2021•Journal of Neurological Surgery Part B Skull BaseRequires access
Multicenter Study on Clinical Outcomes of Olfactory Neuroblastoma
Matt Lechner, Yoko Takahashi, Mario A. J. A. Hermsen, Mario Turri‐Zanoni, Volker Hans Schartinger, Diana Bell, Sam Helman, Jordan J. Varghese, Kelly R. Magliocca, József Dudás, Herbert Riechelmann, Raman Preet Kaur, Susanne Sprung, David J. Howard, Nils W. Engel, Tianna Zhao, Jacklyn Liu, Fabio Facchetti, Roberta Maragliano, Simonetta Battocchio, Alessandro Franchi, Jacklyn Liu, Dawn M. Carnell, David Capper, Ulrich Schueller, Martin David Forster, Cillian T. Forde, Amrita Jay, Richard Peter Manes, Benjamin L. Judson, Eugenia M. Vining, Robbie Woods, Clementino Arturo Solares, James Paul O'Neill, Piero Nicolai, Pavol Šurda, Paolo Bossi, Vittorio Rampinelli, Oswaldo A. Henriquez, Claire Hopkins, Selbam Thavaraj, Wendell Gray Yarbrough, Nyall R. London, Gary L. Gallia, Sarah K. Wise, José Luís Llorente, Paolo Castelnuovo, Ehab Y. Hanna, Valerie Joan Lund
Abstract
Background and Objectives: Olfactory neuroblastoma (also known as esthesioneuroblastoma) is a rare sinonasal neoplasm which originates from the olfactory neuroepithelium in the upper portion of the nasal cavity adjacent to the cribriform plate. Our objectives were to analyze multicenter clinical data from olfactory neuroblastoma patients to inform on outcomes and to identify novel diagnostic and prognostic biomarkers. Material and Methods: For this multicenter analysis, we included clinical data from 370 olfactory neuroblastoma patients (56.0% male; mean age: 50.5 years) from various centers in the US and Europe, in particular data on clinical presentation, diagnosis, treatment and clinical outcomes. We also analyzed immunohistochemical data (SSTR2) and imaging data (68Ga-DOTA-TOC PET-CT-scanning and 68Ga-DOTA-TOC PET-MRI-scanning) where available to inform about diagnostic and prognostic potential. Statistical testing was performed in SPSS version 24. Results: At initial presentation patients complained of nasal obstruction (66.0%), epistaxis (41.4%), rhinorrhea (24.9%), anosmia (21.8%), headache (19.9%), epiphora (4.8%), and diplopia (4.2%). The mean age at presentation was 50.5 years. The most useful poor prognostic indicator, in addition to Dulguerov and Kadish-Morita staging, was dural infiltration ( p = 0.011, respectively). 88.1% of patients received adjuvant therapy, with improved survival outcome, the majority of whom received adjuvant radiotherapy only. Mean follow-up was 88.7 (±77.9) months; 5-year overall survival 80%. 84.0% of tissue samples stained positive for SSTR2; this was significantly associated with age at diagnosis ( p = 0.036) and expression was associated with 68Ga-DOTA-TOC uptake both in 68Ga-DOTA-TOC PET-CT scanning and 68Ga-DOTA-TOC PET-MRI scanning. Conclusion: Less than a third of olfactory neuroblastoma patients present with anosmia. Our analysis also shows that the most significant prognostic indicator in our set of samples is dural infiltration rather than stage based on commonly used staging systems. The high rate of expression of Somatostatin receptor 2 (SSTR2) with 68Ga-DOTA-TOC uptake in both 68Ga-DOTA-TOC PET-CT-scans and 68Ga-DOTA-TOC PET-MRI-scans shows the enormous translational potential of this marker with regard to imaging and novel targeted therapies. Publication History Article published online: 12 February 2021 © 2021. Thieme. All rights reserved. Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany