Clinical outcomes, Kadish-INSICA staging and therapeutic targeting of somatostatin receptor 2 in olfactory neuroblastoma

Matt Lechner, Yoko Takahashi, Mario Turri-Zanoni, Jacklyn Liu, Nicholas Counsell, Mario Hermsen, Raman Preet Kaur, Tianna Zhao, Murugappan Ramanathan, Volker H. Schartinger, Oscar Emanuel, Sam Helman, Jordan Varghese, Jozsef Dudas, Herbert Riechelmann, Susanne Sprung, Johannes Haybaeck, David Howard, Nils Wolfgang Engel, Sarah StewartLaura Brooks, Jessica C. Pickles, Thomas S. Jacques, Tim R. Fenton, Luke Williams, Francis M. Vaz, Paul O'Flynn, Paul Stimpson, Simon Wang, S. Alam Hannan, Samit Unadkat, Jonathan Hughes, Raghav Dwivedi, Cillian T. Forde, Premjit Randhawa, Simon Gane, Jonathan Joseph, Peter J. Andrews, Gary Royle, Alessandro Franchi, Roberta Maragliano, Simonetta Battocchio, Helen Bewicke-Copley, Christodoulos Pipinikas, Amy Webster, Chrissie Thirlwell, Debbie Ho, Andrew Teschendorff, Tianyu Zhu, Christopher D. Steele, Nischalan Pillay, Bart Vanhaesebroeck, Ahmed Mohyeldin, Juan Fernandez-Miranda, Ki Wan Park, Quynh Thu Le, Robert B. West, Rami Saade, R. Peter Manes, Sacit Bulent Omay, Eugenia M. Vining, Benjamin L. Judson, Wendell G. Yarbrough, Maddalena Sansovini, Nicolini Silvia, Ilaria Grassi, Alberto Bongiovanni, David Capper, Ulrich Schüller, Selvam Thavaraj, Ann Sandison, Pavol Surda, Claire Hopkins, Marco Ferrari, Davide Mattavelli, Vittorio Rampinelli, Fabio Facchetti, Piero Nicolai, Paolo Bossi, Oswaldo A. Henriquez, Kelly Magliocca, C. Arturo Solares, Sarah K. Wise, Jose L. Llorente, Zara M. Patel, Jayakar V. Nayak, Peter H. Hwang, Peter D. Lacy, Robbie Woods, James P. O'Neill, Amrita Jay, Dawn Carnell, Martin D. Forster, Masaru Ishii, Nyall R. London, Diana M. Bell, Gary L. Gallia, Paolo Castelnuovo, Stefano Severi, Valerie J. Lund, Ehab Y. Hanna

Research output: Contribution to journalArticlepeer-review


Introduction: Olfactory neuroblastoma (ONB) is a rare cancer of the sinonasal region. We provide a comprehensive analysis of this malignancy with molecular and clinical trial data on a subset of our cohort to report on the potential efficacy of somatostatin receptor 2 (SSTR2)-targeting imaging and therapy. Methods: We conducted a retrospective analysis of 404 primary, locally recurrent, and metastatic olfactory neuroblastoma (ONB) patients from 12 institutions in the United States of America, United Kingdom and Europe. Clinicopathological characteristics and treatment approach were evaluated. SSTR2 expression, SSTR2-targeted imaging and the efficacy of peptide receptor radionuclide therapy [PRRT](177Lu-DOTATATE) were reported in a subset of our cohort (LUTHREE trial; NCT03454763). Results: Dural infiltration at presentation was a significant predictor of overall survival (OS) and disease-free survival (DFS) in primary cases (n = 278). Kadish-Morita staging and Dulguerov T-stage both had limitations regarding their prognostic value. Multivariable survival analysis demonstrated improved outcomes with lower stage and receipt of adjuvant radiotherapy. Prophylactic neck irradiation significantly reduces the rate of nodal recurrence. 82.4% of the cohort were positive for SSTR2; treatment of three metastatic cases with SSTR2-targeted peptide-radionuclide receptor therapy (PRRT) in the LUTHREE trial was well-tolerated and resulted in stable disease (SD). Conclusions: This study presents pertinent clinical data from the largest dataset, to date, on ONB. We identify key prognostic markers and integrate these into an updated staging system, highlight the importance of adjuvant radiotherapy across all disease stages, the utility of prophylactic neck irradiation and the potential efficacy of targeting SSTR2 to manage disease.

Original languageEnglish (US)
Pages (from-to)221-236
Number of pages16
JournalEuropean Journal of Cancer
StatePublished - Feb 2022


  • Adjuvant
  • Diagnostic imaging
  • Esthesioneuroblastoma
  • Human
  • Olfactory
  • Prognosis
  • Radiotherapy
  • Receptors
  • SSTR2 protein
  • Somatostatin

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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