肝胚胎性肉瘤的临床病例分析

  目的  分析肝脏未分化(胚胎性)肉瘤(UESL)的临床表现、影像特点以及治疗方案, 为诊断和治疗提供依据。  方法  报道广西医科大学附属肿瘤医院收治的2例UESL, 并检索国内近25年间文献报道的UESL, 共120例, 提取病例资料进行统计分析。  结果  UESL病例, 多表现为腹部包块, HBsAg和AFP阳性率低(17.72%和8.64%), 肿瘤平均直径为(13.82±4.90)em。72.34%的病例超声表现以实质性高回声为主, 91.78%的病例CT以囊性低密度为主要表现。单纯手术切除组的术后复发率高于手术联合化疗组(为72.97%和30.77%), 但两组的复发时间和生存曲线无明显差别。总体中位生存期为11(5.4~16.6)个月。  结论  UESL具有独特的影像学特征, 早期诊断及早期手术切除是治疗UESL的关键, 术后联合化疗有望降低术后复发风险, 延长生存时间。 

Clinical Analysis of Undifferentiated (Embryonal) Sarcoma of the Liver

  Objective  To evaluate the clinical manifestation, imaging characteristics, and treatment plans of undifferentiated embryonal sarcoma of the liver (UESL) to provide bases for the diagnosis and treatment.  Methods  Data of two UESL cases were obtained from The Affiliated Tumor Hospital of Guangxi Medical University, Naning, China. Information on relevant cases was retrieved from the CNKI, Wanfang, and VIP databases between January 1988 and June 2011. Clinical data of all the cases were analyzed.  Results  A total of 120 UESL cases previously reported in China were included in this study. Most of the cases presented with an abdominal mass, with a low positive rate of hepatitis B surface antigen and alpha fetoprotein (17.72 % and 8.64 %). The average diameter of the tumors was 13.82 cm-4.90 cm. Imageological diagnosis revealed that 72.34 % of the UESL cases showed a solid hyperechogenieity in ultrasound, whereas 91.78 % of the cases demonstrated a cystic low density on CT scan. The recurrence rate was higher in the group with surgery than in the group with surgery plus chemotherapy (72.97 % and 30.77 %). However, no significant differences in recurrence time and survival curves were found between the two groups. The overall median survival time (95 % confidence interval) was 11 (5.4±16.6) months.  Conclusion  Early diagnosis and prompt excision play an important role in the treatment and prognosis of UESL. Furthermore, postoperative adjuvant chemotherapy can reduce the risk of tumor recurrence and prolong the survival time for UESL patients.