晚期儿童神经母细胞瘤的临床和预后分析

Clinical features and prognosis of advanced neuroblastoma in children

目的探讨晚期儿童神经母细胞瘤的临床特点、治疗策略和预后。方法对63例确诊为III～IV期的神经母细胞瘤患者的临床资料进行回顾性分析,有60例患者接受了手术切除和(或)化疗和(或)局部放疗,其中14例患者还接受了自体外周血造血干细胞移植治疗。结果63例患者中,男女比例为2.7∶1,中位年龄4岁;常见首发症状为发热、腹痛、腹部肿块、腿痛或关节疼痛;常见原发部位为肾上腺(38%)、腹膜后(35%)、后纵隔(17%)、盆腔(6%)和颈部(2%);确诊时常见转移部位为局部(41%)和(或)远处(37%)淋巴结、骨髓(60%)、骨(46%)、肝脏(16%)。中位生存时间32.7个月,2年生存率44.3%。年龄>1岁(P<0.05)、血清神经元特异性烯醇化酶>100mg/L(P<0.05)、血清乳酸脱氢酶>1500U/L(P<0.01)、血清铁蛋白>150mg/L(P<0.05)是预后不良指标。完整切除原发肿瘤可延长患者总体生存时间(P<0.05);强烈化疗联合自体外周血造血干细胞移植的综合治疗可延长患者总体生存时间(P<0.01)。结论晚期儿童神经母细胞瘤临床表现多样,预后差,熟悉其临床和实验室检查特点尽早明确诊断、完整切除原发肿瘤、进行自体外周血造血干细胞移植支持下的强烈化疗的综合治疗有利于改善预后。

Objective To investigate the clinical features, treatment modalities and the prognosis of advanced neuroblastoma in children. Methods The medical records of 63 children with stage III or IV neuroblastoma from January 1996 to December 2005 were retrospectively reviewed. Sixty patients were treated by tumor resection and （or） chemotherapy and （or） radiation. Fourteen out of the 60 patients received another autologous peripheral blood stem cell transplantation. Results Of the 63 patients with advanced neuroblastoma, the male/female ratio was 2.7：1 and the median age at diagnosis was 4 years old. Most of the initial symptoms included pyrexia, abdominal pain, abdominal mass, and leg or articular pain. Primary tumor sites were adrenal （38%）, retroperitoneal （35%）, mediastinal （17%）, pelvic （6%） and cervical （2%）. The sites of metastasis at diagnosis included local （41%） and （or） distant （37%） lymph nodes, bone marrow （60%）, bone （46%） and liver （ 16% ）. The median survival time of the 63 patients was 32.7 months. The 2-year survival rate was 44.3%. Statistical analysis demonstrated that unfavorable survival prognostic factors were the following： age 〉 1 year at diagnosis （P 〈0.05） ; serum neuro-specific enolase 〉 100 mg/L （P 〈0.05） ; serum lactic dehydrogenase 〉 1 500 U/L （P 〈0. 01 ） ; serum ferritin 〉 150 mg/L （P 〈0.05）. The overall survival period of the patients was prolonged through total resection of the primary tumor （ P 〈 0. 05 ）. Intensive chemotherapy in combination with autologous peripheral blood stem cell transplantation could also result in a prolonged overall survival period （ P 〈 0.01 ）. Conclusions Neuroblastoma with advanced stages often presents with various clinical manifestations and has a poor prognosis. It is beneficial to improve the prognosis of neuroblastoma through an early diagnosis and a comprehensive therapy including total resection of the primary tumor, autologous peripheral blood stem cell transplantation and intensive chemotherapy.