新生儿神经母细胞瘤：发病特点、治疗刍议以及临床转归

Neonatal neuroblastoma： the controversy of treatment, clinical features and outcome

目的总结新生儿神经母细胞瘤的发病特点、随访结果，针对治疗方案提出本中心的观点。方法对2003－2009年我院收治的14例新生儿神经母细胞瘤患儿进行回顾性分析，14例新生儿神经母细胞瘤中，男8例，女6例。发病年龄最大29d，最小2d，平均（19±3．5）d，中位数16d。Ⅰ期5例，Ⅱ期1例，Ⅲ期1例，Ⅳ期1例，4S期6例。腹膜后占位8例，纵隔4例，盆腔2例。分析每个患儿的发病特点，临床分期、生物学特征、治疗方案、及其与预后的关系。结果8例患儿接受手术切除，其中6例完整切除，2例部分切除后辅以化疗。4例患儿活检后化疗，2例患儿单纯行经验性化疗，未手术／活检。所有患儿中只有4例患儿出现尿VMA的增高（28．6％），Mycn扩增3例，不扩增9例，UH：FH为4：8。两年的总体生存率（OS）是92．9％，局限性神经母细胞瘤患儿的生存率是100％。单纯手术组的生存率（7／8，87．5％），化疗组生存率（5／6，83．3％）（P＝0．065）；Mycn扩增组的生存率为50％，不扩增组的生存率为100％（P=0．021）；VMA阳性生存率为50％，阴性者生存率为100％（P=0．021）。死亡2例，其中1例死于化疗后的并发症，另1例死于肿瘤的复发。结论N-myc不扩增和VMA不增高的新生儿神经母细胞瘤的预后很好，单纯手术组和化疗组生存率没有差别。手术、化疗以及其他的相关的并发症要比疾病本身所致的死亡例数要多，手术、化疗应仔细考量。手术治疗提倡改变既往观念，建议短期内密切观察和随访，尽量避免在新生儿期手术。

Objective To review the clinical features and outcome of newborns with neuroblas- toma. Methods A medical record review and retrospective data analysis were performed on the neo- natal neuroblastoma between January 2001 and December 2009, including clinical features, stages, bionomics, treatment and outcome. Results There were 14 patients with 8 males and 6 females. There were 5 cases in stage Ⅰ , 1 in stage Ⅱ, 1 in stage Ⅲ, 1 in stage Ⅳ and 6 in stage IVs, respectively. Tumor site distribution were retroperitoneum in 8 cases （6 was in adrenal gland site of the 8）, thoracic cavity in 8 and pelvis in 2. N-myc amplification was detected in 3 out of 12 available cases. According to the Shimada pathology classification, the ratio of unfavorable histology （UH） to favorable histology （FH） was 4 to 8. Eight patients received surgical excision, in which 6 were completely resected. The 2-year overall survival （OS） of neonatal neuroblastoma patients was 92. 9%. Two-year OS of the simple chemotherapy group was not as higher as that of no chemotherapy group. Two-year OS of N-myc amplification group was lower than that of no amplification group（P = 0. 021 ）. One patient died of complication of chemotherapy. Conclusions The prognosis of NB in neonates is fairly good except the patients with N-myc amplification or elevated vanillymandelic（VMA）. There was no difference in the the five years of OS rate between simple chemotherapy and simple operation. Newborns with neuroblastoma were more sensitive and vulnerable to chemotherapy, operation and other therapeutic modalities.