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目的:探讨原发性腹膜后滑膜肉瘤的临床表现、诊断、病理特点及治疗,提高对该疾病的认识。方法:回顾性分析我院1例原发性腹膜后滑膜肉瘤合并妊娠患者的临床资料及随访结果,并结合国内外文献进行分析。结果:本例患者彩超发现腹膜后巨大混杂回声团块时合并妊娠7个月,3个月后剖宫产分娩一健康女婴后予以手术,术前MRI示长T1长T2混杂信号影,大小约16cm×15cm,予行根治性切除术。病理肉眼观示肾脏包膜完整,肾脏及肾上腺被肿瘤包绕,镜下所见为梭形细胞瘤样增生伴黏液变性,免疫组化示WT1、CD56、CD57、CD99、Bc12阳性,分子病理检测到t(18q;v)SYT易位遗传学异常,诊断为原发性腹膜后滑膜肉瘤。术后行TOMO刀放疗及MAID方案化疗,每3个月复查CT,术后随访18个月,尚未见明确复发。结论:原发性腹膜后滑膜肉瘤是非常罕见的、高度恶性的肿瘤,具有恶性程度高,进展迅速、容易复发转移,预后差,生存期短的临床特点。早期诊断及根治性切除并结合放化疗能在一定程度上延长患者生存期。
Objective: To investigate the clinical manifestations, diagnosis, pathological features and treatment of primary retroperitoneal synovial sarcoma and to raise awareness of the disease. Methods: The clinical data and follow-up results of one patient with primary retroperitoneal synovial sarcoma in our hospital were analyzed retrospectively. The results were analyzed with literature at home and abroad. Results: In our study, we found that there was 7 months pregnant with large mixed echogenic mass in the retroperitoneum and 3 months after cesarean delivery. About 16cm × 15cm, radical resection. Pathological macroscopic renal capsule integrity, kidney and adrenal surrounded by tumor, microscopic view of spindle-like tumor-like hyperplasia with mucinous degeneration, immunohistochemistry showed WT1, CD56, CD57, CD99, Bc12 positive, molecular pathology To t (18q; v) SYT translocation genetic abnormalities, the diagnosis of primary retroperitoneal synovial sarcoma. Postoperative TOMO knife radiotherapy and MAID regimen chemotherapy, CT review every 3 months, postoperative follow-up 18 months, no clear recurrence. Conclusion: Primary retroperitoneal synovial sarcoma is a very rare and highly malignant tumor with the characteristics of high malignant degree, rapid progression, recurrence and metastasis, poor prognosis and short survival. Early diagnosis and radical resection combined with radiotherapy and chemotherapy can extend the survival of patients to some extent.