论文部分内容阅读
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。
Objective To improve the understanding of the clinical features and prognosis of abdominal neuroblastoma. Methods The clinical manifestations, tumor markers, pathological types, treatment and prognosis of 84 patients with complete neuroblastoma were analyzed retrospectively. Results There were 44 males and 40 females, with a median age of 34.5 months. The tumors were found in 42 cases of adrenal gland, 39 cases of retroperitoneum and 3 cases of pelvic cavity. Common manifestations of first visit is fever and (or) leg pain, abdominal pain, bloating, mass. The clinical stage of the tumor was as follows: stage Ⅰ in 1 case (1.2%), stage Ⅱ in 6 cases (7.1%), stage Ⅲ in 16 cases (19.0%), stage Ⅳ in 55 cases (65.5%) and stage Ⅳs in 6 cases (7.1%). Serum NSE> 100 ng / L was 76.2%, significantly higher than mediastinal and cervical neuroblastoma (18.8%, P <0.001), the complete resection rate of primary tumor was 63.0%. The 3-year overall survival rate was 63.0%. The poor prognosis was observed in patients with initial diagnosis> 18 months (P = 0.007), stage III and IV (P = 0.027), serum NSE> 100 ng / L (P <0.001) and incomplete surgical resection (P = 0.007) Conclusions The age of onset of neuroblastoma in the abdomen is relatively large. There is clinical manifestations at first visit, but it is nonspecific. Serum NSE> 100 ng / L is more common. Complete resection of the primary tumor is helpful to improve the prognosis.