Castel Victoria, Cañete Adela
Pediatric Oncology Unit, Hospital Infantil Universitario La Fe, Avda Campanar 21, 46009 Valencia, Spain.
Expert Opin Pharmacother. 2004 Jan;5(1):71-80. doi: 10.1517/14656566.5.1.71.
Neuroblastoma is the most common solid tumour in childhood. Modern management includes a biopsy to perform genetic studies. Based on clinical data and Myc-N amplification (MNA), patients are divided in three prognostic groups: the low-risk (Stage 1, 2, 4S without MNA) has an event-free survival (EFS) of > 90% with surgery alone; the intermediate-risk (Stage 3, > 1 year of age, without MNA and Stage 3 and 4 infants without MNA) has an EFS of approximately 80% with mild chemotherapy and surgery; the high-risk group includes Stage 4, > 1 year of age and any stage and age with MNA. These patients are treated with chemotherapy, surgery, megatherapy, irradiation and 13-cis-retinoic acid. With this complex therapy, a 5-year EFS of 30-50% can be obtained.
神经母细胞瘤是儿童期最常见的实体瘤。现代治疗方法包括进行活检以开展基因研究。根据临床数据和Myc-N扩增(MNA)情况,患者被分为三个预后组:低危组(1期、2期、无MNA的4S期)单纯手术治疗的无事件生存率(EFS)>90%;中危组(3期、年龄>1岁且无MNA,以及3期和4期婴儿且无MNA)经轻度化疗和手术治疗后的EFS约为80%;高危组包括4期、年龄>1岁以及任何分期和年龄且伴有MNA的患者。这些患者接受化疗、手术、大剂量疗法、放疗和13-顺式维甲酸治疗。采用这种综合治疗方法,5年EFS可达30%-50%。