Broida Samuel E, Arguello Alexandra M, Sullivan Mikaela H, Robinson Steven I, Okuno Scott H, Siontis Brittany L, Ho Thanh P, Rose Peter S, Xu-Welliver Meng, Houdek Matthew T
Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN 55905, USA.
Department of Medical Oncology, Mayo Clinic, Rochester, MN 55905, USA.
Cancers (Basel). 2024 Sep 14;16(18):3157. doi: 10.3390/cancers16183157.
: 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. : 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. : 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 ( > 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], < 0.01) and DSS (HR 7.66, 95% CI [1.76, 33.4], < 0.01). Patients who received radiotherapy had improved MFS (HR 6.4, 95% CI [1.42, 29.0], = 0.02) and DSS (HR 5.86, 95% CI [1.27, 26.9], = 0.02). : 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.
滑膜肉瘤较为罕见,可能表现为一个小的、生长缓慢的肿块。这些肿瘤常常被误诊为良性,因此容易被进行非计划性和/或非肿瘤学切除。我们试图确定滑膜肉瘤的非计划性切除率以及该队列中复发和生存的危险因素。
对1997年至2022年在单一机构评估的246例滑膜肉瘤患者的病历进行了回顾性研究。其中,87例(35%)接受了非计划性、非肿瘤学切除。该队列的平均年龄为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)。原发肿瘤的中位最大治疗前尺寸为4.8厘米(四分位间距7 - 2.4)。77例(86%)患者对肿瘤床进行了再次切除,39例(45%)接受了化疗,63例(72%)接受了放疗。
在接受非计划性切除的患者中,1年局部无复发生存率(LRFS)为98%,5年为82%。无转移生存率(MFS)1年为91%,5年为72%。疾病特异性生存率(DSS)1年为98%,5年为72%。在调整肿瘤大小后,与计划性切除的肿瘤相比,接受非计划性切除的肿瘤在复发或生存方面并无更差(>0.10)。肿瘤大小>5厘米、单相亚型和轴向位置与疾病复发风险增加相关。46例患者在再次切除后有残留肿瘤,这与更差的MFS(风险比8.17,95%置信区间[1.89, 35.2],<0.01)和DSS(风险比7.66,95%置信区间[1.76, 33.4],<0.01)相关。接受放疗的患者MFS(风险比6.4,95%置信区间[1.42, 29.0],P = 0.02)和DSS(风险比5.86,95%置信区间[1.27, 26.9],P = 0.02)得到改善。
三分之一的滑膜肉瘤患者在非计划性、非肿瘤学切除后被诊断出来。患有大型轴向肿瘤的患者生存情况更差。大约一半接受非计划性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。我们的结果表明,在滑膜肉瘤非计划性切除后,可行时应考虑进行切缘阴性的再次切除和放疗。