RESUMEN
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.
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Estesioneuroblastoma Olfatorio , Neuroblastoma , Neoplasias Nasales , Estesioneuroblastoma Olfatorio/patología , Estesioneuroblastoma Olfatorio/terapia , Humanos , Cavidad Nasal/metabolismo , Cavidad Nasal/patología , Neuroblastoma/patología , Neoplasias Nasales/radioterapia , Tomografía de Emisión de Positrones , Radioisótopos , Cintigrafía , Receptores de Somatostatina/metabolismo , Estudios RetrospectivosRESUMEN
OBJECTIVES: We set out to create Consensus Guidelines, based on current evidence and relative risks of adverse effects and the costs of different treatments, which reflect the views of the British Rhinological Society (BRS) Council on where the use of biologics should be positioned within treatment pathways for CRSwNP, specifically in the setting of the National Health Service (NHS). DESIGN: An expert panel of 16 members was assembled. A review of the literature and evidence synthesis was undertaken and circulated to the panel. We used the RAND/UCLA methodology with a multi-step process to make recommendations on the use of biologics. SETTING: N/A. PARTICIPANTS: N/A. RESULTS: Recommendations were made, based on underlying disease severity, prior treatments and co-morbidities. A group of patients for whom biologics were considered an appropriate treatment option for CRSwNP was defined. CONCLUSIONS: Although biologics are not currently available for the treatment of CRSwNP, the BRS Council have defined a group of patients who have higher rates of "failure" with current treatment pathways, higher resource use and are more likely to suffer with uncontrolled symptoms. We would urge NICE to consider approval of biologics for such indications without applying further restrictions on use.
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Productos Biológicos/uso terapéutico , Terapia Biológica/normas , Pólipos Nasales/terapia , Rinitis/terapia , Sinusitis/terapia , Enfermedad Crónica , Inglaterra , Humanos , Medicina EstatalRESUMEN
OBJECTIVES: The primary aim of the study is to provide recommendations for the investigation and management of patients with new onset loss of sense of smell during the COVID-19 pandemic. DESIGN: After undertaking a literature review, we used the RAND/UCLA methodology with a multi-step process to reach consensus about treatment options, onward referral, and imaging. SETTING AND PARTICIPANTS: An expert panel consisting of 15 members was assembled. A literature review was undertaken prior to the study and evidence was summarised for the panellists. MAIN OUTCOME MEASURES: The panel undertook a process of ranking and classifying appropriateness of different investigations and treatment options for new onset loss of sense of smell during the COVID-19 pandemic. Using a 9-point Likert scale, panellists scored whether a treatment was: Not recommended, optional, or recommended. Consensus was achieved when more than 70% of responses fell into the category defined by the mean. RESULTS: Consensus was reached on the majority of statements after 2 rounds of ranking. Disagreement meant no recommendation was made regarding one treatment, using Vitamin A drops. Alpha-lipoic acid was not recommended, olfactory training was recommended for all patients with persistent loss of sense of smell of more than 2 weeks duration, and oral steroids, steroid rinses, and omega 3 supplements may be considered on an individual basis. Recommendations regarding the need for referral and investigation have been made. CONCLUSION: This study identified the appropriateness of olfactory training, different medical treatment options, referral guidelines and imaging for patients with COVID-19-related loss of sense of smell. The guideline may evolve as our experience of COVID-19 develops.