Objective: To evaluate the efficacy of neoadjuvant chemotherapy (NACT) in the treatment of locally advanced olfactory neuroblastoma (ONB), and to explore the factors related to the efficacy of NACT. Methods: A total of 25 patients with ONB who underwent NACT in Beijing TongRen Hospital from April 2017 to July 2022 were retrospectively analyzed. There were 16 males and 9 females, with an average age of 44.9 years (ranged 26-72 years). There were 22 cases of Kadish stage C and 3 cases of stage D. After multiple disciplinary team(MDT) discussion, all patients were treated sequentially with NACT-surgery-radiotherapy. Among them, 17 cases were treated with taxol, cis-platinum and etoposide (TEP), 4 cases with taxol, nedaplatin and ifosfamide (TPI), 3 cases with TP, while 1 case with EP. SPSS 25.0 software was used for statistical analysis, and survival analyses were calculated based on the Kaplan-Meier method. Results: The overall response rate of NACT was 32% (8/25). Subsequently, 21 patients underwent extended endoscopic surgery and 4 patients underwent combined cranial-nasal approach. Three patients with stage D disease underwent cervical lymph node dissection. All patients received postoperative radiotherapy. The mean follow-up time was 44.2 months (ranged 6-67 months). The 5-year overall survival rate was 100.0%, and the 5-year disease-free survival rates was 94.4%. Before NACT, Ki-67 index was 60% (50%, 90%), while Ki-67 index was 20% (3%, 30%) after chemotherapy [M (Q1, Q3)]. The change of Ki-67 before and after NACT was statistically significant (Z=-24.24, P<0.05). The effects of age, gender, history of surgery, Hyams grade, Ki-67 index and chemotherapy regimen to NACT were analyzed. Ki-67 index≥25% and high Hyams grade were related to the efficacy of NACT (all P<0.05). Conclusions: NACT could reduce Ki-67 index in ONBs. High Ki-67 index and Hyams grade are clinical indicators sensitive to the efficacy of NACT. NACT-surgery-radiotherapy is effective for patients with locally advanced ONB.
目的： 评估局部晚期嗅神经母细胞瘤（olfactory neuroblastoma，ONB）使用新辅助化疗等综合治疗方案的临床效果，并分析新辅助化疗有效性相关临床因素。 方法： 回顾性分析2017年4月至2022年7月于北京同仁医院接受新辅助化疗的ONB患者25例，其中男性16例，女性9例，年龄26~72岁，平均年龄44.9岁。其中Kadish C期22例，D期3例。所有患者经多学科讨论后，采用新辅助化疗-手术-放疗进行治疗，其中17例采用紫杉醇、顺铂和依托泊苷（TEP）方案；4例采用紫杉醇、奈达铂和异环磷酰胺（TPI）方案，3例采用紫杉醇、顺铂/奈达铂（TP）方案，1例采用顺铂和依托泊苷（EP）方案。采用SPSS 25.0软件进行统计学分析，Kaplan-Meier法绘制生存曲线。 结果： 新辅助化疗的整体有效率为32%（8/25）。21例患者采用扩大经鼻内镜肿瘤切除术，4例采用颅鼻联合入路手术。3例D期患者接受了颈部淋巴结清扫术。所有患者均行术后放疗。随访时间6~67个月，全部患者的5年总体生存率和无病生存率分别为100.0%和94.4%。新辅助化疗前Ki-67指数为60%（50%，90%）［M（Q1，Q3），后同］，化疗后Ki-67指数为20%（3%，30%），化疗前后瘤体组织Ki-67指数变化有统计学意义（Z=-24.24，P<0.05）。分析年龄、性别、手术史、病理分级、新辅助化疗前Ki-67指数以及化疗方案对化疗效果的影响，Ki-67指数≥25%和病理分级高与新辅助化疗有效相关（P值均<0.05）。 结论： 新辅助化疗可以降低ONB患者肿瘤组织Ki-67指数。病理分级和Ki-67指数高是新辅助化疗敏感的相关临床指标。新辅助化疗-手术-放疗的治疗策略，可使局部晚期ONB患者获得满意的5年生存率。.