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Ann Otol Rhinol Laryngol 2020 Jul 4:3489420939582. Epub 2020 Jul 4.

Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, NJ, USA.

Background: Olfactory neuroblastoma, or esthesioneuroblastoma (ENB), is an uncommon sinonasal malignancy arising from olfactory neuroepithelium that is optimally treated with surgical resection. The objective of this study is to determine preoperative predictors of undergoing open versus endoscopic approach for ENB and to investigate significant postoperative survival outcomes between the two surgical approaches.

Methods: The National Cancer Database (NCDB) was queried for cases of ENB histology that were treated surgically from 2010 to 2015. Read More

Clin Nucl Med 2020 Aug;45(8):e363-e364

The Persian Gulf Nuclear Medicine Research Center, Department of Molecular Imaging and Radionuclide Therapy, Bushehr Medical University Hospital, Bushehr University of Medical Sciences, Bushehr, Iran.

We present a 48-year-old woman with an olfactory neuroblastoma who was referred for accurate staging using PET/CT. The Ga-DOTATATE PET/CT showed a 51 × 32-mm mass with an SUVmax of 7.59 in the sphenoidal sinuses, whereas radiotracer uptake on F-FDG PET/CT was similar to that of brain tissue. Read More

Int Forum Allergy Rhinol 2020 Jun 18. Epub 2020 Jun 18.

Department of Otorhinolaryngology, Head and Neck Surgery, Rush Center for Skull Base and Pituitary Surgery, Rush University Medical Center, Chicago, IL.

Background: Olfactory neuroblastoma (ONB) is a rare skull-base malignancy associated with delayed local recurrence. Treatment options in recurrent disease are few and unreliable. We undertook analysis of the ONB exome and immune environment in order to identify potential future immunotherapy treatment options. Read More

J Neurooncol 2020 May 18. Epub 2020 May 18.

Department of Neurosurgery, The University of Texas M.D. Anderson Cancer Center, Houston, TX, USA.

Introduction: Sinonasal tumors that harbor neuroendocrine histologic features include olfactory neuroblastoma (previously known as esthesioneuroblastoma), sinonasal neuroendocrine carcinoma, and sinonasal undifferentiated carcinoma. These tumors represent a diverse spectrum of clinical behavior and as such require histology-specific management. Herein, we review the management of these sinonasal tumors with neuroendocrine features and discuss fundamentals of multi-modality care for each histology. Read More

Ann R Coll Surg Engl 2020 Jul 6;102(6):e118-e121. Epub 2020 May 6.

Ear, Nose and Throat Department, Royal Surrey County Hospital, Guildford, Surrey, UK.

Tumour-to-tumour metastasis is a rare phenomenon. It occurs when a primary tumour is a recipient of a separate tumour within the same individual. We present a case of a 66-year-old woman with known breast cancer who presented with one-sided nasal symptoms. Read More

J Neurosurg Sci 2020 Apr 29. Epub 2020 Apr 29.

Department of Neurosurgery, Bangabandhu Sheikh Mujib Medical University, Dacca, Bangladesh.

Introduction: To report an extremely rare case of sellar neuroblastoma, which mimicked invasive pituitary adenoma with supra- and parasellar extensions, treated through endoscopic endonasal approach. A systematic review of the literature on diagnosis and management of patients affected by sellar neuroblastomas has been performed.

Evidence Acquiition: A literature search according to the PRISMA statement was conducted using MEDLINE, Google Scholar, and EBSCO databases, searching for the following MeSH terms: (sellar OR parasellar OR suprasellar OR sphenoidal) AND (neuroblastoma OR extranasal esthesioneuroblastoma OR central neuroblastoma). Read More

Otolaryngol Head Neck Surg 2020 Apr 14:194599820915487. Epub 2020 Apr 14.

SUNY Upstate Medical University, Syracuse, New York, USA.

Objective: To compare the Kadish and the modified Dulguerov staging of individual participants to determine the impact of stage and other prognostic factors on disease-free (DFS) and overall survival (OS).

Data Sources: Systematic review of EMBASE, MEDLINE, Cochrane Library, and CINAHL databases.

Review Methods: The Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) was followed for this study. Read More

Zh Vopr Neirokhir Im N N Burdenko 2020 ;84(1):101-108

Burdenko Neurosurgical Center, Moscow, Russia.

Sinonasal malignant tumors are characterized by high histological variability and complexity of the differential diagnosis. Currently, there are classifications of these tumors, which are based on their localization and involvement of various anatomical structures. However, generally accepted algorithms for treatment of this pathology have not yet been developed. Read More

Yonsei Med J 2020 Mar;61(3):257-261

Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea.

Esthesioneuroblastoma as a source of ectopic Cushing's syndrome is rare, and to the best of our knowledge, only 20 cases have been reported worldwide. A 46-year-old healthy man visited a local clinic for general weakness and hyposmia, and underwent examination with serial endocrinological workup and brain imaging. Gallium-DOTA-TOC positron emission tomography scan was helpful where diagnosis of sellar MRI and inferior petrosal sinus sampling were discordant. Read More

Childs Nerv Syst 2020 Feb 26. Epub 2020 Feb 26.

Department of Otolaryngology, University of Pittsburgh, Pittsburgh, PA, USA.

Esthesioneuroblastomas are uncommon tumors in pediatric patients and are typically treated with multimodal therapy. Changes in gross tumor quality and character in response to adjuvant treatment have not been clearly reported. We report the case of a 15-year-old female with a diagnosis of Kadish stage C esthesioneuroblastoma who was treated with neoadjuvant chemotherapy and surgical resection. Read More

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2020 Feb;34(2):128-130

Department of Otolaryngology Head and Neck Surgery，First Affiliated Hospital of Chinese Academy of Medical Sciences，Hefei，230001，China.

The aim of this study is to evaluate the efficacy of endoscopic surgery and conventional surgery combined with radiotherapy in the treatment of Neuroblastoma. Forty-three patients with olfactory neuroblastoma undergoing surgery combined with radiotherapy were retrospectively analyzed. The patients were divided into endoscopic surgery and conventional surgery. Read More

Dis Aquat Organ 2020 02;138:29-33

Vancouver Aquarium and Marine Science Centre, Vancouver, BC V6G 3E2, Canada.

One spotted pike-characin Boulengerella maculata, managed in a public display aquarium for 7 yr, developed a small unilateral pink growth associated with the right nare. The growth eventually extended through both nares after 5 mo. B. Read More

J Nucl Med 2020 Jan 31. Epub 2020 Jan 31.

Center for Cancer Imaging, Peter MacCallum Cancer Center, Australia.

Esthesioneuroblastoma (ENB) is rare with limited therapeutic options when unresectable or metastatic; however, expression of somatostatin receptors qualifies it for peptide receptor radionuclide therapy (PRRT). We report outcomes of PRRT in ENB from two referral centers. Using PRRT databases at two European Neuroendocrine Tumour Society Centers of Excellence, case finding was undertaken between 2004-2018 for patients who had PRRT with recurrent/metastatic ENB deemed unsuitable for further conventional therapies. Read More

Radiol Case Rep 2020 Apr 20;15(4):330-333. Epub 2020 Jan 20.

Department of Pathology and Laboratory Medicine, University of North Carolina Hospitals, Women and Children Hospital, Chapel Hill, NC, USA.

Pulmonary metastasis of an esthesioneuroblastoma is uncommon. In this report, we present a case of an esthesioneuroblastoma pulmonary metastasis in an adult. A 39-year-old man treated with surgical resection of olfactory neuroblastoma was found to have a solitary pulmonary mass on a surveillance computed tomography scan performed 5 years after undergoing primary surgery and radiation treatment. Read More

Childs Nerv Syst 2020 Mar 27;36(3):485-489. Epub 2020 Jan 27.

Department of Neurosciences, Section of Otolaryngology and Regional Centre for Head and Neck Cancer, University of Padova, Padua, Italy.

Background: Olfactory neuroblastoma, also called esthesioneuroblastoma (ONB), is a rare neuroectodermal neoplasm that originates from the olfactory epithelium of the nose-sinus tract. It generally occurs with epistaxis, nasal obstruction, diplopia, and anosmia.

Methods: A 16-year-old female was admitted to our Unit with a complaint of nasal obstruction, recurrent epistaxis, anosmia, and intermittent headache of sixth month's duration. Read More

Pediatr Blood Cancer 2020 04 13;67(4):e28154. Epub 2020 Jan 13.

SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Institut Curie, PSL University, Paris, France.

Background: Esthesioneuroblastoma (ENB) is a rare neuroectodermal tumor that seldom occurs during childhood. Multimodal treatments are currently proposed, but the place of each therapy is still in debate. Our objective is to describe clinical evolution, especially the pattern of relapses and determine contributors to tumor progression. Read More

Am J Clin Oncol 2020 Mar;43(3):203-209

Department of Radiation Oncology.

Objective: Chemotherapy has been proposed as an adjunct to primary local therapy in esthesioneuroblastoma (ENB)/olfactory neuroblastoma (ON), but its role has not been precisely defined. Here, we evaluated its role in ENB treatment.

Materials And Methods: The Surveillance Epidemiology and End Results (SEER) database was queried for ENB/ON (International Classification of Diseases-3 9522). Read More

World Neurosurg 2020 Mar 27;135:e187-e193. Epub 2019 Nov 27.

Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. Electronic address:

Background: Programmed cell death ligand 1 (PD-L1) is a transmembrane glycoprotein that interacts with the receptor programmed cell death 1 (PD-1) to suppress T-cell activation, reduce adjacent tissue damage, and promote tolerance to self-antigens. Tumors may express PD-L1 as a mechanism to evade immune detection. Recent clinical trials have demonstrated the efficacy of PD-L1/PD-1 antagonists through activation of tumor-infiltrated CD8 T cells. Read More

World Neurosurg 2020 Feb 14;134:361-364. Epub 2019 Nov 14.

Department of Neurological Surgery, The Ohio State University, Wexner Medical Center, Columbus, Ohio, USA; Department of Head and Neck Surgery, The Ohio State University, Wexner Medical Center, Columbus, Ohio, USA. Electronic address:

Background: Olfactory neuroblastoma (ON) is a highly aggressive and locally recurrent neoplasm. Distant systemic metastases are not uncommon, but remote leptomeningeal dissemination is extremely rare.

Case Description: We report 2 cases of ON previously treated with endoscopic endonasal radical surgical resection and radiotherapy. Read More

Endocrinol Diabetes Metab Case Rep 2019 Oct 17;2019. Epub 2019 Oct 17.

Department of Diabetology-Endocrinology, AZ Nikolaas, Sint-Niklaas, Belgium.

Summary: ACTH-dependent hypercortisolism is caused by an ectopic ACTH syndrome (EAS) in 20% of cases. We report a rare cause of EAS in a 41-year-old woman, presenting with clinical features of Cushing's syndrome which developed over several months. Biochemical tests revealed hypokalemic metabolic alkalosis and high morning cortisol and ACTH levels. Read More

OTO Open 2018 Jul-Sep;2(3):2473974X18792488. Epub 2018 Aug 2.

St Paul's Sinus Centre, Division of Otolaryngology, University of British Columbia, Vancouver, Canada.

Case Rep Oncol Med 2019 19;2019:3789317. Epub 2019 Aug 19.

Medical Oncology Department, Instituto Português de Oncologia do Porto Francisco Gentil (IPO-PORTO), Porto, Portugal.

Esthesioneuroblastoma (ENB) is a rare malignant tumor that commonly develops in the upper nasal cavity. Standard treatment is not established, especially in locally advanced disease which portends the worse prognosis. Hereby, we report a case of a 27-year-old, 23-week pregnant woman, with a 2-month history of progressively growing right cervical lymphadenopathy, nasal obstruction, anosmia, frequent episodes of epistaxis, and right frontal headache. Read More

Cureus 2019 Jun 13;11(6):e4897. Epub 2019 Jun 13.

Neurosurgery, King Faisal Specialist Hospital and Research Center, Riyadh, SAU.

Esthesioneuroblastoma (ENB) is a rare malignant tumor of the nasal cavity. The genetic basis of its development is still under study and has not been fully delineated. It has varying symptoms depending on the lesion's location within the nasal cavity. Read More

Head Neck Pathol 2019 Aug 6. Epub 2019 Aug 6.

Department of Cellular Pathology, John Radcliffe Hospital, Oxford, UK.

Olfactory neuroblastoma (ONB) is a rare malignant neoplasm arising from the superior aspect of the nasal vault. Cases are characterised by insidious clinical presentation and high rates of recurrence despite surgical resection and adjuvant radiotherapy. There are a small number of reports showing ONB with divergent epithelial or ganglionic differentiation, and ONB has also been found to coincide with adenocarcinoma. Read More

Int Forum Allergy Rhinol 2019 07;9(S3):S145-S365

University of Pittsburgh, Pittsburgh, PA.

Background: Endoscopic skull-base surgery (ESBS) is employed in the management of diverse skull-base pathologies. Paralleling the increased utilization of ESBS, the literature in this field has expanded rapidly. However, the rarity of these diseases, the inherent challenges of surgical studies, and the continued learning curve in ESBS have resulted in significant variability in the quality of the literature. Read More

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2019 Jul;33(7):599-602

Department of Otolaryngology, Otorhinolaryngology Hospital, First Affiliated Hospital of Sun Yatsen University, Guangzhou, 510800, China.

Esthesioneuroblastoma(ENB) is a sinonasal rare tumor, and the assessment on the prognosis have not been used with on consensus, our study aims to set an accuracy indicator to predict the outcomes of ENB.A retrospective review was performed on 31 ENB patients. We collected 31 patients with ENB and reviewed the clinical data and pathological slides; modified Kadish stages were evaluated by otolaryngologist and imaging specialist; Hyams grading system were confirmed by two pathologists, who reviewed and paid attention to the pathological characteristics of Hyams grading system. Read More

Histopathology 2019 Dec 6;75(6):853-864. Epub 2019 Oct 6.

Department of Otolaryngology - Head and Neck Surgery, Assistance Publique-Hopitaux de Paris, Lariboisière Hospital, Paris, France.

Aims: Olfactory neuroblastomas (ONBs) are rare malignant tumours that arise in the nasal vault. To date, the Hyams grade remains the only widely used histological grading system. However, it is based only on morphological criteria, and has not been updated since 1988. Read More

Clin Nucl Med 2019 Aug;44(8):e486-e488

From the Departments of Nuclear Medicine.

Esthesioneuroblastoma is a rare neoplasm arising from the neural crest cells of olfactory epithelium mostly in the nasal vault. We describe the Ga DOTANOC PET/CT findings of a 44-year-old woman who was operated for right nasal mass diagnosed as paraganglioma in the past and then develop a mass in the right nasal cavity after 2 years which upon surgery diagnosed to be esthesioneuroblastoma on histopathology. Read More

Int Forum Allergy Rhinol 2019 09 28;9(9):1054-1062. Epub 2019 Jun 28.

Department of Otolaryngology-Head and Neck Surgery, University of California, Irvine, CA.

Background: Esthesioneuroblastoma (ENB), also known as olfactory neuroblastoma, represents up to 3% of all sinonasal neoplasms. Hyams histologic grading is a promising tool in predicting metastases and establishing prognoses for this complex tumor.

Methods: A systematic literature search was performed in the PubMed, Ovid MEDLINE, and Cochrane databases. Read More

Radiol Case Rep 2019 Aug 6;14(8):997-1002. Epub 2019 Jun 6.

Carver College of Medicine, University of Iowa, 375 Newton Rd, Iowa City, IA 52242, USA.

Olfactory neuroblastoma (ONB, also called esthesioneuroblastoma) is a rare malignant tumor of neuroectodermal olfactory cells. We report a case of an undifferentiated ONB with unfavorable histology arising ectopically in the nasopharynx. The patient was a 15-year-old male who presented with a right-sided painful neck mass, nasal obstruction, and weight loss. Read More

Radiat Oncol 2019 Jun 13;14(1):107. Epub 2019 Jun 13.

Department of Radiation Oncology, Eye & ENT Hospital, Fudan University, 83 Fenyang Road, Xuhui, Shanghai, 200031, People's Republic of China.

Background: There is a scarcity of data about the prognostic value of orbital invasion in esthesioneuroblastoma (ENB), as well as about its management strategies. Indications for the preservation of orbital contents remain controversial, and the evaluation of orbital invasion has been ill defined.

Methods: This retrospective analysis contained 60 ENB patients with orbital invasion who underwent radiotherapy with or without surgery over the past 14 years. Read More

Cureus 2019 Mar 22;11(3):e4298. Epub 2019 Mar 22.

Neurosurgery, Penn State Milton S. Hershey Medical Center, Hershey, USA.

A 72-year-old female presented with the complaint of declining vision. Workup included magnetic resonance imaging (MRI) which revealed a large enhancing mass extending into the nasal cavity, nasopharynx, and anterior cranial fossa. Computed tomography (CT) imaging revealed extensive calcification. Read More

Vet Comp Oncol 2019 Dec 1;17(4):578-584. Epub 2019 Jul 1.

Department of Pathology, Microbiology, and Immunology, School of Veterinary Medicine, University of California at Davis, Davis, California.

Olfactory neuroblastoma (ONB) is a rare intranasal neoplasm in both dogs and humans. Similar clinical presentation and overlapping histologic and immunohistochemical features of ONB with other intranasal neoplasms can make diagnosis and treatment of intranasal neoplasia challenging. Furthermore, in part because of their rarity, there is a lack of reporting on therapeutic regimen for these neoplasms. Read More

BMJ Case Rep 2019 May 28;12(5). Epub 2019 May 28.

Department of Otolaryngology, Head and Neck Surgery, Westmead Hospital, Sydney, New South Wales, Australia.

Esthesioneuroblastoma is an uncommon tumour, and isolated primary involvement of the maxillary sinus is exceedingly rare. Esthesioneuroblastoma has infrequently been reported as a source of paraneoplastic ectopic hormone production. We report a case of isolated primary maxillary esthesioneuroblastoma, presenting as idiopathic syndrome of inappropriate antidiuretic hormone (SIADH). Read More

World Neurosurg 2019 Aug 24;128:458-463. Epub 2019 May 24.

Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania; Department of Otolaryngology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania. Electronic address:

Background: Endoscopic endonasal approaches have been shown to be as effective as transcranial approaches for sinonasal malignancies involving the skull base. Although they are associated with less risk than an open cranial resection, cerebrospinal fluid (CSF) leaks remain a main concern. Some drugs can raise the intracranial pressure and exacerbate the risk of a postoperative CSF leak. Read More

Childs Nerv Syst 2019 11 12;35(11):2091-2098. Epub 2019 May 12.

Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, India.

Objective: The role of endonasal endoscopic approach for pathologies in the paediatric population is evolving and has still not been accepted as standard of care in neurosurgery. It represents a challenge in terms of narrow access, instrument manipulation and adequate reconstruction of defects. We have described our experience in 49 cases from a single neurosurgical unit in paediatric skull base surgeries through this approach over the last 12 years. Read More

South Asian J Cancer 2019 Apr-Jun;8(2):124-126

Department of Otorhinolaryngology, All India Institute of Medical Sciences, New Delhi, India.

Objective: Although till date no management protocol for esthesioneuroblastoma (ENB) has been standardized due to tumor rarity, still multimodality approach shows better treatment outcomes as compared to surgery alone. The objective of this study was to analyze the clinicopathological spectrum of ENB and to correlate treatment response with tumor staging, histopathological grading, and various treatment modalities.

Materials And Methods: Twenty-one consecutive patients with biopsy-proven ENB were studied and evaluated for response to treatment in the form of complete tumor resolution. Read More

World Neurosurg 2019 Aug 28;128:e409-e416. Epub 2019 Apr 28.

Division of Otorhinolaryngology, Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy; Head and Neck Surgery and Forensic Dissection Research Center (HNS & FDRC), Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy.

Background: Endoscopic endonasal anterior skull base malignant sinonasal tumor resection and reconstruction remains a challenge. We describe our septal flip flap (SFF) reconstruction, a new surgical technique for repairing anterior skull base defects and report our outcomes.

Methods: We retrospectively reviewed the clinical data of 24 patients who underwent skull base reconstruction using a SFF following endoscopic resection with transnasal craniectomy. Read More

Pan Afr Med J 2018 25;31:144. Epub 2018 Oct 25.

Service de Neurochirurgie, CHU Habib Bourguiba de Sfax, Tunisie.

Esthesioneuroblastoma (ENB) is a rare malignant tumor accounting for 3% of all sinonasal cancers. It arises from the olfactory epithelium and usually affects subjects aged 30-50 years. It is uncommon in children. Read More

Int Forum Allergy Rhinol 2019 07 8;9(S3):S127-S144. Epub 2019 Apr 8.

Department of Otolaryngology, School of Medicine, University of North Carolina, Chapel Hill, NC.

Background: Endoscopic skull-base surgery (ESBS) is a rapidly growing discipline that often combines the expertise of otolaryngology-head and neck surgeons and neurosurgeons to provide care for these challenging conditions. As the field has grown, so too has the literature evaluating this surgical approach and the challenges and complications associated with these interventions. An international, multidisciplinary effort to critically evaluate and grade the current literature on ESBS has resulted in the International Consensus Statement on Endoscopic Skull Base Surgery (ICAR:SB). Read More

Auris Nasus Larynx 2019 Dec 4;46(6):871-875. Epub 2019 Apr 4.

Department of Otorhinolaryngology, Dokkyo Medical University Saitama Medical Center, Saitama, Japan.

Objective: The Caldwell-Luc (CL) approach with a sublabial incision is used to manipulate the anterior wall of the maxillary sinus. Paresthesia is one of the major complications associated with the CL approach. We developed a new method, "direct approach to the anterior and lateral part of the maxillary sinus with an endoscope" (DALMA), that negates the need for a sublabial incision and minimizes dental paresthesia by reducing the risk of anterior superior alveolar nerve injury. Read More

Eur Ann Otorhinolaryngol Head Neck Dis 2019 Jun 21;136(3):223-226. Epub 2019 Mar 21.

Service de neurochirurgie, CHU Maison Blanche, 45, rue Cognacq-Jay, 51100 Reims, France.

Introduction: The authors describe their experience of surgical closure of the anterior skull base after tumour resection, using the posterior wall of the frontal sinus.

Material And Method: The authors describe their anterior skull base closure technique performed in three patients after tumour resection. Tumour resection via a transglabellar approach resulted in an anterior skull base defect. Read More

J Vis Exp 2019 02 28(144). Epub 2019 Feb 28.

Department of Medicine and Surgery, University of Insubria.

OTX homeobox (HB) genes are expressed during embryonic morphogenesis and during the development of olfactory epithelium in adult organisms. Mutations occurring in these genes are often related to tumorigenesis in human. No data are available today regarding the possible correlation between OTX genes and tumors of the nasal cavity. Read More

World Neurosurg 2019 Jun 9;126:194-211. Epub 2019 Mar 9.

Department of Neurosurgery, Desert Regional Medical Center, Palm Springs, California.

Introduction: Many controversies exist in the diagnosis and management of this aggressively malignant condition, mainly because of limited literature and lack of randomized control trials, resulting in nonstandardized treatment methods. We performed a comprehensive review of the literature to identify management approach and treatment options for esthesioneuroblastoma.

Methods: An extensive review of the published literature was conducted in PubMed, OVID Medline, and EMBASE journals for studies of esthesioneuroblastoma. Read More

Head Neck 2019 05 7;41(5):1161-1164. Epub 2019 Mar 7.

Department of Radiology, University of California San Francisco, San Francisco, California.

Background: Differentiating esthesioneuroblastoma (ENB) from other sinonasal tumors is difficult by MRI. We tested whether diffusion weighted imaging (DWI) could distinguish ENB from other sinonasal tumors.

Methods: Hundred forty-six patients underwent sinonasal MRI, 75 with technically successful DWI. Read More