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

2021; Elsevier BV; Volume: 162; Linguagem: Inglês

10.1016/j.ejca.2021.09.046

1879-0852

Matt Lechner, Yoko Takahashi, Mario Turri‐Zanoni, Jacklyn Liu, Nicholas Counsell, Mario Hermsen, Raman Preet Kaur, Tianna Zhao, Murugappan Ramanathan, Volker Hans Schartinger, Oscar Emanuel, Sam Helman, Jordan Varghese, József Dudás, Herbert Riechelmann, Susanne Sprung, Johannes Haybaeck, David Howard, Nils W. Engel, Sarah Stewart, Laura Brooks, Jessica C. Pickles, Thomas S. Jacques, Tim R. Fenton, Luke Williams, Francis Vaz, Paul O’Flynn, Paul Stimpson, Shih‐Yu Wang, S. Alam Hannan, Samit Unadkat, Jonathan Hughes, Raghav C. Dwivedi, Cillian T. Forde, Premjit S. Randhawa, Simon Gane, J. Joseph, Peter Andrews, Gary Royle, Alessandro Franchi, Roberta Maragliano, Simonetta Battocchio, Helen Bewicke‐Copley, Christodoulos P. Pipinikas, Amy P. Webster, Chrissie Thirlwell, Debbie L. L. Ho, Andrew E. Teschendorff, Tianyu Zhu, Christopher D. Steele, Nischalan Pillay, Bart Vanhaesebroeck, Ahmed Mohyeldin, Juan C. 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, Silvia Nicolini, Ilaria Grassi, Alberto Bongiovanni, David Capper, Ulrich Schüller, Selvam Thavaraj, Ann Sandison, Pavol Šurda, Claire Hopkins, Marco Ferrari, Davide Mattavelli, Vittorio Rampinelli, Fabio Facchetti, Piero Nicolai, Paolo Bossi, Oswaldo A. Henriquez, Kelly R. Magliocca, C. Arturo Solares, Sarah K. Wise, José Luís Llorente, Zara M. Patel, Jayakar V. Nayak, Peter H. Hwang, Peter D. Lacy, Robbie Woods, James P. O’Neill, Amrita Jay, Dawn Carnell, Martin Förster, Masaru Ishii, Nyall R. London, Diana Bell, Gary L. Gallia, Paolo Castelnuovo, Stefano Severi, Valerie J. Lund, Ehab Y. Hanna,

Ocular Oncology and Treatments

Abstract 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]( 177 Lu-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.