Update on olfactory neuroblastoma.

Olfactory neuroblastomas are uncommon malignancies that arise from olfactory receptor cells located high in the nasal cavity. Accurate diagnosis plays a crucial role in determining clinical results and guiding treatment decisions. Diagnosis can be a major challenge for pathologists, especially when dealing with tumours with poor differentiation. The discovery of several molecular and immunohistochemical markers would help to overcome classification difficulties. Due to the paucity of large-scale studies, standardisation of diagnosis, treatment and prediction of outcome remains a challenge. Surgical resection by endoscopic techniques with the addition of postoperative irradiation is the treatment of choice. In addition, it is advisable to consider elective neck irradiation to minimise the risk of nodal recurrence. Molecular characterisation will help not only to make more accurate diagnoses but also to identify specific molecular targets that can be used to develop personalised treatment options tailored to each patient. The present review aims to summarise the current state of knowledge on histopathological diagnosis, the molecular biology and management of this disease.

A multi-institutional retrospective study of 340 cases of sinonasal malignant tumor.

OBJECTIVE: Sinonasal malignant tumors (SNMT) are relatively rare among head and neck malignant tumors. Most are squamous cell carcinomas, and malignant melanomas, olfactory neuroblastomas, adenoid cystic carcinomas, sarcomas, and others also occur. The most common primary site of nasal sinus squamous cell carcinoma is the maxillary sinus. In recent years, a decrease in incidence of maxillary sinus squamous cell carcinoma (MSSCC) has been reported along with a decrease in the incidence of sinusitis. MSSCC is treated with a combination of surgery, radiation, and chemotherapy. Treatment decisions are made according to the progression of the disease, the patient's general condition, and the patient's own wishes. There are variations in treatment policies among facilities due to the specialty of staff and cooperation with other departments at each facility. We conducted a multi-institutional retrospective study to compare outcomes by treatment strategy. METHODS: In this study, 340 patients with SNMT who were treated at 13 Hospitals (Head and Neck Oncology Group (Kyoto-HNOG) ) during the 12-year period from January 2006 to December 2017 were included. There were 220 patients with squamous cell carcinoma, 32 with malignant melanoma, 21 with olfactory neuroblastoma, and 67 with other malignancies. Of the squamous cell carcinomas, 164 were of maxillary sinus origin. One hundred and forty cases of MSSCC that were treated radically were included in the detailed statistical analysis. RESULTS: There were 5 cases of cStage I, 9 cases of cStage II, 36 cases of cStage III, 74 cases of cStage IVa, and 16 cases of cStage IVb. There were 92 cases without clinical lymph node metastasis (cN(-)) and 48 cases with clinical lymph node metastasis(cN(+)). Primary tumors were treated mainly by surgery in 85 cases (Surg) and by radical radiation therapy (with or without chemotherapy) of 6-70 Gy in 55 cases(non-Surg). The 5-year overall/disease-free survival rate (OS/DFS) for MSSCC was 65.1%/51.6%. Old age, renal dysfunction, and clinical T progression were independent risk factors for OS, and renal dysfunction was an independent risk factor for DFS. In cN(-) patients, OS and DFS were significantly better in Surg group than in non-Surg group. In cN(+) patients, there was no significant difference in OS and DFS between Surg and non-Surg groups. CONCLUSION: For patients with MSSCC without lymph node metastasis, aggressive surgery on the primary tumor contributes to improved prognosis.

Multicenter Survival Analysis and Application of an Olfactory Neuroblastoma Staging Modification Incorporating Hyams Grade.

Importance: Current olfactory neuroblastoma (ONB) staging systems inadequately delineate locally advanced tumors, do not incorporate tumor grade, and poorly estimate survival and recurrence. Objective: The primary aims of this study were to (1) examine the clinical covariates associated with survival and recurrence of ONB in a modern-era multicenter cohort and (2) incorporate Hyams tumor grade into existing staging systems to assess its ability to estimate survival and recurrence. Design, Setting, and Participants: This retrospective, multicenter, case-control study included patients with ONB who underwent treatment between January 1, 2005, and December 31, 2021, at 9 North American academic medical centers. Intervention: Standard-of-care ONB treatment. Main Outcome and Measures: The main outcomes were overall survival (OS), disease-free survival (DFS), and disease-specific survival (DSS) as C statistics for model prediction. Results: A total of 256 patients with ONB (mean [SD] age, 52.0 [15.6] years; 115 female [44.9%]; 141 male [55.1%]) were included. The 5-year rate for OS was 83.5% (95% CI, 78.3%-89.1%); for DFS, 70.8% (95% CI, 64.3%-78.0%); and for DSS, 94.1% (95% CI, 90.5%-97.8%). On multivariable analysis, age, American Joint Committee on Cancer (AJCC) stage, involvement of bilateral maxillary sinuses, and positive margins were associated with OS. Only AJCC stage was associated with DFS. Only N stage was associated with DSS. When assessing the ability of staging systems to estimate OS, the best-performing model was the novel modification of the Dulguerov system (C statistic, 0.66; 95% CI, 0.59-0.76), and the Kadish system performed most poorly (C statistic, 0.57; 95% CI, 0.50-0.63). Regarding estimation of DFS, the modified Kadish system performed most poorly (C statistic, 0.55; 95% CI, 0.51-0.66), while the novel modification of the AJCC system performed the best (C statistic, 0.70; 95% CI, 0.66-0.80). Regarding estimation of DSS, the modified Kadish system was the best-performing model (C statistic, 0.79; 95% CI, 0.70-0.94), and the unmodified Kadish performed the worst (C statistic, 0.56; 95% CI, 0.51-0.68). The ability for novel ONB staging systems to estimate disease progression across stages was also assessed. In the novel Kadish staging system, patients with stage VI disease were approximately 7 times as likely to experience disease progression as patients with stage I disease (hazard ratio [HR], 6.84; 95% CI, 1.60-29.20). Results were similar for the novel modified Kadish system (HR, 8.99; 95% CI, 1.62-49.85) and the novel Dulguerov system (HR, 6.86; 95% CI, 2.74-17.18). Conclusions and Relevance: The study findings indicate that 5-year OS for ONB is favorable and that incorporation of Hyams grade into traditional ONB staging systems is associated with improved estimation of disease progression.

Comparing the role of different treatment modalities in locoregionally advanced olfactory neuroblastoma: The 20-year clinical experience of a single institution.

BACKGROUND: The clinical value of different treatment modalities, especially systemic chemotherapy (CT) in patients with locoregionally advanced olfactory neuroblastoma (LA ONB) remains unclear. METHODS: Patients with LA ONB from 2000 to 2020 at our center were collected retrospectively. The entire cohort was divided into combined systemic and local therapy (CSLT) versus local therapy (LT) groups (grouping method 1), and the same cohort was divided into neoadjuvant chemotherapy (NAC) versus non-NAC groups (grouping method 2). CSLT group included patients treated with CT + LT. LT group included patients treated with surgery (SG), radiotherapy (RT), concurrent chemoradiotherapy (CCRT), or any combination of the above methods. LT group was further divided into mono-modality local therapy (MOLT) group and multi-modality local therapy (MULT) group. MOLT group included patients treated with RT alone or SG alone. MULT group included patients treated with SG + RT/CCRT, or CCRT alone. NAC group included patients treated with NAC + LT ± adjuvant chemotherapy (ADC). Non-NAC group included patients who received LT ± ADC. RESULTS: A total of 111 patients with LA ONB were included. The median follow-up was 80.2 months (range, 2.1-254.9). The 5- and 10-year OS rates were 70.2% and 61.3%, respectively. In univariate analysis, patients treated with NAC (n = 43) had significantly better overall survival (OS) compared with those without NAC (n = 68) (p = 0.041). Patients in MULT group (n = 45) had significantly improved OS (p = 0.004) and PFS (p = 0.003) compared with those in MOLT group (n = 15). Multivariate analysis identified NAC and CSLT (n = 51) were independent prognostic factors for superior OS (p = 0.020, p = 0.046). CONCLUSIONS: Our study suggested that CSLT, especially a combination of NAC and LT, improved the survival of patients with LA ONB. Multiple treatment modalities yielded better PFS and OS compared to single-modality treatment.

[Neoadjuvant chemotherapy in the treatment of locally advanced olfactory neuroblastoma in 25 cases].

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.

Olfactory neuroblastoma with rhabdomyoblasts: A rare case report and review of the literature.

Herein we present a case of a 62-year-old male patient who was admitted with the chief complaints of nasal obstruction. The histopathological and immunohistochemical evaluation led to a diagnosis of olfactory neuroblastoma with rhabdomyoblasts. A review of the literature revealed that this is only the fourth case of olfactory neuroblastoma with rhabdomyoblasts. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and identify the best treatment to improve prognosis.

Lymph node metastasis from olfactory neuroblastoma at presentation and as disease relapse: A systematic review and proportion meta-analysis of prevalence data and variables influencing regional control.

BACKGROUND: Aim of this study is to investigate the prevalence of cervical nodal metastasis at presentation and as disease relapse in primary, treatment-naive olfactory neuroblastoma (ONB), and to review treatment modalities, risk factors for regional failure and survival outcomes according to nodal status. METHODS: A systematic review and proportion meta-analysis were conducted following PRISMA guidelines based on PubMed, Web of Science, and Scopus. RESULTS: Eighteen articles were examined. The pooled proportion of patients with nodal metastasis at presentation (11.5%) was comparable to that of cN0 patients not receiving elective neck treatment developing nodal metastasis during follow-up (12.3%). Of the latter, most were Kadish stage C tumors (85.5%). CONCLUSIONS: Cervical involvement is common both at presentation and during follow-up of cN0 ONB. The highest risk of developing late nodal metastasis is seen in cN0 patients with Kadish stage C tumors not receiving elective neck treatment. Elective cN0 neck treatment should be encouraged in selected patients to increase regional control.

A rare presentation of esthesioneuroblastoma: a case report and review of the literature.

Esthesioneuroblastoma is a rare malignant tumor developing from the olfactory neuroepithelium. It represents less than 5% of all cancers of the nasal cavity. We are going to report the observation of a patient followed at the regional oncology center of Oujda in Morocco who presented a locally advanced esthesioneuroblastoma. Treatment consisted of surgical resection followed by adjuvant radiotherapy on the tumor bed. Currently, the patient is in good control of his disease.

Clinical outcomes, Kadish-INSICA staging and therapeutic targeting of somatostatin receptor 2 in olfactory neuroblastoma.

INTRODUCTION: Olfactory neuroblastoma (ONB) is a rare cancer of the sinonasal region. We provide a comprehensive analysis of this malignancy with molecular and clinical trial data on a subset of our cohort to report on the potential efficacy of somatostatin receptor 2 (SSTR2)-targeting imaging and therapy. METHODS: We conducted a retrospective analysis of 404 primary, locally recurrent, and metastatic olfactory neuroblastoma (ONB) patients from 12 institutions in the United States of America, United Kingdom and Europe. Clinicopathological characteristics and treatment approach were evaluated. SSTR2 expression, SSTR2-targeted imaging and the efficacy of peptide receptor radionuclide therapy [PRRT](177Lu-DOTATATE) were reported in a subset of our cohort (LUTHREE trial; NCT03454763). RESULTS: Dural infiltration at presentation was a significant predictor of overall survival (OS) and disease-free survival (DFS) in primary cases (n = 278). Kadish-Morita staging and Dulguerov T-stage both had limitations regarding their prognostic value. Multivariable survival analysis demonstrated improved outcomes with lower stage and receipt of adjuvant radiotherapy. Prophylactic neck irradiation significantly reduces the rate of nodal recurrence. 82.4% of the cohort were positive for SSTR2; treatment of three metastatic cases with SSTR2-targeted peptide-radionuclide receptor therapy (PRRT) in the LUTHREE trial was well-tolerated and resulted in stable disease (SD). CONCLUSIONS: This study presents pertinent clinical data from the largest dataset, to date, on ONB. We identify key prognostic markers and integrate these into an updated staging system, highlight the importance of adjuvant radiotherapy across all disease stages, the utility of prophylactic neck irradiation and the potential efficacy of targeting SSTR2 to manage disease.

Presentation and Outcomes of Non-Squamous Cell Carcinoma Sinonasal Malignancies: A National Perspective.

BACKGROUND: Non-squamous cell carcinoma sinonasal malignancies (NSCCSM) are relatively rare. Neoadjuvant radiotherapy and/or chemotherapy (NTx) have been proposed to improve outcomes compared to surgery alone. In this study, we aim to examine the prevalence of NTx utilization and associated outcomes. METHODS: A retrospective study utilizing the National Cancer Database, 2004 to 2015. The study population included adult patients diagnosed with primary NSCCSM. RESULTS: A total of 574 patients were included. The mean age of the study population was 61.7 ± 16.5 years. The median follow-up time was 40.4 months (interquartile range: 15.3-81.3 months). The histopathological diagnoses identified included: (i) 37.0% adenocarcinoma, (ii) 22.8% adenoid cystic carcinoma, (iii) 20.0% mucosal melanoma, (iv) 11.9% esthesioneuroblastoma, and (v) 8.2% sinonasal undifferentiated carcinoma (SNUC). NTx was utilized in 70 (12.20%) of the study population. Patients who received NTx were more likely to have SNUC or esthesioneuroblastoma (P < .01 each) and to have stage III or IV disease (P < .01 each). NTx was most likely to be administrated in a high-volume center [OR: 3.94, 95%CI: (1.47, 10.53), P = .006]. Patients who received NTx had a significantly lower prevalence of positive margin postoperatively [OR: 0.48, 95%CI: (0.26, 0.87), P = .016]. In patients with NSCCSM, negative margin was associated with improved overall survival [HR: 0.55, 95%CI: (0.36, 0.82), P = .004]. CONCLUSIONS: This study provides an epidemiological perspective regarding NSCCSM and related practice patterns and survival outcomes. Neoadjuvant radiotherapy and/or chemotherapy is likely to decrease the risk of positive margin which ultimately could improve survival in this population.

Orbital invasion by Esthesioneuroblastoma: a comparative case series and review of literature.

PURPOSE: To review the current literature on esthesioneuroblastoma (ENB) as it pertains to clinical features, grading systems, treatment options, and survival. METHODS: A literature search in PubMed was performed to include all articles published in English with orbit involving ENB. Only articles that included each patient's demographics, tumor stage, treatment, or survival were included. A total of 22 articles with 104 patients were considered for this literature review. We also present five cases of ENB, all encountered in our health system, between 2010 and 2020. RESULTS: The median age of diagnosis of orbit involving ENB was 44.5 years. Males were more likely affected than females at 72.9%. Common presenting ocular symptoms were visual change (38.1%), periorbital pain (33.3%), and diplopia (14.3%). Common clinical exam findings were proptosis (47.6%), extraocular movement deficit (23.8%), and periorbital edema (19.0%). Twenty-seven patients (77.1%) received surgery, 22 patients (62.9%) received chemotherapy, and 30 patients (85.7%) received radiation therapy as part of their treatment. Median duration of survival was 124.0 months and 5-year overall survival (OS) was 67.1%. Hyams, Kadish, and Dulguerov T-staging showed inconsistent survival prognosis while orbital invasion and lymph node metastasis had worse outcomes. Our five cases exhibited the spectrum of disease processes evidenced above, with four involving the orbit. CONCLUSIONS: ENB is a rare sinonasal tumor that can invade the orbit. Because of its rarity, no single staging system appears superior. Resection with radiation therapy has superior survival results while the benefits of chemotherapy are currently unknown.

Medulloblastoma and esthesioneuroblastoma in a pediatric patient: a co-incidence or result of common genetic anomaly.

INTRODUCTION: Medulloblastoma is the commonest embryonal brain tumor in children. Their association with other neuroepithelial brain tumors is less known. Here we discuss a pediatric patient who developed esthesioneuroblastoma 2 years after treatment of medulloblastoma. MATERIALS AND METHODS: A 12-year-old male presented with features of raised intracranial hypertension, and radiology showed posterior fossa midline lesion. The tumor was excised, and biopsy revealed medulloblastoma (non-WNT non-SHH type). He received chemoradiation. Two years later he presented with loss of vision, and radiology revealed a sinonasal mass with subfrontal extension. Subtotal resection was done, and biopsy showed blue round cell tumor in favor of esthesioneuroblastoma. RESULT: Parents refused further treatment and the patient died 8months after the second surgery. CONCLUSION: Mixed embryonal and neuroepithelial brain tumors are rare. These may have a common genetic abnormality. They have an aggressive course and bear a poor prognosis.

Long-Term Outcomes of Olfactory Neuroblastoma: MD Anderson Cancer Center Experience and Review of the Literature.

OBJECTIVES/HYPOTHESIS: Olfactory neuroblastoma (ONB) is a rare sinonasal malignant neoplasm that is known to develop late recurrence. The aim of this study is to evaluate the long-term outcomes of patients with ONB and to determine the factors associated with prognosis. STUDY DESIGN: Retrospective study. METHODS: A retrospective review of the medical records of 139 patients diagnosed with ONB at MD Anderson Cancer Center was performed between 1991 and 2016. Descriptive statistics were calculated, and Kaplan-Meier curves were utilized to assess survival. RESULTS: Median follow-up time was 75 months. Overall, 129 patients (92.8%) had surgery as part of their treatment and 82 (58.9%) patients received postoperative radiation therapy (PORT) or concurrent chemoradiotherapy. Endoscopic approaches were utilized for 72 patients, 69.4% of whom had pure endoscopic endonasal approaches. Five-year overall survival and disease-specific survival were 85.6% and 93.4%, respectively. Recurrence rate was 39.6% with a median time to recurrence of 42 months. Among the 31 patients who received elective nodal irradiation (ENI), two patients developed neck recurrence (6.4%) compared with 20 who developed neck recurrence when ENI was omitted (34.4%) (P = .003). Advanced Kadish stage, orbital invasion, intracranial invasion, and presence of cervical lymphadenopathy at the time of presentation were significantly associated with poor survival. CONCLUSION: ONB has an excellent survival. Surgical resection with PORT when indicated is the mainstay of treatment. Endoscopic approaches can be used as a good tool. Elective neck irradiation reduces the risk of nodal recurrence among patients with clinically N0 neck. Despite the excellent survival, recurrence rate remains high and delayed, highlighting the need for long-term surveillance. LEVEL OF EVIDENCE: Level 4 Laryngoscope, 132:290-297, 2022.

Emerging concepts in sinonasal tumor research.

PURPOSE OF REVIEW: Sinonasal malignancies are rare and understudied, often diagnosed at late stages, and may behave aggressively. This review explores investigative diagnostic, therapeutic, and scientific advances specific to sinonasal undifferentiated carcinoma (SNUC), intestinal-type adenocarcinoma (ITAC), and olfactory neuroblastoma (ONB). RECENT FINDINGS: A number of studies have recently contributed more robust knowledge of the genetic and molecular landscapes of SNUC, ITAC, and ONB. These analyses have identified SMARCB1 and IDH2 mutations in SNUC, potentially allowing for the tumor's subdivision. Recent studies have also defined a role for induction chemotherapy in SNUC. Somatic mutations for ITAC have been identified and may be potentially targetable with FDA approved therapies. Studies defining the tumor microenvironment for ITAC and ONB have introduced the possibility of immune checkpoint inhibition for these tumor types. SUMMARY: Studies reviewed here detail promising results of the most current and novel characterization of SNUC, ITAC, and ONB genetic and molecular landscapes, which have informed ongoing therapeutic discovery. With continued multi-institutional efforts, the field of sinonasal tumor research will achieve higher disease control and improved treatment outcomes for patients afflicted with these rare cancers.

[Primary experience and discussion on the treatment of esthesioneuroblastoma].

Objective: To summarize the preliminary experience in the treatment of esthesioneuroblastoma (ENB) and to explore the effect of age, chemotherapy, modified Kadish stage and pathological grade on the prognosis of ENB. Methods: The clinical data of 87 ENB patients from the First Affiliated Hospital of Sun Yat-sen University and Sun Yat-sen University Cancer Center between June 2002 and November 2017 were retrospectively analyzed. The modified Kadish stage was used to evaluate the extent of the lesions, and the Hyams grading system was used for pathological grading. The patients were followed up regularly to evaluate the recurrence and metastasis of the tumor. Cox proportional hazard model was used for univariate and multivariate analyses. Prognostic factors with P<0.05 in univariate analysis were included in multivariate analysis. After controlling the confounding factors, the model coefficients were used to calculate the hazard ratio (HR) and 95% confidence interval (CI). Results: The median follow-up time of ENB patients was 29 months, and the 5-year overall survival rate was 39.3%. In univariate analysis, age, chemotherapy, modified Kadish stage and pathology grade were independent predictors of overall survival, while gender, radiotherapy and surgery were not prognostic factors. Multivariate analysis showed that modified Kadish stage and pathology grade were independent predictors of overall survival rate after excluding confounding factors. Conclusions: Age, chemotherapy, modified Kadish stage and pathological grade are taking important role in the overall survival rate of patients with ENB. Modified Kadish stage and pathological grade are independent predictors of overall survival rate.

Model to predict cause-specific mortality in patients with olfactory neuroblastoma: a competing risk analysis.

PURPOSE: The main objective of this study was to evaluate the cumulative incidence of cause-specific mortality and other causes of mortality for patients with olfactory neuroblastoma (ONB). The secondary aim was to model the probability of cause-specific death and build a competing risk nomogram to predict cause-specific mortality for this disease. METHODS: Patients with ONB from 1975 to 2016 were identified from the Surveillance, Epidemiology, and End Results database. We estimated the cumulative incidence function (CIF) for cause-specific mortality and other causes of mortality, and constructed the Fine and Gray's proportional subdistribution hazard model, as well as a competing-risk nomogram based on Fine and Gray's model, to predict the probability of cause-specific mortality for patients with ONB. RESULTS: After data selection, 826 cases were included for analysis. Five-year cumulative incidence of cause-specific mortality was 19.5% and cumulative incidence of other causes of mortality was 11.3%. Predictors of cause-specific mortality for ONB included tumor stage, surgery and chemotherapy. Age was most strongly predictive of other causes of mortality: patients aged > 60 years exhibited subdistribution hazard ratios of 1.063 (95 % confidence interval [CI] 1.05-1.08; p = 0.001). The competing risk nomogram for cause-specific mortality was well-calibrated, and had good discriminative ability (concordance index = 0.79). CONCLUSIONS: We calculated the CIF of cause-specific mortality and other causes of mortality in patients with the rare malignancy ONB. We also built the first competing risk nomogram to provide useful individualized predictive information for patients with ONB.

[Clinical analysis of 31 cases of esthesioneuroblastoma].

Objective:To investigate the clinical characteristics and prognostic factors of esthesioneuroblastoma. Methods:The clinical data of 31 patients with esthesioneuroblastoma were retrospectively studied. Results:The average time from first onset to diagnosis in 31 patients was 7.84 months, among which 3 patients（9.68%） had cervical lymph node metastasis at the first visit. By the end of follow-up, there were 25 coexisting cases and 6 deaths. The mean recurrence time of 6 patients was 10.6 months. There were 6 patients with distant metastasis, including 4 patients with cervical lymph node metastasis, 1 patient with liver metastasis and 1 patient with bone metastasis. Modified Kadish stage, different treatment methods, recurrence, first diagnosis of cervical lymph node metastasis, and distant metastasis were all factors affecting the prognosis of the patients. Conclusion:The incidence of esthesioneuroblastoma is low and the comprehensive treatment of surgery combined with radiotherapy is the optimal treatment plan. Patients with low Kadish staging, surgical combined with radiotherapy, no recurrence, no initial cervical lymph node metastasis, and no distant metastasis have a better prognosis.