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Abstract：
Esthesioneuroblastoma (ENB) or olfactory neuroblastoma is a rare malignant neoplasm arising from the neural crest cells of the olfactory epithelium. The optimum treatment for this rare disease is still unclear. Most of the available literature on this rare head and neck tumour is limited to small retrospective series and single institutional reports. We conducted a retrospective study to investigate the clinical profile, treatment outcomes and prognostic factors of patients with ENB treated at a tertiary cancer centre in south India. Patients with a histopathological diagnosis of ENB treated from 2000 to 2019 were included. Patient demographics, tumour characteristics, stage, treatment details and outcome data were identified from medical records. Overall survival (OS) and disease-free survival (DFS) were estimated using the Kaplan-Meier method and the log-rank test was used for comparison. The prognostic factors were identified using Cox regression analysis. Forty-two patients underwent treatment for ENB from 2000 to 2019. Twenty-six patients underwent surgery. Twelve patients received radical radiotherapy (RT) while 24 patients underwent adjuvant radiation. After a median follow-up of 71 months, the estimated OS and DFS at 4 years were 64.4% and 54%, respectively. The estimated 4-year OS for modified Kadish A, B, C and D stages was 75.0%, 90.9%, 56.4% and 0%, respectively. Modified Kadish stage, nodal involvement, orbital invasion, intracranial extension, surgery, RT treatment and use of chemotherapy were significant predictors of OS and DFS in univariate Cox regression analysis. Orbital invasion and RT treatment were significant predictors of DFS in the multivariate analysis as well. However, only RT treatment came out to be a significant predictor for OS in multivariate Cox regression analysis. Surgery is the mainstay of treatment. Adjuvant RT may improve local control and survival in advanced cases. Advanced modified Kadish stage, lymph node involvement and orbital invasion are associated with poor outcomes.
摘要：
嗅觉神经母细胞瘤（ENB）或嗅觉神经母细胞瘤是一种罕见的恶性肿瘤，由嗅觉上皮的神经c细胞引起。这种罕见疾病的最佳治疗方法仍不清楚。关于这种罕见的头颈部肿瘤的大多数可用文献仅限于小型回顾性系列和单一机构报告。我们进行了一项回顾性研究，以调查临床概况，在印度南部三级癌症中心接受治疗的ENB患者的治疗结果和预后因素。纳入2000年至2019年接受组织病理学诊断为ENB的患者。患者人口统计学，肿瘤特征，舞台,从医疗记录中确定治疗细节和结果数据.使用Kaplan-Meier方法估计总生存期（OS）和无病生存期（DFS），并使用对数秩检验进行比较。采用Cox回归分析确定预后因素。2000年至2019年，42例患者接受了ENB治疗。26名患者接受了手术。12例患者接受根治性放疗（RT），24例患者接受辅助放疗。经过71个月的中位随访，估计4年的OS和DFS分别为64.4%和54%，分别。改良KadishA的估计4年OS，B,C和D阶段为75.0%，90.9%,56.4%和0%，分别。改良Kadish阶段，节点参与，轨道入侵,颅内扩张,手术,在单因素Cox回归分析中，RT治疗和化疗的使用是OS和DFS的重要预测因素。在多变量分析中，轨道侵袭和RT治疗也是DFS的重要预测因子。然而,在多变量Cox回归分析中，只有RT治疗是OS的重要预测因子。手术是治疗的主要手段。辅助RT可以改善晚期病例的局部控制和生存率。先进的改良Kadish阶段，淋巴结受累和眼眶侵犯与不良预后相关。
