Background: Synovial sarcoma is rare and may present as a small, slow-growing mass. These tumors are often mistaken as benign and are therefore prone to unplanned and/or non-oncologic excision. We sought to identify the rate of unplanned excision of synovial sarcoma and risk factors for recurrence and survival among this cohort.Methods: The medical records of 246 patients evaluated at a single institution for synovial sarcoma between 1997 and 2022 were retrospectively reviewed. Of these, 87 (35%) underwent unplanned, non-oncologic excision. The mean age of the cohort was 49 years. Primary tumors were located in the extremity (n = 63), abdomen (n = 6), thorax (n = 7), head/neck (n = 8), and paraspinal region (n = 3). The median maximum pre-treatment dimension of the primary tumor was 4.8 cm (IQR 7–2.4). Seventy-seven (86%) patients underwent re-excision of the tumor bed, 39 (45%) received chemotherapy, and 63 (72%) received radiation therapy.Results: Among patients who underwent unplanned excision, local recurrence-free survival (LRFS) was 98% at 1 year and 82% at 5 years. Metastasis-free survival (MFS) was 91% at 1 year and 72% at 5 years. Disease-specific survival (DSS) was 98% at 1 year and 72% at 5 years. When adjusting for tumor size, tumors which underwent unplanned excision did not have worse recurrence or survival compared to those which had planned excision (p> 0.10). Size > 5 cm, monophasic subtype, and axial location were associated with increased risk of disease recurrence. Forty-six patients had residual tumor following re-excision, which was associated with worse MFS (HR 8.17, 95% CI [1.89, 35.2],p< 0.01) and DSS (HR 7.66, 95% CI [1.76, 33.4],p< 0.01). Patients who received radiotherapy had improved MFS (HR 6.4, 95% CI [1.42, 29.0],p= 0.02) and DSS (HR 5.86, 95% CI [1.27, 26.9],p= 0.02).Conclusions: One-third of patients presenting with synovial sarcoma were diagnosed after unplanned, non-oncologic excision. Patients with large, axial tumors had worse survival. Approximately half of patients who underwent unplanned excision had no residual tumor after pre-operative radiation. The use of radiation was associated with decreased rates of recurrence and improved disease-specific survival. Our results suggest that margin-negative re-resection and radiotherapy should be considered when feasible following unplanned excision of synovial sarcoma.
背景:滑膜肉瘤较为罕见,常表现为生长缓慢的小肿块。这些肿瘤常被误诊为良性,因此易发生计划外和/或非肿瘤学切除。本研究旨在确定滑膜肉瘤计划外切除的发生率,以及该队列中复发和生存的风险因素。 方法:回顾性分析了1997年至2022年间在同一机构接受评估的246例滑膜肉瘤患者的医疗记录。其中87例(35%)接受了计划外的非肿瘤学切除。队列平均年龄为49岁。原发肿瘤位于四肢(n=63)、腹部(n=6)、胸部(n=7)、头颈部(n=8)和脊柱旁区域(n=3)。原发肿瘤治疗前最大径中位数为4.8厘米(四分位距7-2.4)。77例(86%)患者接受了肿瘤床再切除,39例(45%)接受了化疗,63例(72%)接受了放射治疗。 结果:在接受计划外切除的患者中,1年和5年局部无复发生存率分别为98%和82%。1年和5年无转移生存率分别为91%和72%。1年和5年疾病特异性生存率分别为98%和72%。在校正肿瘤大小后,接受计划外切除的肿瘤与计划切除的肿瘤相比,复发或生存结局并未更差(p>0.10)。肿瘤大小>5厘米、单相亚型和轴心位置与疾病复发风险增加相关。46例患者在再切除后仍有残留肿瘤,这与较差的无转移生存率(风险比8.17,95%置信区间[1.89, 35.2],p<0.01)和疾病特异性生存率(风险比7.66,95%置信区间[1.76, 33.4],p<0.01)相关。接受放疗的患者无转移生存率(风险比6.4,95%置信区间[1.42, 29.0],p=0.02)和疾病特异性生存率(风险比5.86,95%置信区间[1.27, 26.9],p=0.02)有所改善。 结论:三分之一的滑膜肉瘤患者在计划外的非肿瘤学切除后被诊断。肿瘤较大、位于轴心位置的患者生存结局较差。约半数接受计划外切除的患者在术前放疗后无残留肿瘤。放疗的使用与复发率降低和疾病特异性生存率提高相关。我们的研究结果表明,在滑膜肉瘤计划外切除后,条件允许时应考虑切缘阴性的再次切除和放射治疗。
Unplanned Excision of Synovial Sarcoma: Factors Associated with Recurrence and Survival