Background:Primary intrathoracic synovial sarcoma (SS) is a rare entity. The objective of this study was to evaluate survival outcomes for patients with intrathoracic SS presenting with localized disease at diagnosis.Methods:We conducted a retrospective review of 63 patients diagnosed with intrathoracic SS between 1997 and 2020. The Kaplan–Meier method and log-rank test were used to estimate the progression-free survival (PFS), overall survival (OS), local recurrence-free survival (LRFS), and metastasis-free survival (MFS). The hazard ratios were estimated by using Cox proportional hazards regression. Median follow-up time, age-at-diagnosis, and primary tumor size were 31 months (range: 4–218 months), 43 years (range: 18–77), and 7 cm (range: 1–23), respectively.Results:Sixty-two of sixty-three (98%) patients had their primary tumor resected, from whom eighteen (29%) and forty-three (69%) had received neo/adjuvant radiotherapy and chemotherapy, respectively. Median PFS, OS, and MFS were 1.2, 3.0, and 1.1 years, respectively. Based on multivariable analyses, patients with ≥5 cm tumor size had poorer OS (versus < 5 cm; HR: 2.66; 95% CI: 1.16, 6.11; LR-p= 0.014). Importantly, the receipt of neo/adjuvant chemotherapy was the only factor associated with both a more favorable PFS (HR: 0.33; 95% CI: 0.17, 0.65; LR-p= 0.0002) and a more favorable MFS (median 1.33 years versus no chemo 0.5 years; HR: 0.35; 95% CI: 0.17, 0.73; LR-p= 0.005).Conclusions:Outcomes associated with intrathoracic SS remain poor. Factors associated with poorer outcomes include larger tumors and omission of chemotherapy in the management of localized disease. We recommend providing perioperative chemotherapy to all patients with ≥5 cm tumor size to improve progression and metastasis-free survival.
背景：原发性胸腔内滑膜肉瘤是一种罕见疾病。本研究旨在评估诊断时表现为局限性病变的胸腔内滑膜肉瘤患者的生存结局。 方法：我们对1997年至2020年间诊断为胸腔内滑膜肉瘤的63例患者进行了回顾性分析。采用Kaplan-Meier法和时序检验评估无进展生存期、总生存期、局部无复发生存期和无转移生存期。风险比通过Cox比例风险回归模型进行估计。中位随访时间、诊断时年龄和原发肿瘤大小分别为31个月（范围：4-218个月）、43岁（范围：18-77岁）和7厘米（范围：1-23厘米）。 结果：63例患者中有62例（98%）接受了原发肿瘤切除术，其中18例（29%）和43例（69%）分别接受了新辅助/辅助放疗和化疗。中位无进展生存期、总生存期和无转移生存期分别为1.2年、3.0年和1.1年。多变量分析显示，肿瘤大小≥5厘米的患者总生存期较差（对比<5厘米；HR：2.66；95% CI：1.16-6.11；LR-p=0.014）。值得注意的是，接受新辅助/辅助化疗是唯一与更优无进展生存期（HR：0.33；95% CI：0.17-0.65；LR-p=0.0002）和更优无转移生存期（中位1.33年对比未化疗组0.5年；HR：0.35；95% CI：0.17-0.73；LR-p=0.005）均相关的因素。 结论：胸腔内滑膜肉瘤的临床结局仍然较差。预后不良的相关因素包括肿瘤体积较大以及在局限性病变治疗中未使用化疗。我们建议对所有肿瘤大小≥5厘米的患者实施围手术期化疗，以改善无进展生存期和无转移生存期。
Primary Intrathoracic Synovial Sarcoma: An Analysis of Outcomes of This Rare Disease
肿瘤(癌症)患者之家