J Neurol Surg B Skull Base 2021; 82(S 02): S65-S270
DOI: 10.1055/s-0041-1725285
Presentation Abstracts
Live Session Abstracts

Multicenter Study on Clinical Outcomes of Olfactory Neuroblastoma

Matt Lechner
1   Stanford University, Stanford, California, United States
,
Yoko Takahashi
2   Department of Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, United States
,
Mario Hermsen
3   Department Head and Neck Oncology, Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Avenida de Roma, Oviedo, Spain
,
Mario Turri-Zanoni
4   Unit of Otorhinolaryngology - Head and Neck Surgery, University of Insubria, Varese, Italy
,
Volker Schartinger
5   Department of Otorhinolaryngology, Medical University of Innsbruck, Innsbruck, Austria
,
Diana M. Bell
6   Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas, United States
,
Sam Helman
7   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
Jordan Varghese
7   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
Kelly Magliocca
8   Department of Pathology, Emory University, Atlanta, Georgia, United States
,
Jozsef Dudas
5   Department of Otorhinolaryngology, Medical University of Innsbruck, Innsbruck, Austria
,
Herbert Riechelmann
5   Department of Otorhinolaryngology, Medical University of Innsbruck, Innsbruck, Austria
,
Raman Kaur
9   Department of Otolaryngology - Head and Neck Surgery, Johns Hopkins School of Medicine, Baltimore, Maryland, United States
,
Susanne Sprung
10   Institute of Pathology, Medical University of Innsbruck, Innsbruck, Austria
,
David Howard
11   Head and Neck Cancer Unit, Imperial College Healthcare NHS Trust, South Wharf Road, London, United Kingdom
,
Nils Engel
12   Ludwig-Maximilians University, Munich, Germany
,
Tianna Zhao
13   Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Jacklyn Liu
14   Head and Neck Centre, University College London Hospitals NHS Trust, Euston Road, London, United Kingdom
,
Fabio Facchetti
15   Department of Pathology, University of Brescia School of Medicine, Spedali Civili Brescia, Brescia, Italy
,
Roberta Maragliano
16   Unit of Pathology, University of Insubria, Varese, Italy
,
Simonetta Battocchio
15   Department of Pathology, University of Brescia School of Medicine, Spedali Civili Brescia, Brescia, Italy
,
Alessandro Franchi
17   Department of Surgery and Translational Medicine. University of Florence. Largo Brambilla, Firenze, Italy
,
Jacklyn Liu
18   UCL Cancer Institute, University College London, London, United Kingdom
,
Dawn Carnell
14   Head and Neck Centre, University College London Hospitals NHS Trust, Euston Road, London, United Kingdom
,
David Capper
19   Department of Neuropathology, Charite University, Berlin, Germany
,
Ulrich Schueller
20   University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Martin Forster
14   Head and Neck Centre, University College London Hospitals NHS Trust, Euston Road, London, United Kingdom
,
Cillian Forde
21   Royal National Throat, Nose and Ear Hospital and Head and Neck Centre, University College London Hospitals NHS Trust, London, United Kingdom
,
Amrita Jay
22   Department of Histopathology, University College London Hospitals NHS Trust, Euston Road, London, United Kingdom
,
R. Peter Manes
23   Division of Otolaryngology, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, United States
,
Benjamin L. Judson
23   Division of Otolaryngology, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, United States
,
Eugenia Vining
23   Division of Otolaryngology, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, United States
,
Robbie Woods
24   Department of Otolaryngology, Head and Neck Surgery, Beaumont Hospital, Dublin, Ireland
,
C. Arturo Solares
7   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
James P. O'Neill
24   Department of Otolaryngology, Head and Neck Surgery, Beaumont Hospital, Dublin, Ireland
,
Piero Nicolai
25   Section of Otorhinolaryngology - Head and Neck Surgery Department of Neurosciences University of Padova, Italy
,
Pavol Surda
26   Guy's Hospital, King's College, London, United Kingdom
,
Paolo Bossi
27   Medical Oncology, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health University of Brescia, ASST-Spedali Civili, Brescia, Italy
,
Vittorio Rampinelli
28   Unit of Otorhinolaryngology - Head and Neck Surgery, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, ASST Spedali Civili of Brescia, Italy
,
Oswaldo A. Henriquez
7   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
Claire Hopkins
26   Guy's Hospital, King's College, London, United Kingdom
,
Selbam Thavaraj
29   Centre for Clinical, Oral and Translational Science, King's College London, Department of Head and Neck pathology, Guys Hospital, London, United Kingdom
,
Wendell G. Yarbrough
30   Department of Otolaryngology/Head and Neck Surgery, University of North Carolina, Chapel Hill, North Carolina, United States
,
Nyall R. London
9   Department of Otolaryngology - Head and Neck Surgery, Johns Hopkins School of Medicine, Baltimore, Maryland, United States
,
Gary L. Gallia
13   Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Sarah K. Wise
7   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
Jose L. Llorente
3   Department Head and Neck Oncology, Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Avenida de Roma, Oviedo, Spain
,
Paolo Castelnuovo
4   Unit of Otorhinolaryngology - Head and Neck Surgery, University of Insubria, Varese, Italy
,
Ehab Y. Hanna
2   Department of Head and Neck Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, United States
,
Valerie J. Lund
21   Royal National Throat, Nose and Ear Hospital and Head and Neck Centre, University College London Hospitals NHS Trust, London, United Kingdom
› Author Affiliations
 

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

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