Karaca Mustafa Onur, Başarır Kerem, Dursun Savran Merve, Yüksel Seher, Kaygusuz Gülşah, Yıldız Hüseyin Yusuf
Ankara University Medical Faculty, İbni Sina Hospital, Department of Orthopedics and Traumatology, Talatpasa Boulevard no:82, 06630 Ankara, Turkey.
Orthopedics and Traumatology, Kızılırmak, Dumlupınar Blv. Next Level Office A Block No: 34, 06530 Çankaya/Ankara, Turkey.
Orthop Traumatol Surg Res. 2025 Sep;111(5):104160. doi: 10.1016/j.otsr.2025.104160. Epub 2025 Jan 11.
Synovial sarcoma (SS) is classified as an aggressive high-grade soft tissue sarcoma that predominantly affects the extremities. Despite its prevalence in the extremities (up to 80%), diagnostic and treatment challenges persist. This study aims to address these challenges by providing a comprehensive analysis of SS in extremities, focusing on diagnostic accuracy and treatment outcomes. The central questions of this study are: What are the diagnostic and treatment challenges associated with SS? How do tumor volume and histologic subtype influence prognosis? What role do immunohistochemistry and genetic markers play in SS diagnosis and management?
We hypothesize that larger tumor volume and poor histologic differentiation are associated with worse survival outcomes in patients with SS.
This retrospective study analyzed data from 63 patients diagnosed with SS between 2005 and 2020 at a single center. Patients with complete records of pathology, radiology, and surgery, and a minimum follow-up of 12 months were included. Tumor characteristics, treatment modalities, and follow-up data were reviewed.
The study included 63 patients and 65 tumors. The mean age was 38 ± 17 years. There were 31 females (49%) and 32 males (51%). SS predominantly affected the extremities (n = 63, 97%), especially the lower limbs (n = 49, 75%). Tumor volume, with a mean of 110 ± 176 cm³, was a significant factor, with tumors >30 cm³ associated with higher rates of metastasis (p = 0,006) and reduced survival (p = 0,027). Histologically, 25 (38%) were monophasic, 24 (37%) were biphasic, and 16 (25%) were poorly differentiated, with poorer survival linked to poorly differentiated subtypes. Immunohistochemistry showed high positivity rates for Bcl-2 (89%, 17/19), EMA (88%, 52/59), and TLE1 (87%, 13/15). SS18-SSX fusion gene detected in 73% of cases (8/11). Metastasis occurred in 27 (42%) patients and recurrence in 24 (37%). 15 (23%) patients died from the disease.
Accurate diagnosis of SS is crucial for effective management. Clinicians should be aware of negative predictive factors, including tumor volume >30 cm³ and poor histologic differentiation, when making treatment decisions. The study highlights the importance of extended follow-up due to the risk of late recurrence.
IV.
滑膜肉瘤（SS）被归类为侵袭性高级别软组织肉瘤，主要累及四肢。尽管其在四肢的发病率较高（高达80%），但诊断和治疗挑战依然存在。本研究旨在通过对四肢滑膜肉瘤进行全面分析，重点关注诊断准确性和治疗结果，以应对这些挑战。本研究的核心问题是：与滑膜肉瘤相关的诊断和治疗挑战有哪些？肿瘤体积和组织学亚型如何影响预后？免疫组织化学和基因标志物在滑膜肉瘤的诊断和管理中起什么作用？
我们假设滑膜肉瘤患者中，较大的肿瘤体积和较差的组织学分化与较差的生存结果相关。
这项回顾性研究分析了2005年至2020年在单一中心诊断为滑膜肉瘤的63例患者的数据。纳入具有完整病理、放射学和手术记录且随访至少12个月的患者。回顾了肿瘤特征、治疗方式和随访数据。
该研究纳入63例患者和65个肿瘤。平均年龄为38±17岁。女性31例（49%），男性32例（51%）。滑膜肉瘤主要累及四肢（n = 63，97%），尤其是下肢（n = 49，75%）。肿瘤体积平均为110±176 cm³，是一个重要因素，肿瘤体积>30 cm³与更高的转移率（p = 0.006）和更低的生存率（p = 0.027）相关。组织学上，25例（38%）为单相型，24例（37%）为双相型，16例（25%）为低分化型，低分化亚型的生存率较低。免疫组织化学显示Bcl-2（89%，17/19）、EMA（88%，52/59）和TLE1（87%，13/15）的阳性率较高。73%的病例（8/11）检测到SS18-SSX融合基因。27例（42%）患者发生转移，24例（37%）患者复发。15例（23%）患者死于该疾病。
滑膜肉瘤的准确诊断对于有效管理至关重要。临床医生在做出治疗决策时应了解阴性预测因素, 包括肿瘤体积>30 cm³和组织学分化差。该研究强调了由于晚期复发风险而进行延长随访的重要性。
IV级。
