Patel Riddhi R, Bishop Andrew J, Lazar Alexander J, Lin Patrick P, Benjamin Robert S, Patel Shreyaskumar R, Ludwig Joseph, Ravi Vinod, Vaporciyan Ara A, Araujo Dejka M
Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd. Unit 450, Houston, TX 77030, USA.
Department of Epidemiology, The University of Texas Health Science Center, Houston, TX 77030, USA.
Cancers (Basel). 2025 Feb 22;17(5):745. doi: 10.3390/cancers17050745.
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. 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. 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- = 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- = 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- = 0.005). 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.
原发性胸内滑膜肉瘤（SS）是一种罕见的疾病。本研究的目的是评估诊断时表现为局限性疾病的胸内SS患者的生存结局。我们对1997年至2020年间诊断为胸内SS的63例患者进行了回顾性研究。采用Kaplan-Meier法和对数秩检验来估计无进展生存期（PFS）、总生存期（OS）、无局部复发生存期（LRFS）和无转移生存期（MFS）。通过Cox比例风险回归估计风险比。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 cm（范围：1 - 23 cm）。63例患者中有62例（98%）进行了原发肿瘤切除，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PFS、OS和MFS分别为1.2年、3.0年和1.1年。基于多变量分析，肿瘤大小≥5 cm的患者OS较差（与<5 cm相比；HR：2.66；95%CI：1.16，6.11；P = 0.014）。重要的是，接受新辅助/辅助化疗是唯一与更有利的PFS（HR：0.33；95%CI：0.17，0.65；P = 0.0002）和更有利的MFS（中位1.33年与未化疗的0.5年相比；HR：0.35；95%CI：0.17，0.73；P = 0.005）相关的因素。胸内SS的相关结局仍然较差。与较差结局相关的因素包括肿瘤较大以及在局限性疾病管理中未进行化疗。我们建议为所有肿瘤大小≥5 cm的患者提供围手术期化疗，以改善无进展和无转移生存期。
