摘要
目的:探讨小儿肝脏未分化胚胎性肉瘤(UES)的临床表现、诊断与治疗的特点。方法:回顾性分析1995-01-2008-10经手术病理确诊的14例肝脏UES患儿的临床表现、影像学、实验室检查、手术、化疗及生存情况。结果:14例中男女各7例,年龄2～11岁,平均年龄6.6岁。主要症状表现为腹部包块13例,伴有腹痛者5例。反复腹痛1例。B超提示实性为主包块10例,CT显示以囊性为主病灶12例。11例手术完整切除肿瘤者生存6例,2例部分切除及1例活检者均死亡。多药化疗8例生存6例,生存率75%(6/8);三药化疗3例及3例拒绝化疗者均死亡;术后分期Ⅰ、Ⅱ期者生存率60%(6/10),Ⅲ、Ⅳ者均死亡。随访9个月～9年8个月,8例死亡(死亡时间术后2～30个月),1例死于化疗后心功能衰竭,7例死于肿瘤复发。结论:影像学B超与CT的不一致性是本病的重要特点。手术完整切除,同时辅以多药化疗联合治疗可明显提高患儿生存率。
OBJECTIVE： To discuss the clinical manifestations, diagnosis and treatment of undifferentiated embryonal sarcoma（UES） of the liver（UESL） in children. METHODS： A retrospective analysis of the clinical presentation, imaging findings, laboratory data, operation, chemotherapy and survival features in 14 cases of UES of the liver from Jan 1995 to Oct 2008 was performed. RESULTS： Fourteen patients（7 boys and 7 girls） with UESL age ranging from 2 to 11 years （median 6.6 years） were identified. The main symptoms were large abdominal mass（13 patients） accompanied by abdominal pain in 5 cases. One patient developed intermittent abdominal pain. Uhrasonography（US） revealed predominant solid lesion in 10 cases. CT demonstrated predominant cystic masses in 12 cases. Among the eleven patients who underwent complete tumor resection, there were six patients still alive. Two patients underwent partial tumor resection and one underwent biopsy all died. Eight patients received multiagent chemotherapy, there were six patients still alive, the survival rate was 75% （6/8）. Three patients received chemotherapy with 3 kind of drugs and three patients refused chemotherapy all died. About 60%（6/10） patients with UES of the liver in staging Ⅰand Ⅱ survived. Patients with UES of the liver in staging Ⅲ and Ⅳ all died after operation, Follow-up of 9 months to 9 years and 8 months showed 6 patients using multiagent chemotherapy alive. Eight patients suffered tumor recurrence from 2 to 30 months after operation and all of them died. Another patient died of heart failure related to chemotherapy. CONCLUSION ： Discrepancy of imaging between US and CT is one of the important characteristics in UES of liver. Complete tumor resection and multiagent adjuvant chemotherapy can improve the survival rate significantly.
出处
《中华肿瘤防治杂志》
CAS
2009年第14期1108-1110,共3页
Chinese Journal of Cancer Prevention and Treatment
关键词
肝肿瘤/诊断
肝肿瘤/治疗
肉瘤
liver neoplasms/diagnosis
liver neoplasms/therapy sarcoma
