Recurrence Morbidity of Olfactory Neuroblastoma
Bibliographic record
Abstract
Background: With modern treatment paradigms, olfactory neuroblastoma (ONB) has a favorable overall survival (OS); however, the incidence of recurrence remains high. The primary aims of this multi-institutional study were to delineate the prognosis of recurrence and explore how recurrence subsites associate with OS, disease-specific survival (DSS), and additional recurrence. Methods: Retrospective chart review of ONB cases from nine academic centers, between 2005 and 2021, was completed. Tumor characteristics, recurrence sub-sites (intracranial, local/ sinus and skull base, neck, and distant), timeline to recurrence, additional recurrence and survival estimates were determined using descriptive and time-to-event analyses. Results: A final study cohort of 233 patients were identified for study inclusion. A total of 70 (30.0%) subjects recurred within 50.4 months [SD ± 40.9] of initial diagnosis. The sub-sites of recurrence were local (50%), neck (36%), intracranial (9%), and distant (6%). Patients with second recurrence ( n = 13) consisted of neck (69%), distant (23%) and intracranial (8%) sub-sites. Patients who recurred had significantly different prevalence of AJCC T stage ( p < 0.001), AJCC overall stage ( p < 0.001), and modified Kadish staging ( p < 0.001) when compared with those without recurrence. Specific imaging findings, contributing to increased stage, and found to be significantly higher in the recurrent cohort included: skull base involvement on CT imaging (74.3% in recurrent group; 56.4% in nonrecurrent group; p = 0.007), periorbital involvement on MRI (25.7% in recurrent group; 12.3% in nonrecurrent; p = 0.019), and dural involvement on MRI (54.3% in recurrent group; 37.4% in nonrecurrent; p = 0.009). Histopathologically, dural involvement (50.0% in recurrent group; 36.2 in nonrecurrent; p = 0.037) and positive margins (31.4% in recurrent group; 21.5% in nonrecurrent; p = 0.022) are significantly higher in the recurrence group. Patients who recurred ( n = 70) had an OS of 43.4% at 60-months from the time of recurrence compared with the entire cohort ( n = 233) OS of 66.7% ( p = 0.839). First recurrence was significantly associated with worse 60-month DSS (HR = 5.62; 95%; p = 0.003) compared with the entire cohort. In addition, patients with either neck (DSS = 94.7% [SE ± 5.1%]) or local sinonasal/skull base recurrence (DSS = 80.3% [SE ± 7.3%]) have a significantly better DSS compared with intracranial recurrence (DSS = 62.5% [SE ± 21.3%]) or distant recurrence (DSS = 50.0% [SE ± 25.0%]). The worse prognosis for intracranial and distance recurrences was not significantly associated with initial tumor stage or Hyam's grade. Conclusions: Recurrent cases of ONB have significantly different AJCC T and overall stage as well as higher modified Kadish staging. Pre-operative imaging factors including skull base, dura and periorbita involvement were found to be statistically different and likely represent the more significant variables of increased staging. Recurrence of ONB was associated with a significantly worse DSS. Specifically, patients with local or neck recurrences have better DSS than those with intracranial or distant recurrence, independent of initial tumor stage or Hyam's grade. Generally, ONB patients have a better prognosis; however, there remains a high recurrence rate in this population. Identifying specific factors that confer increased risk of recurrence and DSS is important for patient counseling in addition to surveillance planning. Publication History Article published online: 01 February 2023 © 2023. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.000 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".