Feasibility and long-term outcomes of surgery for primary thoracic synovial sarcoma

Abstract

Objectives: Primary thoracic synovial sarcoma (SS) is a rare, high-grade, malignancy. Involvement of vital organs is frequent and may decrease the benefits of surgical resection. We reviewed our practice at a highly experienced thoracic-surgery centre to assess early- and long-term outcomes after surgery.

Methods: We conducted a retrospective, observational, single-centre study of patients undergoing curative-intent surgery for primary thoracic SS between 1 January 2000 and 31 January 2021 as part of a multidisciplinary management. We assessed demographics, medical history, histopathology and follow-up information.

Results: We enrolled 20 patients (13 males) with a median age of 40 years old and a median tumour size of 11 cm. Neoadjuvant chemotherapy was administered to 13 patients. Surgery consisted in extrapleural pneumonectomy (n = 7), extrapleural lobectomy (n = 5), chest wall resection (n = 4) or tumour resection (n = 4). R0 resection was achieved in 16 (80%) patients. Adjuvant therapy was given to 13 patients. 6 patients developed postoperative complications. The median hospital stay was 11.5 days. Overall survival at 2 and 5 years was 51% and 22%, respectively; median overall survival was 25 months and median disease-free survival was 8.5 months. Relapses occurred in 15 patients. By univariate analysis, incomplete resection was the only significant predictor of survival (P = 0.01).

Conclusions: Primary thoracic SS is an aggressive disease. Surgery included in a multimodal treatment may contribute to achieving a good outcome, providing that an R0 resection is obtained. Given the considerable technical challenges of surgery, patient selection and referral to an experienced centre are crucial to minimize morbidity and mortality.

Keywords: Feasibility; Primary thoracic synovial sarcoma; Surgical treatment.

Figure 1:

Patients’ selection flow chart.

Figure 2:

Example of a patient with a pulmonary and pleural tumour. (A and B) Twenty-six-year-old female with a 10 cm × 12 cm × 12 cm right intrathoracic mass, pleural lesions and a pleural effusion. (C) She had a partial response to 5 chemotherapy cycles then underwent a right EPP. The postoperative course was uneventful. (D) Operative specimen: histology showed an R0 resection of a biphasic synovial sarcoma that measured 10.5 cm × 7.5 cm × 3 cm and partially infiltrated the diaphragm; the tumour contained a necrotic haemorrhagic component, cystic areas and microcalcifications.

Figure 3:

Kaplan–Meier estimate of overall survival after the diagnosis of primary thoracic synovial sarcoma.

Figure 4:

Kaplan–Meier estimate of disease-free survival after the diagnosis of primary thoracic synovial sarcoma.

Figure 5:

Kaplan–Meier estimate of overall survival after the diagnosis in the groups with tumour-free resection margins (R0) versus contaminated resection margins (R+).