Gao Xiao-Ning, Tang Suo-Qin, Lin Ji
Department of Pediatrics, General Hospital of People's Liberation Army, Beijing 100853, China.
Zhongguo Dang Dai Er Ke Za Zhi. 2007 Aug;9(4):351-4.
To investigate the clinical features, treatment modalities and the prognosis of advanced neuroblastoma in children.
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.
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 > 1500 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).
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.
探讨儿童晚期神经母细胞瘤的临床特征、治疗方式及预后。
回顾性分析1996年1月至2005年12月期间63例Ⅲ期或Ⅳ期神经母细胞瘤患儿的病历资料。60例患儿接受了肿瘤切除和(或)化疗和(或)放疗。60例患儿中有14例接受了另一次自体外周血干细胞移植。
63例晚期神经母细胞瘤患儿中,男女比例为2.7∶1,诊断时的中位年龄为4岁。多数首发症状包括发热、腹痛、腹部肿块以及腿部或关节疼痛。原发肿瘤部位为肾上腺(38%)、腹膜后(35%)、纵隔(17%)、盆腔(6%)和颈部(2%)。诊断时的转移部位包括局部(41%)和(或)远处(37%)淋巴结、骨髓(60%)、骨(46%)和肝(16%)。63例患儿的中位生存时间为32.7个月。2年生存率为44.3%。统计分析表明,不良生存预后因素如下:诊断时年龄>1岁(P<0.05);血清神经特异性烯醇化酶>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)。通过原发肿瘤的全切除可延长患儿的总生存期(P<0.05)。强化化疗联合自体外周血干细胞移植也可延长总生存期(P<0.01)。
晚期神经母细胞瘤常表现出多种临床表现,预后较差。早期诊断以及包括原发肿瘤全切除、自体外周血干细胞移植和强化化疗在内的综合治疗有利于改善神经母细胞瘤的预后。