概述
Synovial sarcoma is a rare and aggressive soft tissue sarcoma that most commonly arises near joints, tendons, or bursae, especially in the arms and legs. Despite the name, it does not originate from synovial cells. It affects young adults and adolescents, with a distinct chromosomal translocation
症状
- Slow-growing painless lump near joints
- Swelling or tenderness
- Decreased range of motion
- Numbness or pressure sensation (if compressing nerves)
病因与风险因素
- Chromosomal translocation
- Mostly sporadic, not hereditary
- More common in males and people under 40
诊断方法
- MRI of the affected limb
- Core needle biopsy for histopathology
- Immunohistochemistry and molecular testing (SS18-SSX fusion gene)
- CT chest to detect lung metastases
治疗方案
- Surgical resection with wide margins (limb-sparing if possible)
- Radiation therapy (pre- or post-op to reduce recurrence)
- Chemotherapy (e.g., doxorubicin, ifosfamide)
- Targeted therapy (e.g., Tazemetostat in trials)
- Clinical trials and genetic-guided approaches
预后情况
- Depends on tumor size, location, margin status, and metastasis
- 5-year survival: 50–70% (localized), ~20–30% (metastatic)
- High recurrence risk — long-term monitoring required
与此类癌症共处
- Rehabilitation post-surgery (physical therapy)
- Pain and nerve symptom management
- Psychological support for young adult patients
- Monitoring for recurrence and lung metastasis
预防与筛查
- No known prevention
- Early evaluation of unusual limb masses
- Genetic counseling not typically required
