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

Oswaldo, Henriquez Ajami, Emory University; Clementino, Solares Rivera, Emory University; Matt, Lechner, Stanford University; Yoko, Takahashi, The University of Texas MD Anderson Cancer Center; Mario, Turri-Zanoni, University of Insubria; Jacklyn, Liu, University College London; Nicholas, Counsell, Cancer Research UK & UCL Cancer Trials Centre; Mario, Hermsen, Centro de Investigación Biomédica en Red (CIBER-ONC), Oviedo, Spain; Raman Preet, Kaur, Johns Hopkins School of Medicine; Tianna, Zhao, Johns Hopkins University; Murugappan, Ramanathan Jr, Johns Hopkins School of Medicine; Volker H, Schartinger, Medical University of Innsbruck; Oscar, Emanuel, University College London; Sam, Helman, Emory University; Jordan, Varghese, Emory University; Jozsef, Dudas, Medical University of Innsbruck; Herbert, Riechelmann, Medical University of Innsbruck; Susanne, Sprung, Medical University of Innsbruck; Johannes, Haybaeck, Medical University of Innsbruck; David, Howard, Imperial College Healthcare NHS Trust, London; Nils Wolfgang, Engel, University Medical Center Hamburg-Eppendorf; Sarah, Stewart, University College London Hospitals NHS Trust; Laura, Brooks, University College London Hospitals NHS Trust; Jessica C, Pickles, Great Ormond Street Hospital for Children NHS Foundation Trust; Thomas S, Jacques, Great Ormond Street Hospital for Children NHS Foundation Trust; Tim, Fenton, University of Kent; Luke, Williams, University College London; Francis M, Vaz, University College London Hospitals NHS Trust; Paul, O'Flynn, University College London Hospitals NHS Trust; Paul, Stimpson, University College London Hospitals NHS Trust; Simon, Wang, University College London; Alam S, Hannan, University College London Hospitals NHS Trust; Samit, Unadkat, University College London Hospitals NHS Trust; Jonathan, Hughes, University College London Hospitals NHS Trust; Raghav, Dwivedi, University College London Hospitals NHS Trust; Cillian T, Forde, University College London Hospitals NHS Trust; Premjit, Randhawa, University College London Hospitals NHS Trust; Simon, Gane, UCL Great Ormond St Inst Child Hlth; Jonathan, Joseph, University College London Hospitals NHS Trust; Peter J, Andrews, University College London Hospitals NHS Trust; Gary, Royle, University College London; Alessandro, Franchi, University of Pisa; Roberta, Maragliano, University of Insubria; Simonetta, Battocchio, University of Brescia; Helen, Bewicke-Copley, University College London; Christodoulos, Pipinikas, University College London; Amy, Webster, University College London; Chrissie, Thirlwell, University College London; Debbie, Ho, University of Cambridge; Andrew, Teschendorff, University of Chinese Academy of Sciences; Tianyu, Zhu, University of Chinese Academy of Sciences; Christopher D, Steele, Johns Hopkins University; Nischalan, Pillay, University College London; Bart, Vanhaesebroeck, University College London; Ahmed, Mohyeldin, Stanford University; Juan, Fernandez-Miranda, Stanford University; Ki Wan, Park, Stanford University; Quynh-Thu, Le, Stanford University; Robert B, West, Stanford University; Rami, Saade, The University of Texas MD Anderson Cancer Center; Peter R, Manes, Yale School of Medicine; Sacit B, Omay, Yale School of Medicine; Eugenia M, Vining, Yale School of Medicine; Benjamin L, Judson, Yale School of Medicine; Wendell G, Yarbrough, UNC Sch Med; Maddalena, Sansovini, IRCCS Ist Romagnolo Studio Tumori IRST Dino Amado; Nicolini, Silvia, IRCCS Ist Romagnolo Studio Tumori IRST Dino Amado; Ilaria, Grassi, IRCCS Ist Romagnolo Studio Tumori IRST Dino Amado; Alberto, Bongiovanni, IRCCS Ist Romagnolo Studio Tumori IRST Dino Amado; David, Capper, Charite Univ Med Berlin; Ulrich, Schueller, Univ Med Ctr Hamburg Eppendorf; Selvam, Thavaraj, Kings College London; Ann, Sandison, Guy's Hospital, London; Pavol, Surda, Guy’s Hospital, King’s College, London; Claire, Hopkins, .Guy’s Hospital, King’s College, London; Marco, Ferrari, University of Padua; Davide, Mattavelli, University of Brescia; Vittorio, Rampinelli, University of Brescia; Fabio, Facchetti, University of Brescia; Piero, Nicolai, University of Padua; Paolo, Bossi, University of Brescia; Oswaldo A, Henriquez, Emory University; Kelly, Magliocca, Emory University; Arturo C, Solares, Emory University; Sarah, Wise, Emory University; Jose L, Llorente, Hospital Universitario Central de Asturias; Zara M, Patel, Stanford University; Jayakar, V. Nayak, Stanford University; Peter H, Hwang, Stanford University; Peter D, Lacy, Beaumont Hospital; Robbie, Woods, Beaumont Hospital; James P, O'Neill, Beaumont Hospital; Amrita, Jay, University College London Hospitals NHS Trust; Dawn, Carnell, University College London Hospitals NHS Trust; Martin D, Forster, University College London; Masaru, Ishii, Johns Hopkins School of Medicine; Nyall R, London Jr, Johns Hopkins School of Medicine; Diana M, Bell, University of Texas MD Anderson Cancer Center Houston; Gary L, Gallia, Johns Hopkins School of Medicine; Paolo, Castelnuovo, University of Insubria; Stefano, Severi, IRCCS-Istituto Romagnolo per lo Studio dei Tumori “Dino Amadori” IRST, Meldola FC, Italy; Valerie J, Lund, University College London Hospitals NHS Trust; Ehab Y, Hanna, The University of Texas MD Anderson Cancer Center

Publication

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

Persistent URL

https://open.library.emory.edu/purl/750tqjq3gh-emory

Last modified

08/18/2025

Type of Material

Article

Authors

Oswaldo Henriquez Ajami, Emory University

Clementino Solares Rivera, Emory University

Matt Lechner, Stanford University

Yoko Takahashi, The University of Texas MD Anderson Cancer Center

Mario Turri-Zanoni, University of Insubria

Jacklyn Liu, University College LondonNicholas Counsell, Cancer Research UK & UCL Cancer Trials CentreMario Hermsen, Centro de Investigación Biomédica en Red (CIBER-ONC), Oviedo, SpainRaman Preet Kaur, Johns Hopkins School of MedicineTianna Zhao, Johns Hopkins UniversityMurugappan Ramanathan Jr, Johns Hopkins School of MedicineVolker H Schartinger, Medical University of InnsbruckOscar Emanuel, University College LondonSam Helman, Emory UniversityJordan Varghese, Emory UniversityJozsef Dudas, Medical University of InnsbruckHerbert Riechelmann, Medical University of InnsbruckSusanne Sprung, Medical University of InnsbruckJohannes Haybaeck, Medical University of InnsbruckDavid Howard, Imperial College Healthcare NHS Trust, LondonNils Wolfgang Engel, University Medical Center Hamburg-EppendorfSarah Stewart, University College London Hospitals NHS TrustLaura Brooks, University College London Hospitals NHS TrustJessica C Pickles, Great Ormond Street Hospital for Children NHS Foundation TrustThomas S Jacques, Great Ormond Street Hospital for Children NHS Foundation TrustTim Fenton, University of KentLuke Williams, University College LondonFrancis M Vaz, University College London Hospitals NHS TrustPaul O'Flynn, University College London Hospitals NHS TrustPaul Stimpson, University College London Hospitals NHS TrustSimon Wang, University College LondonAlam S Hannan, University College London Hospitals NHS TrustSamit Unadkat, University College London Hospitals NHS TrustJonathan Hughes, University College London Hospitals NHS TrustRaghav Dwivedi, University College London Hospitals NHS TrustCillian T Forde, University College London Hospitals NHS TrustPremjit Randhawa, University College London Hospitals NHS TrustSimon Gane, UCL Great Ormond St Inst Child HlthJonathan Joseph, University College London Hospitals NHS TrustPeter J Andrews, University College London Hospitals NHS TrustGary Royle, University College LondonAlessandro Franchi, University of PisaRoberta Maragliano, University of InsubriaSimonetta Battocchio, University of BresciaHelen Bewicke-Copley, University College LondonChristodoulos Pipinikas, University College LondonAmy Webster, University College LondonChrissie Thirlwell, University College LondonDebbie Ho, University of CambridgeAndrew Teschendorff, University of Chinese Academy of SciencesTianyu Zhu, University of Chinese Academy of SciencesChristopher D Steele, Johns Hopkins UniversityNischalan Pillay, University College LondonBart Vanhaesebroeck, University College LondonAhmed Mohyeldin, Stanford UniversityJuan Fernandez-Miranda, Stanford UniversityKi Wan Park, Stanford UniversityQuynh-Thu Le, Stanford UniversityRobert B West, Stanford UniversityRami Saade, The University of Texas MD Anderson Cancer CenterPeter R Manes, Yale School of MedicineSacit B Omay, Yale School of MedicineEugenia M Vining, Yale School of MedicineBenjamin L Judson, Yale School of MedicineWendell G Yarbrough, UNC Sch MedMaddalena Sansovini, IRCCS Ist Romagnolo Studio Tumori IRST Dino AmadoNicolini Silvia, IRCCS Ist Romagnolo Studio Tumori IRST Dino AmadoIlaria Grassi, IRCCS Ist Romagnolo Studio Tumori IRST Dino AmadoAlberto Bongiovanni, IRCCS Ist Romagnolo Studio Tumori IRST Dino AmadoDavid Capper, Charite Univ Med BerlinUlrich Schueller, Univ Med Ctr Hamburg EppendorfSelvam Thavaraj, Kings College LondonAnn Sandison, Guy's Hospital, LondonPavol Surda, Guy’s Hospital, King’s College, LondonClaire Hopkins, .Guy’s Hospital, King’s College, LondonMarco Ferrari, University of PaduaDavide Mattavelli, University of BresciaVittorio Rampinelli, University of BresciaFabio Facchetti, University of BresciaPiero Nicolai, University of PaduaPaolo Bossi, University of BresciaOswaldo A Henriquez, Emory UniversityKelly Magliocca, Emory UniversityArturo C Solares, Emory UniversitySarah Wise, Emory UniversityJose L Llorente, Hospital Universitario Central de AsturiasZara M Patel, Stanford UniversityJayakar V. Nayak, Stanford UniversityPeter H Hwang, Stanford UniversityPeter D Lacy, Beaumont HospitalRobbie Woods, Beaumont HospitalJames P O'Neill, Beaumont HospitalAmrita Jay, University College London Hospitals NHS TrustDawn Carnell, University College London Hospitals NHS TrustMartin D Forster, University College LondonMasaru Ishii, Johns Hopkins School of MedicineNyall R London Jr, Johns Hopkins School of MedicineDiana M Bell, University of Texas MD Anderson Cancer Center HoustonGary L Gallia, Johns Hopkins School of MedicinePaolo Castelnuovo, University of InsubriaStefano Severi, IRCCS-Istituto Romagnolo per lo Studio dei Tumori “Dino Amadori” IRST, Meldola FC, ItalyValerie J Lund, University College London Hospitals NHS TrustEhab Y Hanna, The University of Texas MD Anderson Cancer Center

Language

English

Date

2022-02-01

Publisher

ELSEVIER SCI LTD

Publication Version

Author Accepted Manuscript (After Peer Review)

Copyright Statement

License

Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International

Final Published Version (URL)

http://dx.doi.org/10.1016/j.ejca.2021.09.046

Title of Journal or Parent Work

EUROPEAN JOURNAL OF CANCER

Volume

Start Page

End Page

Grant/Funding Information

This work was supported by the Rhinology and Laryngology Research Fund, Royal College of Surgeons (Lionel Colledge Family Research Grant) and the UCL/UCLH Biomedical Research Centre (BRC). Additional support was provided by the Intramural Research Program of the National Institutes of Health/National Institute on Deafness and Other Communication Disorders.

Supplemental Material (URL)

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9554673/bin/NIHMS1838753-supplement-Supplemental_Material.pdf

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](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.

Author Notes

Matt Lechner MD PHD, mlechner@stanford.edu

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