肝脏未分化胚胎性肉瘤的临床诊治

Clinical diagnosis and treatment of undifferentiated embryonal sarcoma of liver

目的:探讨肝脏未分化胚胎性肉瘤的临床特征、治疗方案及预后。方法:回顾性分析17例肝脏未分化胚胎性肉瘤患者的临床资料,总结临床诊治经验。结果:17例患者中,男性10例、女性7例,年龄11~66岁(中位年龄47岁)。临床主要表现为腹部包块,可伴有腹痛、腹胀;诊断时肿瘤直径中位数为13cm(6.5~40cm)。15例患者接受外科手术治疗,其中8例接受单纯外科手术治疗,1例接受外科手术+经导管肝动脉化疗栓塞术(TACE),2例接受外科手术+化疗,4例接受外科手术+TACE+化疗;另外2例仅接受TACE。17例患者中位随访时间为11个月(1~60个月),2例失访,1例复发。完成随访的患者中,1年生存率为42.9%(6/14),2年生存率为28.6%(4/14)。6例术后联合化疗的患者中,1例至随访7个月时未复发,3例获得疾病缓解,2例出现疾病进展;6例患者均出现4级骨髓抑制。结论:肝脏未分化胚胎性肉瘤较罕见,病灶大,术后复发转移率高,治疗以外科手术为主,联合介入、化疗可提高患者生存率,但预后仍较差。

Objective: To explore the clinical features, treatment, and prognosis of undifferentiated embryonal sarcoma of liver. Methods: The clinical data of 17 patients with undifferentiated embryonal sarcoma of liver were collected and retrospectively analyzed. Results: Among the 17 patients, there were 10 males and 7 females, aged from 11 to 66 years (median age was 47 years). The main clinical manifestations were abdominal mass, accompanied by abdominal pain and distension. The median diameter of the tumors was 13 cm (6.5-40 cm). Among the 17 patients, 15 patients underwent surgical operation, among whom 8 patients were treated by operation alone, 1 patient was treated by operation plus transcatheter arterial chemoembolization (TACE), 2 patients were treated by operation plus chemotherapy, 4 patients were treated by operation plus chemotherapy, and another 2 patients were treated by TACE alone. The median follow-up time was 11 months (1-60 months) for the 17 patients, 2 patients were lost, and 1 patient had recurrence. The overall 1-year survival rate was 42.9%(6/14) and the 2-year survival rate was 28.6%(4/14). Among the 6 patients who received combined chemotherapy after operation, 1 patient had no relapse during the 7 months of follow-up, 3 patients had partial remission, 2 patients had disease progression, and all the 6 patients had grade 4 bone marrow depression. Conclusions: Undifferentiated embryonal sarcoma of liver is a rare tumor, with a large intrahepatic lesion and high recurrence and metastasis rates. The combined treatment of operation, interventional chemoembolization, and chemotherapy may improve the overall survival, but the prognosis is still relatively poor.