Nair Lekha Madhavan, Mathew John Mohan, Rafi Malu, Thommachan Kainickal Cessal, Km Jagathnath Krishna, Varghese Bipin T, Ravikumar Rejnish
Department of Radiation Oncology, Regional Cancer Centre, Thiruvananthapuram 695011, India.
Department of Epidemiology and Biostatistics, Regional Cancer Centre, Thiruvananthapuram 695011, India.
Ecancermedicalscience. 2023 Jul 27;17:1584. doi: 10.3332/ecancer.2023.1584. eCollection 2023.
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.
嗅神经母细胞瘤（ENB）或嗅觉神经母细胞瘤是一种起源于嗅上皮神经嵴细胞的罕见恶性肿瘤。这种罕见疾病的最佳治疗方法仍不明确。关于这种罕见头颈部肿瘤的现有文献大多局限于小型回顾性系列研究和单一机构报告。我们进行了一项回顾性研究，以调查在印度南部一家三级癌症中心接受治疗的ENB患者的临床特征、治疗结果和预后因素。纳入2000年至2019年经组织病理学诊断为ENB的患者。从病历中确定患者的人口统计学特征、肿瘤特征、分期、治疗细节和结局数据。采用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。使用Cox回归分析确定预后因素。2000年至2019年期间，42例患者接受了ENB治疗。26例患者接受了手术。12例患者接受了根治性放疗（RT），24例患者接受了辅助放疗。中位随访71个月后，4年时的估计OS和DFS分别为64.4%和54%。改良Kadish A、B、C和D期的估计4年OS分别为75.0%、90.9%、56.4%和0%。在单因素Cox回归分析中，改良Kadish分期、淋巴结受累、眼眶侵犯、颅内扩展、手术、RT治疗和化疗的使用是OS和DFS的显著预测因素。在多因素分析中，眼眶侵犯和RT治疗也是DFS的显著预测因素。然而，在多因素Cox回归分析中，只有RT治疗是OS的显著预测因素。手术是主要治疗方法。辅助RT可能改善晚期病例的局部控制和生存率。改良Kadish分期晚期、淋巴结受累和眼眶侵犯与不良结局相关。
