From the Guidelines
Synovial Sarcoma is a rare type of cancer that usually occurs near the joints of the arm, neck, or leg, and is a type of soft tissue sarcoma 1.
Main Characteristics
The main characteristics of synovial sarcoma include:
- It is a rare and heterogeneous group of solid tumors of mesenchymal origin
- It can occur near the joints of the arm, neck, or leg
- It is a type of soft tissue sarcoma
Treatment Options
The main treatment options for synovial sarcoma include:
- Surgery: usually the primary treatment, aiming to remove the tumor and a margin of healthy tissue around it 1
- Chemotherapy: may be used before surgery to shrink the tumor, or after surgery to kill any remaining cancer cells, with common medications including ifosfamide and doxorubicin 1
- Radiation therapy: may also be used before or after surgery to help control the tumor 1
Importance of Multidisciplinary Care
It is essential to seek medical attention from a qualified healthcare professional, such as an orthopedic oncologist or a medical oncologist, for proper diagnosis and treatment 1. A multidisciplinary team with extensive expertise and experience in the treatment of soft tissue sarcomas is crucial for optimal management 1.
Individualized Treatment Plan
The specific treatment plan will depend on the individual case, including the size and location of the tumor, as well as the overall health of the patient 1. In some cases, a combination of these treatments may be used.
From the Research
Definition and Characteristics of Synovial Sarcoma
- Synovial sarcoma is a rare and highly malignant soft tissue sarcoma 2
- It is the fourth most common type of soft-tissue sarcoma, accounting for 2.5%-10.5% of all primary soft-tissue malignancies worldwide 3
- Synovial sarcoma most often affects the extremities (80%-95% of cases), particularly the knee in the popliteal fossa, of adolescents and young adults (15-40 years of age) 3
- The oncogenic driver is a translocation between chromosomes X and 18 that leads to the formation of the SS18::SSX fusion oncogenes in >95% of cases 4
Clinical Presentation and Diagnosis
- Synovial sarcoma has inconspicuous and diverse early symptoms, making it a highly misdiagnosed disease 2
- The detection of SS18 gene rearrangement is considered a powerful tool in establishing the diagnosis of synovial sarcomas 2
- Biopsies and testing for gene rearrangements are recommended for all patients in whom SS cannot be excluded 2
- Radiographic features of these tumors are not pathognomonic, but findings of a soft-tissue mass, particularly if calcified (30%), near but not in a joint of a young patient, are very suggestive of the diagnosis 3
Treatment and Prognosis
- Surgery is the mainstay of treatment for synovial sarcomas 2
- Neoadjuvant/adjuvant radiotherapy is recommended for patients with big tumors (>5 cm) or positive resection margins, and neoadjuvant/adjuvant chemotherapy is recommended for patients with high-risk tumors or advanced diseases 2
- Synovial sarcoma is an intermediate- to high-grade lesion, and, despite initial aggressive wide surgical resection, local recurrence and metastatic disease are common and prognosis is guarded 3
- The prognosis in the advanced stages is poor, with treatment strategies that have relied heavily on traditional cytotoxic chemotherapies 4