Primary thoracic synovial sarcomas: clinical profile and treatment outcomes of a rare entity managed at a tertiary care centre

Ghazal Tansir; Sameer Rastogi; Ekta Dhamija; Shamim Ahmed Shamim; Deepali Jain; Adarsh Barwad; Sunil Kumar; Rambha Pandey

doi:10.3332/ecancer.2024.1757

Primary thoracic synovial sarcomas: clinical profile and treatment outcomes of a rare entity managed at a tertiary care centre

Ecancermedicalscience. 2024 Sep 6:18:1757. doi: 10.3332/ecancer.2024.1757. eCollection 2024.

Authors

Ghazal Tansir¹, Sameer Rastogi¹, Ekta Dhamija², Shamim Ahmed Shamim³, Deepali Jain⁴, Adarsh Barwad⁴, Sunil Kumar⁵, Rambha Pandey⁶

Affiliations

¹ Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi 110029, India.
² Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi 110029, India.
³ Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi 110029, India.
⁴ Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India.
⁵ Department of Surgical Oncology, All India Institute of Medical Sciences, New Delhi 110029, India.
⁶ Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi 110029, India.

Abstract

Introduction: Primary thoracic synovial sarcoma (PTSS) is a rare malignancy presenting with varying clinical manifestations. There is a paucity of data with few studies dedicated to this unique subset of neoplasms. We present our findings from one of the largest real-world studies among patients with PTSS.

Methods: This is a single-centre, real-world study in patients with PTSS included between 2017 and 2023. Survival estimates were obtained by the Kaplan-Meier method and Cox regression analysis.

Results: 24 patients with a median age of 34.5 years (range 16-54) presented with chest pain (n = 11, 45.8%) and dyspnea (n = 10, 41.6%). Predominant primary sites of disease were the lung (n = 12, 50%) and mediastinum (n = 6, 25%). The stage at presentation was unresectable locally advanced (n = 10, 41.6%), localised (n = 8, 33.3%) and metastatic (n = 6, 25%) with pulmonary metastases (n = 10, 62.5%) and pleural effusion (n = 4, 25%). 16 (66.6%) patients underwent surgical resection including 7 (43.8%) who received neoadjuvant chemotherapy (NACT). NACT was given in ten patients producing stable disease in 5 (50%) and partial response in 3 (30%) patients, respectively, with surgery performed in 7 (70%). 11 (62.5%) operated patients had a microscopically complete resection and 10 (41.6%) received postoperative radiotherapy. Anthracyclines were given in 23 (95.8%) patients in the first line, while pazopanib was the most common therapy in the second and third lines, respectively. At a median follow-up of 32 months (range 16.7-47.2), the median overall survival (OS) was 41 months (95% CI: 23.7-58.2) and 8 months (95% CI: 1-25.6) overall and in metastatic disease, respectively. Presentation with metastases (p = 0.01) and treatment with surgical resection (p = 0.005) were significantly associated with OS on univariate analysis.

Interpretation: The locally advanced nature of the disease at presentation signifies the need for early diagnosis and technically superior definitive therapies. The survival outcomes for metastatic disease remain poor and the need for novel therapies for advanced disease remains unmet so far.

Clinical trial registration: Not applicable.

Keywords: multidisciplinary treatment; primary thoracic sarcoma; synovial sarcoma; thoracic oncology.