腹腔促结缔组织增生性小圆细胞肿瘤肝转移1例MDT讨论并文献回顾

Intra-abdominal desmoplastic small round cell tumor with liver metastasis:MDT of a case and literatures review

目的探讨促结缔组织增生性小圆细胞肿瘤(desmoplastic small round cell tumor,DSRCT)的临床病理学特点及其治疗方法。方法回顾性总结四川大学华西医院肝脏外科于2017年10月收治的1例多发肝转移DSRCT患者的MDT诊治过程,并结合文献回顾,总结其临床病理学特点及其治疗方法。结果该患者一般情况良好,经影像科、肿瘤科和肝脏外科医师多学科讨论后,认为有手术指征,同患者家属充分沟通后,积极选择手术治疗,手术顺利,术后患者行辅助化疗。手术时间5 h,术中出血量约600 mL。术后无出血、胆汁漏、肠瘘等并发症发生,于术后8 d出院。术后该患者已获访10个月,未见肿瘤复发转移征象,继续随访。文献回顾结果显示,DSRCT的好发人群为年轻男性患者,平均年龄为12～27岁,中位生存期最长为39.2个月,3年生存率为20.8%～71%;手术比例高的研究,患者的中位生存期相对较长。结论 DSRCT是一种罕见的侵袭性软组织肉瘤,发现时大多已发生多器官转移,治疗主要是以手术切除为基础的联合放化疗的多模式治疗,但整体预后较差。

Objective To explore the clinicopathologic features and treatment of desmoplastic small round cell tumor（DSRCT）. Methods By summarizing the diagnosis and treatment of a DSRCT patient with liver metastasis, who was admitted to Department of Liver Surgery in West China Hospital in October 2017, and exploring its clinicopathologic features and treatment by reviewing literatures. Results This patient was generally in good condition, after the multidisciplinary discussion between the imaging physician, the oncologist, and the liver surgeon, it was considered that there were indications of operation, and after communicating with the patient＇ families, actively chose surgical treatment. The performance was successful, and this patient was treated with adjuvant chemotherapy postoperatively.The operative time for this patient was 5 hours, and blood loss was 600 mL. There was no complication occurred, such as bleeding, bile leakage, and intestinal fistula, and discharged on 8 days after surgery. This patient was followed up for10 months, without tumor recurrence and metastasis occurred. The results of literatures showed that, DSRCT was more common in young male population, the mean age was 12–27 years old, the longest median survival time was 39.2 months, and 3-year survival rate was 20.8%–71%. Those patients who received surgery, had longer median survival time. Conclusions DSRCT is a rare aggressive soft tissue sarcoma, which is usually diagnosed with multiple organ metastases. The treatment is mainly multi-mode treatment based on surgical resection combined with radiotherapy and chemotherapy, but the overall prognosis is poor.