Dataset Information

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

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.

SUBMITTER: Lechner M

PROVIDER: S-EPMC9554673 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

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

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

European journal of cancer (Oxford, England : 1990) 20211231

<h4>Introduction</h4>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.<h4>Methods</h4>We conducted a retrospective analysis of 404 primary, locally recurrent, and metastatic olfactory neuroblastoma (ONB) patients from 12 institutions in the United States o ...[more]

PMID: 34980502

Similar Datasets

Project description:ImportanceCurrent olfactory neuroblastoma (ONB) staging systems inadequately delineate locally advanced tumors, do not incorporate tumor grade, and poorly estimate survival and recurrence.ObjectiveThe primary aims of this study were to (1) examine the clinical covariates associated with survival and recurrence of ONB in a modern-era multicenter cohort and (2) incorporate Hyams tumor grade into existing staging systems to assess its ability to estimate survival and recurrence.Design, setting, and participantsThis retrospective, multicenter, case-control study included patients with ONB who underwent treatment between January 1, 2005, and December 31, 2021, at 9 North American academic medical centers.InterventionStandard-of-care ONB treatment.Main outcome and measuresThe main outcomes were overall survival (OS), disease-free survival (DFS), and disease-specific survival (DSS) as C statistics for model prediction.ResultsA total of 256 patients with ONB (mean [SD] age, 52.0 [15.6] years; 115 female [44.9%]; 141 male [55.1%]) were included. The 5-year rate for OS was 83.5% (95% CI, 78.3%-89.1%); for DFS, 70.8% (95% CI, 64.3%-78.0%); and for DSS, 94.1% (95% CI, 90.5%-97.8%). On multivariable analysis, age, American Joint Committee on Cancer (AJCC) stage, involvement of bilateral maxillary sinuses, and positive margins were associated with OS. Only AJCC stage was associated with DFS. Only N stage was associated with DSS. When assessing the ability of staging systems to estimate OS, the best-performing model was the novel modification of the Dulguerov system (C statistic, 0.66; 95% CI, 0.59-0.76), and the Kadish system performed most poorly (C statistic, 0.57; 95% CI, 0.50-0.63). Regarding estimation of DFS, the modified Kadish system performed most poorly (C statistic, 0.55; 95% CI, 0.51-0.66), while the novel modification of the AJCC system performed the best (C statistic, 0.70; 95% CI, 0.66-0.80). Regarding estimation of DSS, the modified Kadish system was the best-performing model (C statistic, 0.79; 95% CI, 0.70-0.94), and the unmodified Kadish performed the worst (C statistic, 0.56; 95% CI, 0.51-0.68). The ability for novel ONB staging systems to estimate disease progression across stages was also assessed. In the novel Kadish staging system, patients with stage VI disease were approximately 7 times as likely to experience disease progression as patients with stage I disease (hazard ratio [HR], 6.84; 95% CI, 1.60-29.20). Results were similar for the novel modified Kadish system (HR, 8.99; 95% CI, 1.62-49.85) and the novel Dulguerov system (HR, 6.86; 95% CI, 2.74-17.18).Conclusions and relevanceThe study findings indicate that 5-year OS for ONB is favorable and that incorporation of Hyams grade into traditional ONB staging systems is associated with improved estimation of disease progression.

Project description:BackgroundMediastinal neuroblastoma (NB) can invade the spinal canal and result in spinal cord compression. Some patients go on to develop severe spinal deformities after decompression of the spinal cord. The optimal therapeutic strategy for mediastinal NB with intraspinal extension is still unclear. Our study is to assess the therapeutic strategies for such patients.MethodsA total of 77 patients suffered mediastinal tumors with intraspinal extension between March 2015 and Aug 2019 were enrolled in the study. According to the primary therapy, NB were classified into 4 groups: chemotherapy, video-assisted thoracoscopic surgery (VATS)/thoracotomy, neurosurgical decompression, and a combined thoracic-neurosurgical approach. Clinical features, including patient demographics, neurologic recovery and survival rate, were assessed.ResultsAmong the 77 patients suffered mediastinal tumors with intraspinal extension, neurological symptoms were present in 44 patients. Neurological deficits improved in 76.5% of patients who underwent neurosurgical intervention and 50% of the other patients (P=0.094). Compression manifestations of ≤4 weeks duration showed an improved outcome compared to a longer compression time, with complete recovery of neurological function in 60% of patients versus 28.6% for patients with a longer symptom duration (P=0.04). NB constituted 49.4% of the 77 patients. An overall survival rate of 90.0%±9.5% was achieved for patients in the combined thoracic-neurosurgical group, 59.5%±15.0% in the thoracotomy group, 40.0%±29.7% in laminectomy group, and 37.0%±20.2% in the chemotherapy group. Complete regression of the tumor was demonstrated in 80% of combined group, which was greater than that of patients in the other groups (P=0.001).ConclusionsNeurological recovery was correlated with the type of initial treatment and the duration of neurological symptoms. Mediastinal NB with intraspinal extension can be effectively managed with a combined neurosurgical and thoracic surgical approach.

Dataset Information

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

Introduction

Methods

Results

Conclusions

Publications

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

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets