目的

探讨腹膜后滑膜肉瘤的临床表现、诊断、病理特征、治疗及预后，提高外科医生对发生于腹膜后区域的滑膜肉瘤的认识。

方法

回顾性分析北京大学国际医院2015 年1 月～ 2024 年1 月收治的7 例腹膜后滑膜肉瘤患者的临床资料并进行随访。

结果

患者临床表现并不典型，多为腰痛（2 例）及腹痛（4 例）；CT 特征性表现为肿瘤呈分叶状或团块状软组织肿块，密度略低于肌肉密度，瘤体内可见坏死区；增强扫描呈均匀或不均匀强化，肿块边缘有时可见钙化。本组患者均行手术治疗，术中见瘤体常较大，多侵犯周围脏器。术后出现并发症2 例（胃瘫1，肠瘘1），围术期死亡1 例（呼吸衰竭）。7 例患者均存在SS18 基因重排。随访截至2024 年3 月，所有患者均死于该疾病，中位生存期56 月。

结论

滑膜肉瘤患者临床表现无特异性，特异性的染色体易位是诊断的金标准。手术切除仍是主要治疗手段；肿瘤恶性程度高，易复发，预后差。

Objective

To investigate the clinical manifestations, diagnosis, pathological features,treatment and prognosis of retroperitoneal synovial sarcoma, and to improve surgeons' understanding of retroperitoneal synovial sarcoma.

Methods

Retrospective analysis was made on the clinical data of 7 cases of retroperitioneal synovial sarcoma patients undergoing surgical resection in Peking University International Hospital from January 2015 to January 2024,and to do the follow-up visit.

Results

The clinical manifestations of the patients were not typical, most of them were low back pain (2 cases) and abdominal pain (4 cases).The characteristic findings of CT were that the tumor was lobed or lumpy soft tissue mass,the density was slightly lower than the muscle density, and necrosis area was visible in the tumor body.The enhanced scan showed uniform or uneven enhancement, and sometimes calcification was seen at the edge of the mass.All patients in this group underwent surgical treatment, and the tumors were often larger and invaded the surrounding organs.Postoperative complications occurred in 2 cases (gastroparesis 1, intestinal basket 1),and perioperative death occurred in 1 case (respiratory failure).SS18 gene rearrangement was present in all 7 patients.All patients were followed up until March 2024 and died of the disease,median survival was 56 months.

Conclusion

The clinical manifestations of patients with synovial sarcoma are nonspecific, and the characteristic chromosomal translocation is the gold standard for diagnosis.Surgical resection is still the main treatment.This kind of tumor has high malignant degree, easy recurrence after operation and poor prognosis.