10例嗅神经母细胞瘤临床疗效分析

Clinical Efficacy Analysis of 10 Cases of Olfactory Neuroblastoma

目的回顾性研究不同治疗方案对嗅神经母细胞瘤患者预后的影响。方法回顾性分析四川省肿瘤医院医院2008年至2020年确诊嗅神经母细胞瘤患者的病例资料。结果总共纳入10例患者,中位随访时间为48个月(7~132个月)。Kadish分期为B、C及D期的患者占总数的20%、70%及10%。病理Hyams分级为1、2、3及4的患者占总数的10%、20%、40%及30%。3例出现颅内转移,1例出现区域淋巴结转移,所有患者均行手术治疗(内镜或颅面切除术)。初始治疗采用单纯手术的2例患者均出现局部复发,复发后均接受二次手术及术后放化疗。2例初始治疗为手术联合放疗,其中1例复发2次,进行再次手术及术后放化疗,至今存活。其余6例患者术后均行术后放化疗,无一例复发。观察到的5年无病生存率为61.71%。无病生存时间为33.4个月,平均总生存时间为53.2个月。与单纯术后患者相比,术后放化疗患者无病生存时间明显延长,两组比较差异有统计学意义(P=0.0018)。结论对于嗅神经母细胞瘤患者来说,术后辅助放化疗是一种有效的控制肿瘤复发的方法,显著延长无病生存时间。本文回顾真实世界中此类罕见疾病不同治疗方案对预后的影响及治疗前沿进行讨论。

Objective By exposing results observed with apparition of new therapeutic options as adjuvant chemotherapy,the objective is to evaluate a series and a review of the current literature.Methods A retrospective review was conducted including patients diagnosed and followed-up for ONB from 2008 to 2020 in Sichuan Tumor Hospital.Results 10 patients were included.Median follow-up was 48 months(from 7 to 132 months).The Kadish proportion of B,C,and D stage was 20%,70%,10%respectively.Hyams Grade 1,2,or 3,4 was 10%,20%,and 40%,30%,respectively.Intracranial metastasis occurred in 3 patients and regional lymph node metastasis occurred in 1 patient.All patients underwent surgical treatment(endoscopic or craniofacial resection).Local recurrence occurred in the 2 patients who were initially treated with surgery alone,and they received secondary surgery and postoperative chemoradiotherapy after recurrence.The initial treatment of 2 patients was surgery combined with radiotherapy,of whom 1 patient relapsed twice,underwent reoperation and postoperative radiotherapy and chemotherapy,and has survived to date.The other 6 patients received postoperative radiotherapy and chemotherapy,and no recurrence was found.The observed 5-year disease-free survival rate was 61.71%.Disease-free survival was 33.4 months and the mean overall survival was 53.2 months.Compared with the postoperative patients alone,the postoperative radiotherapy and chemotherapy patients had a distinct prolonged recurrence time without disease,and the difference between the two groups was statistically significant(P=0.0018).Conclusion For patients with olfactory neuroblastoma,postoperative adjuvant chemoradiotherapy is an effective method to control tumor recurrence and can significantly prolong disease-free survival time.