Clinical Outcomes and Multidisciplinary Patterns of Failure for Olfactory Neuroblastoma: The Ohio State Experience

Abstract

Purpose Olfactory neuroblastoma (ONB) is a rare head and neck cancer believed to be originated from neural crest cells of the olfactory membrane located in the roof of the nasal fossa. This study evaluates clinical outcomes and failure patterns in ONB patients of those patients treated with surgical resection at a high-volume tertiary cancer center. Methods and Materials Thirty-nine ONB patients who underwent surgical resection at our institution from 1996 to 2017 were retrospectively identified. Univariate, multivariate, and survival analysis were calculated using Cox regression analysis and Kaplan–Meier log-rank. Results Median follow-up time was 59 months (range: 5.2–236 months). The median overall survival (OS) and disease-free survival (DFS) for the entire cohort were 15 and 7.6 years, respectively. The 5-year cumulative OS and DFS were 83 and 72%, respectively. The 5-year OS for low Hyams grade (LHG) versus high Hyams grade (HHG) was 95 versus 61% (p = 0.041). LHG was found in 66% of the early Kadish stage patients compared with 28% in the advanced Kadish stage patients (p = 0.057). On multivariate analysis, HHG and positive node status predicted for worse OS and only HHG predicted for worse DFS. Of note, five patients (all Kadish stage A) who received surgical resection alone had no observed deaths or recurrences with a median follow-up of 44 months (range: 5–235 months). Conclusion In this retrospective cohort, patients with positive nodes or HHG have significantly worse clinical outcomes. Future studies should explore treatment intensification for HHG or positive nodes.

Patterns of recurrences in sinonasal cancers undergoing an endoscopic surgery-based treatment: Results of the MUSES* on 940 patients

“…present study, which is slightly lower than what described in literature [39][40][41]. However, the distribution of failures among local (7.1%, 8/112 patients), regional (5.4%, 6/112 patients) and distant sites (7.1%, 8/112 patients), appears to be similar between our study and previous reports [30,[39][40][41]. Noticeably, this distribution has been re-shaped by the protective role of elective neck irradiation in Kadish C ONBs, which has been introduced in the last decade in many centers worldwide [36,42].…”

“…The present series counted a total of 112 cases of ONB, with 10-year OS and DFS of 90.9% and 69.1%, respectively, comparing equal or better with other series [30][31][32]. According to the literature, most recognized negative prognostic factors for ONB are advanced Kadish stage, high Hyams grade, the presence of nodal metastasis and infiltrated postoperative surgical margins [30,31,[33][34][35][36].…”

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

“…Most patients were males (pooled proportion of female patients: 39.6%, 95% CI: 35.6%–43.7%, I2 = 54%). Mean or median age at presentation was most commonly reported in the 5th 2,8,20,22,27,30,31,35 and 6th decades 21,24,25,28,29,32,34 …”

The genomics and epigenetics of olfactory neuroblastoma: A systematic review

“…ONB was first described in 1924 and named “L'esthesioneuroepitheliome olfactif.” 3 Due to lack of information on its histological origin, additional names have since been used including olfactory neurocytoma and others 4,5 . The incidence of ONB is 0.4/million/year, accounting for approximately 2%‐3% of nasal cavity tumors 6,7 . To date, no specific risk factors have been identified for ONB 8 .…”

“… 4 , 5 The incidence of ONB is 0.4/million/year, accounting for approximately 2%‐3% of nasal cavity tumors. 6 , 7 To date, no specific risk factors have been identified for ONB. 8 Hyams histologic grading and Kadish, modified Kadish, and Dulguerov staging are traditionally used to clinically characterize the tumor and aid in treatment planning.…”