What are the chances of surviving synovial sarcoma?
- ashleyligon1967Lv 51 decade agoFavorite Answer
Pathology and Molecular Biology
Patients with localized synovial sarcoma have a good prognosis and most become long-term survivors. In contrast, those who have metastatic disease have a dismal outlook and only a minority survive. The prognostic features most frequently associated with an unfavorable outcome include tumor size greater than 5 cm, invasion into bone or neurovascular structures, and high histologic grade [7, 8]. Older age, nonextremity primary site, and the biphasic histologic pattern have also been associated with a less favorable outcome.
Background: Synovial sarcoma comprises 8-10% of all sarcomas and most commonly affects adults in the third-to-fifth decades of life. The malignancy most commonly involves the extremities, especially the lower extremities around the knees. Synovial sarcoma frequently is misdiagnosed as benign often because of its small size, slow growth, and well-defined appearance.
Pathophysiology: Gross specimens are usually well-demarcated, pink, fleshy masses with a heterogeneous appearance, and specimens may display solid, hemorrhagic, or cystic components on sectioning. Calcification foci occasionally are noted. Heavy calcification tends to indicate less aggressive lesions and offers a more favorable prognosis.
Synovial sarcoma is so named because of its resemblance to developing synovial tissue under light microscopy. It arises from pluripotential mesenchymal cells near joint surfaces, tendons, tendon sheaths, juxta-articular membranes, and fascial aponeuroses. The histologic appearance is that of large polygonal cells (epithelioid) that secrete hyaluronic acid and show an organization suggestive of microscopic joint spaces. These cells are surrounded by spindle cells that simulate subsynovial mesenchymal cells.
Typical morphology is that of 2 strikingly distinct well-differentiated cell populations. Depending on which cell type predominates, overall histologic appearances can be described as biphasic (epithelioid and spindle cell), monophasic spindle cell, or monophasic epithelioid. Marked cellular pleomorphism and atypia are uncommon, and when present, the appearance overlaps with that of high-grade malignant fibrous histiocytoma and fibrosarcoma.
Specific cytogenetic abnormalities have been identified. More than 90% of patients have a t(X;18) translocation mutation, which is not associated with other sarcomas. The translocation involves the SYT gene on chromosome 18 (at 18q11) and the SSX1 or SSX2 gene on the X chromosome (at Xp11). These genes appear to be transcription regulators, whose functions occur primarily through protein-protein interactions. Subtypes of these translocations have been shown to correlate with distinct histologic subtypes.
In the US: Synovial sarcoma is the fourth most commonly occurring sarcoma, accounting for 8-10% of all sarcomas. Approximately 800 new cases of synovial sarcoma are diagnosed in per year in the United States.
Overall, survival rates are 23.5-64% at 5 years and 11.2-34% at 10 years.
For synovial sarcoma of the head and neck region, the prognosis is better than that of sarcoma involving the extremities, with 5-year survival rates of 47-82%.
Sex: A slight male predilection exists in synovial sarcoma. The male-to-female ratio is 3:2.
Age: Synovial sarcoma can occur in patients with a wide age range, but it is most common in patients in the third-to-fifth decades of life. In one series of 121 cases, 83.6% of tumors occurred in patients aged 10-50 years, with a median age of 31.3 years. Another large study included patients with ages ranging from 3 to 85 years.
Anatomy: Synovial sarcoma is the most common sarcoma involving the upper extremity, hip, groin, and buttocks in patients aged 16-25 years. In patients aged 6-45 years, synovial sarcoma is the most common sarcoma in the foot and ankle.
Most synovial sarcomas are found within 5 cm of a joint. Despite the misnomer, only 10% of cases are intra-articular. Tumors are usually well circumscribed, but in unusual cases, they may interdigitate between muscles and tendons or encase neurovascular structures. Invasion of adjacent bone is seen in 11-20% of patients, a feature that is uncommon in other sarcomas.
The region around the knee is the most common site of involvement. In 1 large study, 73% of synovial sarcomas occurred in the lower limb; 34%, in the upper limb; and 16%, in the chest/abdominal wall. Tumors that occur in the upper extremity tend to affect the distal extremity rather than the elbow or shoulder. Less common sites of involvement include the retroperitoneum, mediastinum, and head and neck regions. The most common site in the head and neck is the hypopharynx. Other head and neck locations include the cervical or parapharyngeal regions, masticator space, soft palate, tongue, suboccipital and infratemporal fossa regions, and sinonasal space.
The clinical features of synovial sarcoma are nonspecific. No features distinguish synovial sarcoma from other sarcomas. Most commonly, patients notice a slowly enlarging, deep-seated mass, which is painful in slightly more than one half of patients.
The mass may be present for an extended period before medical evaluation is sought, with an average of approximately 2.5 years before presentation (range, several months to 20 years). Deep-seated tumors tend to be evaluated later than superficial tumors.
In larger joints, such as the knee, vague symptoms of pain may occur for months without a mass being appreciated. Tumors near joint spaces may cause limitation of movements if they grow sufficiently large.
Involvement at sites other than the extremities presents occasionally as a painful mass or with symptoms related to mass effect on adjacent structures. In head and neck involvement, patients complain of symptoms such as dyspnea, dysphagia, hoarseness, and headache.
Rarely, a patient may present with symptoms secondary to pulmonary metastases, such as hemoptysis.
Synovial sarcomas are slow growing, but they can be locally aggressive. Approximately one fourth of synovial sarcomas are associated with pulmonary metastases at the time of initial presentation. The natural history of synovial sarcoma includes local recurrence, especially when resection margins demonstrate positive results on pathology; this recurrence usually appears within 2 years of initial treatment. Recurrences delayed for as long as 10 years are not uncommon.
Although local control of sarcomas has improved with curative resection and adjuvant irradiation, metastases eventually develop in most patients. The sites most commonly involved are the lung and skeleton. Regional lymph nodes are involved in 10-23% of patients.
Tumors are staged according to the American Joint Committee on Cancer staging classification for soft-tissue sarcomas. This system considers the size of the tumor, invasion of adjacent structures (bone, major vessels, major nerves), lymph node involvement, histologic grade, and presence of distant metastases.
Previous studies have been performed to identify important prognostic predictors. Results indicate that a younger age, a smaller tumor size, a distal limb location, and negative resection margins are correlated with improved outcomes. Adjuvant radiation therapy is also correlated with improved outcomes.
Controversy exists over the prognostic implications of histologic subtypes. Some study results have suggested that lesions in which the histology is primarily epithelioid or that heavily calcified lesions are associated with lower risks of early metastasis and improved long-term survival rates. Invasion of bone and neurovascular structures, marked cellular atypia, and distant metastases are associated with a poor prognosis.
Preferred Examination: As with all sarcomata, MRI is the imaging procedure of choice because of its excellent tissue contrast and ability to depict the lesion in multiple planes. MRI is useful for evaluating the extent of the tumor and its involvement of adjacent soft-tissue structures. For instance, MRI is helpful in the differentiation of tumor from muscle tissue and in depicting the involvement of neurovascular structures, tendons, fascial/fat planes, and bone marrow. MRI is also helpful for the differentiation of recurrent soft-tissue tumors from postsurgical or postirradiation changes.
CT can be used in lieu of MRI in patients with contraindications (eg, claustrophobia, pacemakers, aneurysm clips). As with MRI, CT can be useful in determining the gross anatomic extent of the tumor. CT is especially useful in depicting calcifications, bone invasion, or periosteal reaction.
Limitations of Techniques: MRI has proven to be valuable in the detection and staging of soft tissue tumors, but MRI signal intensity characteristics are usually nonspecific for histologic diagnosis, with some exceptions (eg, lipomas, some liposarcomas, pigmented villonodular synovitis). Although certain signs can suggest synovial sarcoma in the differential diagnosis, MRI findings are by no means pathognomic, and histologic analysis of tissue is usually required for definitive diagnosis.Source(s): http://www.ajronline.org/cgi/content/full/179/3/79... http://www.emedicine.com/radio/topic668.htm
- 1 decade ago
About 50 percent of synovial sarcomas develop in the legs, especially the knees. The second most common location is the arms . Less frequently, the disease develops in the trunk, head and neck region, or the abdomen . It is common for synovial cancer to recur (come back), usually within the first two years after treatment. Half of the cases of synovial sarcoma metastasize (spread to other areas of the body) to the lungs, lymph nodes, or bone marrow.Sorry for telling you this but I did a search a while ago for homework.
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- Anonymous4 years ago
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What is synovial sarcoma? Synovial sarcoma is a type of soft tissue sarcoma. Soft tissue sarcomas are cancers of the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body, including synovial tissue. Synovial tissue lines the cavities of joints, such as the knee or elbow, tendons (tissues that connect muscle to bone), and bursae (fluid-filled, cushioning sacs in the spaces between tendons, ligaments, and bones). Although synovial sarcoma does not have a clearly defined cause, genetic factors are believed to influence the development of this disease. How often does synovial sarcoma occur? Synovial sarcoma is rare. It accounts for between 5 and 10 percent of the approximately 10,000 new soft tissue sarcomas reported each year (1). Synovial sarcoma occurs mostly in young adults, with a median age of 26.5 (1). Approximately 30 percent of patients with synovial sarcoma are younger than 20. This disease occurs more often in men than in women (1). Where does synovial sarcoma develop? About 50 percent of synovial sarcomas develop in the legs, especially the knees. The second most common location is the arms (2). Less frequently, the disease develops in the trunk, head and neck region, or the abdomen (1, 2). It is common for synovial cancer to recur (come back), usually within the first two years after treatment. Half of the cases of synovial sarcoma metastasize (spread to other areas of the body) to the lungs, lymph nodes, or bone marrow (1). What are the symptoms of synovial sarcoma? Synovial sarcoma is a slow-growing tumor. Because it grows slowly, a person may not have or notice symptoms for some time, resulting in a delay in diagnosis. The most common symptoms of synovial sarcoma are swelling or a mass that may be tender or painful (1). The tumor may limit range of motion or press against nerves and cause numbness. The symptoms of synovial sarcoma can be mistaken for those of inflammation of the joints, the bursae, or synovial tissue. These noncancerous conditions are called arthritis, bursitis, and synovitis, respectively. How is synovial sarcoma diagnosed? The doctor may use the following procedures and tests to diagnose synovial sarcoma: Biopsy: Tissue is removed for examination under a microscope. Immunohistochemical analysis: Tumor tissue is tested for certain antigen and antibody interactions common to synovial sarcoma. Ultrastructural findings: The tissue is examined using an ultramicroscope and electron microscope. Genetic testing: Tissue is tested for a specific chromosome abnormality common to synovial sarcoma. How is synovial sarcoma treated? The type of treatment depends on the age of the patient, the location of the tumor, its size, its grade (how abnormal the cancer cells look under a microscope and how likely the tumor will quickly grow and spread), and the extent of the disease. The most common treatment is surgery to remove the entire tumor with negative margins (no cancer cells are found at the edge or border of the tissue removed during surgery). If the first surgery does not obtain negative tissue margins, a second surgery may be needed. The patient may also receive radiation therapy before or after surgery to control the tumor or decrease the chance of recurrence (cancer coming back). The use of intraoperative radiation therapy (radiation aimed directly at the tumor during surgery) and brachytherapy (radioactive material sealed in needles, wires, seeds, or catheters, and placed directly into or near a tumor) are under study. Patients may also receive chemotherapy alone or in combination with radiation therapy
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- 1 decade ago
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