Surgery and chemotherapy, with or without radiation therapy

Several treatment centers advocate chemotherapy after resection and radiation therapy for children and young adults with synovial sarcoma.[19,20,40-42]

The International Society of Pediatric Oncology-Malignant Mesenchymal Tumors studies showed that select patients (young age, <5 cm resected tumors) with nonmetastatic synovial sarcoma treated with chemotherapy can have excellent outcomes in the absence of radiation therapy. However, it is still unclear whether that approach obviates an advantage of radiation for local or regional control.[41]

A German trial suggested a benefit for postoperative chemotherapy in children with synovial sarcoma.[42]

A meta-analysis also suggested that chemotherapy may provide benefit.[19]

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients.

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients.

Overall, R0 resection or R1 resection (microscopically positive margins) of the primary tumor was achieved in 129 patients (93.5%): 69 patients (53.5%) at study entry and 60 patients (46.5%) after neoadjuvant chemotherapy. Of these, 104 patients (80.6%) had an R0 resection: 55 patients (53%) at study entry and 49 patients (47%) after neoadjuvant chemotherapy.

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients.

In the 60 patients who received neoadjuvant chemotherapy, response was evaluable in 55 patients. Two patients (3.6%) had complete responses, 9 (16.4%) had partial responses, 41 (74.6%) had stable disease, and 3 (5.5%) had progressive disease. The tissue from 57 tumors was centrally reviewed after definitive resection. Forty-one tumors (72%) had less than 90% necrosis, and 16 tumors (28%) had 90% necrosis or more.

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients.

The study prospectively defined three risk groups:

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients. › The study prospectively defined three risk groups:

Low risk (about 50% of population): Patients with nonmetastatic, grossly resected tumors, except patients who had tumors that were both high grade and >5 cm in maximal diameter.

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients. › The study prospectively defined three risk groups:

Intermediate risk (about 35% of population): Patients with nonmetastatic tumors that were both high grade and >5 cm in maximal diameter and patients with nonmetastatic, nonresectable tumors regardless of grade and size.

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients. › The study prospectively defined three risk groups:

High risk (about 15% of population): Patients with metastatic tumors, including those with metastases restricted to regional lymph nodes.

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients.

For the 46 patients in the low-risk group, the 5-year EFS rate was 81.9% (95% CI, 69%–94.8%), and the OS rate was 97.7% (95% CI, 92.7%–100%).

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients.

For the 23 patients in the intermediate-risk group (treatment arm C), the 5-year EFS rate was 64% (95% CI, 42.4%–85.8%), and the OS rate was 89.5% (95% CI, 75.3%–100%).

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients.

For the 49 patients in the intermediate-risk group (treatment arm D), the 5-year EFS rate was 71.2% (95% CI, 56.5%–85.9%), and the OS rate was 86.5% (95% CI, 75.6%–97.3%).

The COG reported an analysis of the subset of patients with synovial sarcoma treated on the ARST0332 (NCT00346164) trial. This was a prospective treatment assignment trial for patients younger than 30 years with NRSTS.[43] They analyzed the outcomes of 138 eligible patients.

For the 21 patients in the high-risk group, the 5-year EFS rate was 7.6% (95% CI, 0%–22%), and the OS rate was 12.5% (95% CI, 0%–28.7%).

The EpSSG performed a prospective study of patients younger than 21 years with synovial sarcoma (CCLG-EPSSG-NRSTS-2005 [NCT00334854]).[44][Level of evidence C1] Patients were stratified into the following three risk groups and nonrandomly assigned to treatment by risk group:Outcomes for patients treated on the CCLG-EPSSG-NRSTS-2005 trial are described in Table 12.

The EpSSG performed a prospective study of patients younger than 21 years with synovial sarcoma (CCLG-EPSSG-NRSTS-2005 [NCT00334854]).[44][Level of evidence C1] Patients were stratified into the following three risk groups and nonrandomly assigned to treatment by risk group:Outcomes for patients treated on the CCLG-EPSSG-NRSTS-2005 trial are described in Table 12.

Low-risk patients had IRS group I tumors less than 5 cm in size and nonaxial primary tumors.

The EpSSG performed a prospective study of patients younger than 21 years with synovial sarcoma (CCLG-EPSSG-NRSTS-2005 [NCT00334854]).[44][Level of evidence C1] Patients were stratified into the following three risk groups and nonrandomly assigned to treatment by risk group:Outcomes for patients treated on the CCLG-EPSSG-NRSTS-2005 trial are described in Table 12.

Intermediate-risk patients had no axial primary tumors and IRS group I tumors greater than 5 cm or IRS group II tumors.

The EpSSG performed a prospective study of patients younger than 21 years with synovial sarcoma (CCLG-EPSSG-NRSTS-2005 [NCT00334854]).[44][Level of evidence C1] Patients were stratified into the following three risk groups and nonrandomly assigned to treatment by risk group:Outcomes for patients treated on the CCLG-EPSSG-NRSTS-2005 trial are described in Table 12.

High-risk patients included all patients with axial primary sites (head and neck, lung and pleura, trunk, retroperitoneal), IRS group III tumors, or N1 tumors.

The CWS reported results from a prospective trial for the treatment of patients with synovial sarcoma. Eligibility was restricted to patients with localized tumors with macroscopic residual disease after first surgery, before the initiation of systemic therapy (IRS III) and no clinically detectable metastatic disease. There were 145 patients in the study with a median age of 14.5 years (range, 0.2–33.2 years). The protocols recommended but did not require radiation therapy to be given before definitive tumor resection. Radiation therapy was administered to 115 patients (79%), and 23 patients did not receive radiation therapy (no information documented for 7 patients). Of the 115 irradiated patients, 57 were irradiated before tumor excision and 52 after tumor excision.[45]

The CWS reported results from a prospective trial for the treatment of patients with synovial sarcoma. Eligibility was restricted to patients with localized tumors with macroscopic residual disease after first surgery, before the initiation of systemic therapy (IRS III) and no clinically detectable metastatic disease. There were 145 patients in the study with a median age of 14.5 years (range, 0.2–33.2 years). The protocols recommended but did not require radiation therapy to be given before definitive tumor resection. Radiation therapy was administered to 115 patients (79%), and 23 patients did not receive radiation therapy (no information documented for 7 patients). Of the 115 irradiated patients, 57 were irradiated before tumor excision and 52 after tumor excision.[45]

In this nonrandomized comparison, the sequencing of radiation therapy before definitive resection was associated with a statistically significant improvement in local recurrence-free survival rates, compared with definitive surgery before radiation therapy.

The CWS reported results from a prospective trial for the treatment of patients with synovial sarcoma. Eligibility was restricted to patients with localized tumors with macroscopic residual disease after first surgery, before the initiation of systemic therapy (IRS III) and no clinically detectable metastatic disease. There were 145 patients in the study with a median age of 14.5 years (range, 0.2–33.2 years). The protocols recommended but did not require radiation therapy to be given before definitive tumor resection. Radiation therapy was administered to 115 patients (79%), and 23 patients did not receive radiation therapy (no information documented for 7 patients). Of the 115 irradiated patients, 57 were irradiated before tumor excision and 52 after tumor excision.[45]

Omission of radiation therapy was associated with an inferior outcome.

The CWS reported results from a prospective trial for the treatment of patients with synovial sarcoma. Eligibility was restricted to patients with localized tumors with macroscopic residual disease after first surgery, before the initiation of systemic therapy (IRS III) and no clinically detectable metastatic disease. There were 145 patients in the study with a median age of 14.5 years (range, 0.2–33.2 years). The protocols recommended but did not require radiation therapy to be given before definitive tumor resection. Radiation therapy was administered to 115 patients (79%), and 23 patients did not receive radiation therapy (no information documented for 7 patients). Of the 115 irradiated patients, 57 were irradiated before tumor excision and 52 after tumor excision.[45]

Outcomes for patients are described in Table 13.