BACKGROUND: Treatment for dural recurrence of olfactory neuroblastoma (ONB) is not standardized. We assess the outcomes of stereotactic body radiotherapy (SBRT) in this population.
METHODS: ONB patients with dural recurrences treated between 2013 and 2022 on a prospective registry were included. Tumor control, survival, and patient-reported quality of life were analyzed.
RESULTS: Fourteen patients with 32 dural lesions were evaluated. Time to dural recurrence was 58.3 months. Thirty lesions (94%) were treated with SBRT to a median dose of 27 Gy in three fractions. Two patients (3 of 32 lesions; 9%) developed in-field radiographic progression, five patients (38%) experienced progression in non-contiguous dura. Two-year local control was 85% (95% CI: 51-96%). There were no >grade 3 acute toxicities and 1 case of late grade 3 brain radionecrosis.
CONCLUSIONS: In this largest study of SBRT reirradiation for ONB dural recurrence to date, high local control rates with minimal toxicity were attainable.

OBJECTIVE: Due to the rarity of olfactory neuroblastoma (ONB), there is ongoing debate about optimal treatment strategies, especially for early-stage or locally advanced cases. Therefore, our study aimed to explore experiences from multiple centers to identify factors that influence the oncological outcomes of ONB.
METHODS: We retrospectively analyzed 195 ONB patients treated at nine tertiary hospitals in South Korea between December 1992 and December 2019. Kaplan-Meier survival analysis was used to evaluate oncological outcomes, and a Cox proportional hazards regression model was employed to analyze prognostic factors for survival outcomes. Furthermore, we conducted 1:1 nearest-neighbor matching to investigate differences in clinical outcomes according to the use of neoadjuvant chemotherapy.
RESULTS: In our cohort, the 5-year overall survival (OS) rate was 78.6%, and the 5-year disease-free survival (DFS) rate was 62.4%. The Cox proportional hazards model revealed that the modified Kadish (mKadish) stage and Dulguerov T status were significantly associated with DFS, while the mKadish stage and Hyams grade were identified as prognostic factors for OS. The subgroup analyses indicated a trend toward improved 5-year DFS with dural resection in mKadish A and B cases, even though the result was statistically insignificant. Induction chemotherapy did not provide a survival benefit in this study after matching for the mKadish stage and nodal status.
CONCLUSIONS: Clinical staging and pathologic grading are important prognostic factors in ONB. Dural resection in mKadish A and B did not show a significant survival benefit. Similarly, induction chemotherapy also did not show a survival benefit, even after stage matching.

BACKGROUND: Patients with sinonasal malignancy (SNM) present with significant sinonasal quality of life (QOL) impairment. Global sinonasal QOL as measured by the 22-item Sinonasal Outcomes Test (SNOT-22) has been shown to improve with treatment. This study aims to characterize SNOT-22 subdomain outcomes in SNM.
METHODS: Patients diagnosed with SNM were prospectively enrolled in a multi-center patient registry. SNOT-22 scores were collected at the time of diagnosis and through the post-treatment period for up to 5 years. Multivariable regression analysis was used to identify drivers of variation in SNOT-22 subdomains.
RESULTS: Note that 234 patients were reviewed, with a mean follow-up of 22 months (3 months-64 months). Rhinologic, psychological, and sleep subdomains significantly improved versus baseline (all p < 0.05). Subanalysis of 40 patients with follow-up at all timepoints showed statistically significant improvement in rhinologic, extra-nasal, psychological, and sleep subdomains, with minimal clinically important difference met between 2 and 5 years in sleep and psychological subdomains. Adjuvant chemoradiation was associated with worse outcomes in rhinologic (adjusted odds ratio (5.22 [1.69-8.66])), extra-nasal (2.21 [0.22-4.17]) and ear/facial (5.53 [2.10-8.91]) subdomains. Pterygopalatine fossa involvement was associated with worse outcomes in rhinologic (3.22 [0.54-5.93]) and ear/facial (2.97 [0.32-5.65]) subdomains. Positive margins (5.74 [2.17-9.29]) and surgical approach-combined versus endoscopic (3.41 [0.78-6.05])-were associated with worse psychological outcomes. Adjuvant radiation (2.28 [0.18-4.40]) was associated with worse sleep outcomes.
CONCLUSIONS: Sinonasal QOL improvements associated with treatment of SNM are driven by rhinologic, extra-nasal, psychological, and sleep subdomains.

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.

Esthesioneuroblastoma (ENB) is a rare sinonasal malignant neoplasm with 40% 5-year survival. Because of the rarity of the tumor, the optimal treatment and subsequent prediction of prognosis are unclear. We studied a modern series of patients with ENB to evaluate the association of immunohistochemical (IHC) markers and clinical stages/grades with outcomes.
A single-center retrospective review of patients with ENB treated during a 25-year period was performed. A systematic literature review evaluating the prognostic benefits of current staging systems in evaluating survival outcomes in ENB was undertaken.
Among 29 included patients, 25 (85%) were treated surgically at our institution, with 76% of those endoscopically resected; 7 (24.1%) received chemotherapy, and 18 (62.1%) received radiation therapy. The 5-year overall survival (OS) was 91.3%, and 10-year OS was 78.3%. Progression-free survival at 5 and 10 years was 85.6% and 68.2%, respectively. A total of 36 distinct IHC markers were used to diagnose ENB but were inconsistent in predicting survival. A systematic literature review revealed predictive accuracy for OS using the Kadish, TNM, and Hyams staging/grading systems was 68%, 42%, and 50%, respectively.
This study reports the 5- and 10-year OS and progression-free survival in a modern series of patients with ENB. No traditional IHC marker consistently predicted outcome. Some novel reviewed markers show promise but have yet to enter clinical mainstream use. Our systematic review of accepted staging/grading systems also demonstrated a need for further investigation due to limited prognostic accuracy.

The esthesioneuroblastoma (ENB) is a rare malignant sinonasal tumor of neuroectodermal origin. This study aimed to improve the understanding of the clinical features by reviewing the literature and analyzing the medical records of patients diagnosed with ENB in our institution between 2012 and 2019. A total of 6 cases of ENB were available for analysis. The mean age at the time of diagnosis was 36 years. The main complaints at presentation were the rhinologic signs. Tumors were classified as stage C of Kadisk in 3 cases and stage D in the others. According to TNM (modified by Dulguerov), 2 patients were T3N0M0, one T4N0M0, one T3N1M0, and two T4N1M0. The diagnosis of ENB was based on pathological examination. According to Hyams histological grading, low-grade lesions (Hyams I and II) were seen in 2 cases, high-grade undifferentiated lesions (Hyams III and IV) were seen in 4. Of the 6 patients, 4 received surgery. The surgical approaches mainly included an endoscopic endonasal resection in 2 cases, a cranial-facial resection surgery in 1 case, and an expanded endoscopic endonasal approach in combination with craniotomy in 1 case. Four patients received adjuvant radiotherapy (RT). RT dose ranged from 60 to 70 Gy. A total of 3 patients had lymph node metastasis and received RT of the neck. Chemotherapy was delivered in 2 patients. After a mean follow-up of 4.5 years, 4 patients were free of recurrence. Unfortunately, 1 patient died from a progressive disease 6 months after RT. ENB is a rare locally aggressive tumor of the nasosinusal cavities. The first-line treatment for resectable tumors should include primary surgical resection with adjuvant RT. However, this tumor remains of poor prognosis. Therefore, long-term close follow-up based on symptoms, endoscopy, and imaging is essential.

OBJECTIVE: Esthesioneuroblastoma (ENB) is a rare malignancy of the sinonasal tract and its infrequency has confounded efforts at clearly describing the survival trends associated with this neoplasm over the years. In this study, we reviewed survival trends in ENB and investigated the impact of treatment extent and modality on patient outcomes.
METHODS: We accessed the Surveillance, Epidemiology, and End Result (SEER) program to identify ENB cases from 1998 to 2016. A χ2 test was used to compare the categorical covariates and a t test or Mann-Whitney U test was utilized for continuous variables. The impact of prognostic factors on survival was computed using a Kaplan-Meier analysis and multivariate Cox proportional hazards model. We divided ENB patients into 4 periods including 1998-2002, 2003-2007, 2008-2012, and 2013-2016, and investigated survival trends using the Kaplan-Meier curve and log-rank test.
RESULTS: ENB patients who underwent biopsy alone were associated with older age, larger tumor diameter, increased rates of tumor extension, nodal/distant metastases, and advanced stages as compared with patients undergoing tumor resection. Our results also demonstrated that surgical resection and adjuvant radiotherapy could confer survival advantages, whereas chemotherapy was associated with reduced survival in patients with ENB. Over the past 2 decades, surprisingly, there has been no change in survival rates for patient with ENB (P = 0.793).
CONCLUSIONS: Despite advanced diagnostic studies and modernized treatment approaches, ENB survival has remained unchanged over the years, calling for improved efforts to develop appropriate individualized interventions for this rare tumor entity. Our results also confirmed that surgery and adjuvant radiotherapy is associated with improved patient survival whereas the use of chemotherapy should be considered carefully.

Olfactory neuroblastoma (ONB) is a rare neuroepithelial-derived malignancy that usually presents in the nasal cavity. The rarity of ONB has led to conflicting reports regarding associations of patient age and ONB survival and outcome. Moreover, long-term outcomes of chemotherapy and other treatment modalities are speculated. Here, we aimed to compare survival outcomes across age groups through time and determine associations between treatment modality and survival. In this retrospective population-based study, we analyzed the SEER 2000-2016 Database for patients with ONB tumors. Using Kaplan-Meier survival analysis, a significant effect of age and cancer-specific survival (CSS) was observed; geriatric ONB patients had the lowest CSS overall. Generalized linear models and survival analyses demonstrated that CSS of the pediatric patient population was similar to the geriatric group through 100 months but plateaued thereafter and was the highest of all age groups. Radiation and surgery were associated with increased CSS, while chemotherapy was associated with decreased CSS. GLM results showed that tumor grade, stage and lymph node involvement had no CSS associations with age or treatment modality. Our results provide insight for future investigations of long-term outcomes associated with ONB patient age and treatment modality, and we conclude that survival statistics of ONB patients should be analyzed in terms of trends through time rather than fixed in time.

BACKGROUND: In this retrospective surveillance, epidemiology, and end results (SEER) registry analysis, we investigated the role of chemotherapy (CT) in the treatment of olfactory neuroblastoma (ON), an exceedingly rare sino-nasal tumor typically treated with surgery and/or radiation therapy (RT).
METHODS: We analyzed all patients in the SEER registry diagnosed with a single primary malignancy of ON, a primary tumor site within the nasal cavity or surrounding sinuses, sufficient staging information to derive Kadish staging, and >0 days of survival, ensuring follow-up data. Receipt of CT in the SEER registry was documented as either Yes or No/Unknown.
RESULTS: Six hundred and thirty-six patients were identified. One hundred and ninety-five patients received CT as part of their treatment for ON. Following propensity score matching and inverse probability of treatment weighting, there was inferior overall survival (OS) (HR 1.7, 95% CI: 1.3-2.2, P = .001) and cancer-specific survival (CSS) (HR 1.8, 95% CI: 1.3-2.4, P < .001) for patients who received CT compared to those who were not treated with CT or had unknown CT status. On subgroup analysis, the only patient population that derived benefit from CT were patients who did not receive surgery and were treated with CT and/or RT (HR 0.3, 95% CI: 0.14-0.61, P < .001).
CONCLUSIONS: Based on this retrospective SEER registry analysis, the use of CT in the management of ON is associated with decreased OS. Our analysis suggests that patients who are considered nonsurgical candidates may benefit from the addition of CT.

BACKGROUND: Esthesioneuroblastoma (ENB) is an uncommon sinonasal malignancy and is even less common in the pediatric population.
OBJECTIVE: The purpose of this study is to compare characteristics and outcomes of ENB between adult and pediatric patients.
METHODS: The National Cancer Database was queried for patients with histologically proven ENB of the nasal cavity and paranasal sinuses, and then baseline characteristics, treatment, and survival data compared between the pediatric (age < 18 years) and adult (age ≥ 18 years) populations.
RESULTS: 1411 patients were identified, with 45 in the pediatric cohort and 1366 in the adult cohort. Ten-year overall survival (OS) in the pediatric cohort was improved compared to the adult cohort, 87% and 66%, respectively (p < 0.05). Adjuvant chemotherapy was more commonly utilized in the pediatric cohort (p < 0.001). Race was associated with decreased OS in the pediatric cohort (p = 0.013). Pediatric patients had shorter length of stay (p = 0.009) and lived closer to their provider (p = 0.044) than adult ENB patients.
CONCLUSIONS: Treatment of ENB in pediatric patients more commonly includes chemotherapy and more commonly occurs at academic medical centers. OS is improved in pediatric ENB compared to adults as well, but larger studies are necessary.