神经母细胞瘤的外科治疗：一家机构23年的经验

Surgical treatment of neuroblastoma: twenty-three years of experience at a single institution.

作者信息

Koivusalo A I, Pakarinen M P, Rintala R J, Saarinen-Pihkala U M

机构信息

Children's Hospital, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland,

出版信息

Surg Today. 2014 Mar;44(3):517-25. doi: 10.1007/s00595-013-0576-7. Epub 2013 Apr 12.

DOI:10.1007/s00595-013-0576-7

PMID:23580079

Abstract

PURPOSE

Neuroblastoma (NB) is treated with surgery, chemotherapy and radiotherapy. We assessed the effects of surgical resection on the outcome over a 23-year period at our institution.

METHODS

85 children were included with a median age at diagnosis of 2.0 (range 0.1-15) years. We assessed the correlation of the complete surgical resection (CR) rate, metastases, NMYC amplification (NMYCA) and chemotherapeutic response with the 5-year overall survival (OS).

RESULTS

The INSS stage of NB was 1 in 11 (13 %) patients, 2 in 10 (11 %), 3 in 13 (17 %), 4 in 46 (53 %) and 4S in five patients (6 %). Fifty-two (61 %) patients had high-risk NB and 22 (26 %) had NMYCA. The resection was complete in 72 (85 %) patients, incomplete (ICR) in seven (8 %) and six (7 %) patients did not undergo surgery. Fifty-five patients were administered neoadjuvant and 61 were administered adjuvant chemotherapy (high-dose, n = 50). The OS (5 year) was 68 %: stage 1 (100 %), 2 (90 %), 3 (77 %), 4 (52 %), 4S (80 %) and high-risk NB (52 %). The OS in high-risk NB patients was correlated with a good chemotherapeutic response of the primary tumour, with a RR for mortality = 0.3 (95 % CI 0.1-0.7; p = 0.01), but not with the CR, which had an RR = 0.9 (95 % CI 0.3-2.4; p = 0.84).

CONCLUSIONS

The OS in high-risk NB patients was related to a good histological chemotherapeutic response, but not with complete excision of the primary tumour.

摘要

目的

神经母细胞瘤（NB）采用手术、化疗和放疗进行治疗。我们评估了在我院23年期间手术切除对预后的影响。

方法

纳入85例儿童，诊断时的中位年龄为2.0岁（范围0.1 - 15岁）。我们评估了完整手术切除（CR）率、转移、NMYC扩增（NMYCA）和化疗反应与5年总生存率（OS）的相关性。

结果

NB的国际神经母细胞瘤分期系统（INSS）分期为1期的患者有11例（13%），2期10例（11%），3期13例（17%），4期46例（53%），4S期5例（6%）。52例（61%）患者为高危NB，22例（26%）有NMYCA。72例（85%）患者手术切除完整，7例（8%）不完全切除（ICR），6例（7%）患者未接受手术。55例患者接受了新辅助化疗，61例接受了辅助化疗（高剂量，n = 50）。5年总生存率为68%：1期（100%），2期（90%），3期（77%），4期（52%），4S期（80%），高危NB（52%）。高危NB患者的总生存率与原发肿瘤良好的化疗反应相关，死亡相对风险（RR）= 0.3（95%置信区间0.1 - 0.7；p = 0.01），但与CR无关，CR的RR = 0.9（95%置信区间0.3 - 2.4；p = 0.84）。