromosomes 18 and X. This rearrangement alters the function and position of genes, resulting in a fusion transcript that creates a new gene that has never existed before.
Individuals who suffer from the following conditions are found to have a greater risk of suffering from synovial sarcoma:
Neurofibromatosis
Genetic disorders that affect the development of nerve cell tissue. Tumors called neurofibromas (usually derived from the nerves and alter the function of the NF1 gene, which increases the risk of synovial sarcoma.
Li-Fraumeni Syndrome
This rare, congenital genetic disorder increases a person's risk of developing a malignant tumor. The condition affects p53 (the function of genes that function as tumor suppression), so the risk of soft tissue sarcoma increases. Several studies have also shown that exposure to carcinogens and radiation therapy also increases the risk of soft tissue sarcomas.
Main Symptoms of Synovial Sarcoma
In the early stages, synovial sarcoma arises in the form of a rapidly growing and painless inner mass. As it grows larger, the tumor causes swelling and a real lump that can trigger pain, numbness, and limit the range of motion. Other symptoms may also develop depending on the size of the tumor and the anatomical location and whether cancer has spread to other organs or not. The patient may cough up blood or suffer from pneumothorax if cancer has spread to the lungs, which is the most common site of spread or metastasis. If cancer spreads to the bone, symptoms that appear include bone pain and fractures. This disease can trigger gastrointestinal symptoms if it has spread to the abdomen, liver or pelvis.
Diagnosis of Synovial sarcoma
Synovial sarcomas can be diagnosed with the following tests and procedures:
Genetic testing
Used to detect the chromosome translocation between the X chromosome and chromosome 18.
Rontgen
X-rays use X-rays that can show soft tissue tumors and provide information that helps the doctor determine whether the tumor is malignant (cancerous) or benign (non-cancerous). Based on x-rays, your doctor may ask the patient to run another imaging test for confirmation.
Magnetic Resonance Imaging (MRI) and computed tomography (CT) scans
Used to confirm the presence of mass, size, and location. A CT scan of the chest is also done to find out if cancer has spread to the lungs.
Scan or bone scan
Used to determine the cause of bone pain and fractures.
An open needle biopsy or a nucleus
Involves obtaining a sample network for further analysis.
The above test is useful in:
Confirm the diagnosis of synovial sarcoma
Determining the stage of cancer
Determine the most appropriate way of treatment
How to Treat Synovial Sarcoma?
The main treatment of synovial sarcoma is surgical removal of all tumors and also surrounding healthy tissue. Surgery should be performed while ensuring that the function of the affected part of the body will not be disrupted postoperatively. Radiotherapy or chemotherapy may be given before or after surgery. Both therapies are performed before surgery if the tumor has a large size that is difficult to remove safely through surgery. If therapy is performed after the procedure, then the goal is to kill the remaining cancer cells and prevent recurrence.
As much as possible, surgeons use techniques aimed at saving limb-saving surgery. However, in some cases, amputation may be required depending on the size and location of the tumor. This procedure is often followed by postoperative radiation therapy, which is essential if healthy tissue around the tumor approaches the vital neurovascular structure. The most commonly used method is external beam radiation (EBT) where the light emitted from the outside of the patient's body will be targeted at the location of the tumor.
Before beginning any form of cancer treatment, patients will be given information about the risks and possible complications. Among others:
- Negative anesthetic effects
- Infection
- Neurological or vascular injury
- Lung Embolism
- Serial formation
- Severe bleeding
- Wound back open
- Wound infections
After successful treatment, patients are monitored with routine checks and periodic evaluations, which often include the implementation of imaging tests. Patients are advised to consult their physician every 3-6 months for the first two years and then every six months for the next three years. The goal of long-term monitoring is to capture the early signs of metastasis so that the right treatment can be done immediately.
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