【摘要】 目的提高对腹部神经母细胞瘤临床特征及预后的认识。方法回顾性分析资料完整的84例腹部神经母细胞瘤患者临床表现、肿瘤标志物、病理类型、治疗及转归。结果男性44例,女性40例,中位年龄34.5月,肿瘤原发于肾上腺42例,腹膜后39例,盆腔3例。初诊时常见的表现是发热和(或)腿痛,腹痛、腹胀,包块。肿瘤临床分期:Ⅰ期1例(1.2%)、Ⅱ期6例(7.1%)、Ⅲ期16例(19.0%)、Ⅳ期55例(65.5%)、Ⅳs期6例(7.1%)。血清NSE>100 ng/L者76.2%,明显高于纵隔和颈部神经母细胞瘤(18.8%,P<0.001),原发肿瘤完整切除率达63.0%。3年总生存率为63.0%。初诊年龄>18月(P=0.007)、Ⅲ和Ⅳ期(P=0.027)、血清NSE>100 ng/L(P<0.001)及手术中未完整切除(P=0.007)是预后不良的指标。结论腹部神经母细胞瘤发病年龄偏大,初诊时有临床表现,但无特异性;血清NSE>100 ng/L者多见;完整切除原发肿瘤有利于改善预后。更多还原

【Abstract】 Objective To analyze the clinical features of abdominal neuroblastoma.Methods The clinical features of 84 patients with abdominal neuroblastoma,including manifestations,tumor markers,pathology,treatments and outcomes,were retrospectively analyzed.Results Of the 84 cases,44 were males and 40 were females with a median age of 34.5 months.The location of primary tumors was adrenal gland in 42 cases,retroperitoneal region in 39 cases and pelvic cavity in 3 cases.The manifestations at first visit included fever and/or leg pain,abdominal pain,abdominal distention and mass.According to International Neuroblastoma Stage System,there were 1 case in stage I(1.2%),6 stage Ⅱ(7.1%),16 stage Ⅲ(19.0%),55 stage Ⅳ(65.5%)and 6 stage Ⅳs(7.1%).The percentage of the prevalence of serum neuron-specific enolase(NSE) >100 ng/ L was 76.2% in patients with abdominal neuroblastoma,while 18.8% in patients with mediastinum or cervical neuroblastoma(P<0.001).Gross total resection rate was 63.0% and the 3-year overall survival rate was 63.0%.Poor prognostic factors included age >18 months at first visit(P=0.007),stage Ⅲ and Ⅳ(P=0.027),serum NSE>100 ng/L(P<0.001) and extent of resection(P=0.007).Conclusion The age of disease onset was older in patients with abdominal neuroblastoma than those with mediastinum and cervical neoroblastoma.The manifestations are not specific and gross total resection of primary tumors may improve the prognosis of the disease.更多还原