J Neurol Surg B Skull Base 2020; 81(S 01): S1-S272
DOI: 10.1055/s-0040-1702307
Oral Presentations
Georg Thieme Verlag KG Stuttgart · New York

Multicenter Analysis of Clinical Outcomes and Biomarkers of Esthesioneuroblastoma

Matt Lechner
1   UCL Cancer Institute, University College London, London, United Kingdom
,
Mario Hermsen
2   Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Instituto Universitario de Oncología del Principado de Asturias (IUOPA), Centro de Investigación Biomédica en Red (CIBER-ONC), Oviedo, Spain
,
Mario Turri-Zanoni
3   Unit of Otorhinolaryngology - Head & Neck Surgery, University of Insubria, Varese, Italy
,
Volker H. Schartinger
4   Department of Otorhinolaryngology-Head and Neck Surgery, Medical University of Innsbruck, Innsbruck, Austria
,
Sam Helman
5   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
Jordan Varghese
5   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
C. Arturo Solares
5   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
Kelly Magliocca
6   Department of Pathology, Emory University, Atlanta, Georgia, United States
,
Simon Wang
7   Institute of Nuclear Medicine, University College Hospital, London, United Kingdom
,
Susanne Sprung
8   Institute of Pathology, Medical University of Innsbruck, Innsbruck, Austria
,
David Howard
9   Head and Neck Cancer Unit, Imperial College Healthcare NHS Trust, London, United Kingdom
,
Nils Engel
10   Ludwig-Maximilians University, Munich, Germany
,
David Capper
11   Department of Neuropathology, Charite University, Berlin, Germany
,
Ulrich Schueller
12   University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Martin Forster
13   Head and Neck Centre, University College London Hospitals NHS Trust, London, United Kingdom
,
Amrita Jay
14   Department of Histopathology, University College London Hospitals NHS Trust, London, United Kingdom
,
Cillian Forde
13   Head and Neck Centre, University College London Hospitals NHS Trust, London, United Kingdom
,
Jozsef Dudas
4   Department of Otorhinolaryngology-Head and Neck Surgery, Medical University of Innsbruck, Innsbruck, Austria
,
Herbert Riechelmann
4   Department of Otorhinolaryngology-Head and Neck Surgery, Medical University of Innsbruck, Innsbruck, Austria
,
R. Peter Manes
15   Division of Otolaryngology, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, United States
,
Wendell G. Yarbrough
16   Department of Otolaryngology/Head and Neck Surgery and Department of Pathology, University of North Carolina, Chapel Hill, North Carolina, United States
,
Jose L. Llorente
2   Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Instituto Universitario de Oncología del Principado de Asturias (IUOPA), Centro de Investigación Biomédica en Red (CIBER-ONC), Oviedo, Spain
,
Sarah K. Wise
5   Department of Otorhinolaryngology-Head and Neck Surgery, Emory University, Atlanta, Georgia, United States
,
Paolo Castelnuovo
3   Unit of Otorhinolaryngology - Head & Neck Surgery, University of Insubria, Varese, Italy
,
Valerie J. Lund
17   Royal National Throat, Nose and Ear Hospital and Head and Neck Centre, University College London Hospitals NHS Trust, London, United Kingdom
› Author Affiliations
Further Information

Publication History

Publication Date:
05 February 2020 (online)

Also available at
 
 

Background and Objectives: Esthesioneuroblastoma is a rare nasal neoplasm which usually originates from the olfactory neuroepithelium in the upper portion of the nasal cavity adjacent to the cribriform plate.[1] [2] [3] Our objectives were to analyze multicenter clinical data from esthesioneuroblastoma 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 113 esthesioneuroblastoma patients (54 males; mean age: 49.4 years) from various centers in the United States 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 (77.4%), rhinorrhea (40.7%), epistaxis (39%), anosmia (30%), headache (13.8%), epiphora (5%), and diplopia (1.3%). With regard to age at presentation, we did not observe a bimodal distribution, but a peak between 37 and 58 years of age. The most useful poor prognostic indicators were bony skull base involvement and dural infiltration, each associated with significantly worse outcomes (p = 0.024 and p < 0.001, respectively). A total of 86.6% of patients received adjuvant radiotherapy and had a significantly improved survival (p = 0.012). Mean follow-up was 64.7 (±51.6) months. 5-year overall survival was 88.2%. The most common location for recurrent/residual disease was the neck, followed by dura/brain. 83% of samples stained positive for SSTR2 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 esthesioneuroblastoma patients present with anosmia. Our analysis also shows that the most significant prognostic indicators in our set of samples are bony skull base involvement and 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.


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No conflict of interest has been declared by the author(s).

  • References

  • Lund VJ, Howard D, Wei W, Spittle M. Olfactory neuroblastoma: past, present, and future?. Laryngoscope 2003; 113 (03) 502-507
    CrossrefPubMedGoogle Scholar
  • Dulguerov P, Allal AS, Calcaterra TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2001; 2 (11) 683-690
    CrossrefPubMedGoogle Scholar
  • Rimmer J, Lund VJ, Beale T, Wei WI, Howard D. Olfactory neuroblastoma: a 35-year experience and suggested follow-up protocol. Laryngoscope 2014; 124 (07) 1542-1549
    CrossrefPubMedGoogle Scholar

  • References

  • Lund VJ, Howard D, Wei W, Spittle M. Olfactory neuroblastoma: past, present, and future?. Laryngoscope 2003; 113 (03) 502-507
    CrossrefPubMedGoogle Scholar
  • Dulguerov P, Allal AS, Calcaterra TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol 2001; 2 (11) 683-690
    CrossrefPubMedGoogle Scholar
  • Rimmer J, Lund VJ, Beale T, Wei WI, Howard D. Olfactory neuroblastoma: a 35-year experience and suggested follow-up protocol. Laryngoscope 2014; 124 (07) 1542-1549
    CrossrefPubMedGoogle Scholar