From the Guidelines
Synovial sarcoma of the trachea is a rare and aggressive tumor that requires prompt treatment with surgical resection, adjuvant radiation therapy, and potentially chemotherapy, with doxorubicin and ifosfamide being the preferred chemotherapy regimen, as per the most recent guidelines 1. The diagnosis of synovial sarcoma of the trachea is typically made through a combination of imaging studies, such as CT or MRI scans, and biopsy.
- The treatment approach should be multidisciplinary, involving a team of specialists including surgeons, radiation oncologists, and medical oncologists.
- Surgical resection with wide margins is the primary treatment, and may be followed by adjuvant radiation therapy to reduce the risk of local recurrence, with a dose of 50-60 Gy in fractionated doses, as recommended by previous studies 1.
- Chemotherapy may also be used, either as adjuvant therapy after surgery or as primary treatment for advanced or metastatic disease.
- The preferred chemotherapy regimen includes doxorubicin (75 mg/m² every 3 weeks) and ifosfamide (9-12 g/m² per cycle), as stated in the recent guidelines 1.
- Targeted therapies, such as pazopanib (800 mg daily), may be considered in recurrent or metastatic cases, although their use is still being investigated.
- Tracheal reconstruction may be necessary depending on the extent of resection, and close surveillance is essential with imaging every 3-6 months for the first 2-3 years.
- The prognosis remains guarded, with 5-year survival rates around 50-60%, and treatment at specialized centers with experience in both airway surgery and sarcoma management is strongly recommended to optimize outcomes.
- It is also important to note that the treatment of synovial sarcoma of the trachea is highly individualized and depends on the specific characteristics of the tumor and the patient's overall health, as emphasized in the recent guidelines 1.
From the FDA Drug Label
The majority of patients were female (59%) with a median age of 55 years. Seventy-two percent of patients were white, 22% were Asian, and 6% were other. Forty-three percent of patients had leiomyosarcoma, 10% had synovial sarcoma, and 47% had other soft tissue sarcomas Efficacy results are presented in Table 9 and Figure 2. Table 9 Efficacy Results in STS Patients by Independent Assessment in VEG110727 HR Endpoint/Trial populationPazopanibPlacebo(95% CI) PFS Overall ITTN = 246N = 1230.35 a Median (months)4.61.6(0.26,0.48) Synovial sarcoma subgroupN = 25N = 130.43 Median (months)4.10.9(0.19,0.98)
The diagnosis of Synovial Sarcoma of the Trachea is not directly addressed in the provided drug label. However, for Synovial Sarcoma, the treatment with pazopanib showed a median PFS of 4.1 months compared to 0.9 months for the placebo arm [HR = 0.43 (95% CI: 0.19,0.98)] 2. Pazopanib may be considered as a treatment option for patients with Synovial Sarcoma.
From the Research
Diagnosis of Synovial Sarcoma of the Trachea
- Diagnosis of tracheal synovial sarcoma can be achieved with chromosomal translocation studies along with immunohistochemistry 3
- Immunohistochemically, synovial sarcoma stains positive for cytokeratin, epithelial membrane antigen, vimentin, and S100 3
- Chromosomal translocation t(X;18) (p11;q11) is found in almost all synovial sarcomas and is the gold standard diagnostic modality for these tumors 3, 4
Treatment of Synovial Sarcoma of the Trachea
- Margin-free resection is the gold standard treatment for tracheal synovial sarcoma 3
- Tracheal resection surgery can be an effective treatment option for tracheal synovial sarcoma 3
- Systemic treatment with chemotherapy, such as ifosfamide and doxorubicin, can be used to treat synovial sarcoma, especially in the metastatic setting 5, 4, 6
- Other treatment options, such as pazopanib and trabectedin, have shown activity in synovial sarcoma and can be considered in certain cases 5, 4