腹腔促结缔组织增生性小圆细胞肿瘤一例及文献复习

A case report and literature review of Abdominal Desmoplastic Small Round Cell Tumor

目的探讨腹腔促结缔组织增生性小圆细胞肿瘤的病理特征、诊断、治疗决策及近期疗效。方法回顾性分析2018年4月入住甘肃省人民医院的一例促结缔组织增生性小圆细胞肿瘤患者的临床资料。结合文献对促结缔组织增生性小圆细胞肿瘤的临床表现、病理特点、诊断与鉴别诊断、治疗与预后进行总结和分析。结果患者成功行最大限度肿瘤减灭术,手术时间360min,术中估计失血量200ml,无输血。术后第8天拔除腹腔引流管,肝功能恢复良好。术后病理:(腹膜后)小圆细胞恶性肿瘤。结合临床及免疫组织化学染色结果:高度考虑:促结缔组织增生性小圆细胞肿瘤。术后第16天出院。随访4月,术区肿瘤复发,肝脏转移。随访时间目前截至2018年10月。结论促结缔组织增生性小圆细胞肿瘤是一种罕见,且预后极差的高度恶性软组织小细胞肿瘤,影像学检查及肿瘤标志物检测无特异性,诊断困难,最大限度肿瘤细胞减灭术和联合化疗为主要治疗方案,能改善患者预后,但预后不理想,仍需探究更有效的治疗决策。

Objective To investigate the pathological characteristics,diagnosis,treatment decision and short-term curative effect of abdominal desmoplastic small round cell tumor.Methods The clinical data of a case of desmoplastic small round cell tumor admitted to Gansu provincial people′s Hospital in April 2018 were analyzed retrospectively.The clinical manifestations,pathological features,diagnosis and differential diagnosis,treatment and prognosis of desmoplastic small round cell tumor were summarized and analyzed.Results The patient was successfully treated with maximum tumor reduction.The operation time was 360 minutes.The estimated blood loss during operation was 200 ml,and no blood was transfused during operation.The abdominal drainage tube was removed on the 8th day after operation and the liver function recovered well.Postoperative pathology:(retroperitoneal)small round cell malignant tumor.Combined with clinical and immunohistochemical staining results:highly considered:desmoplastic small round cell tumor.The patient was discharged on the 16th day after operation.The patient was followed up for 4 months and the tumor recurred and liver metastasis.The follow-up period is now up to October 2018.Conclusion Desmoplastic small round cell tumor is a rare and highly malignant soft tissue small cell tumor with poor prognosis.Imaging examination and detection of tumor markers have no specificity and diagnose of it is difficult.Complete resection of the tumor and combined chemotherapy can improve the prognosis of the patients,but the prognosis is still not satisfactory,and more effective treatment decisions still need to be explored.