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Olfactory neuroblastoma

Zona Batacchi, Nicole K Andeen, Subbulaxmi Trikudanathan
A 62-year-old woman presented with an 11-month history of worsening nasal symptoms of rhinorrhoea, anosmia, nasal congestion and intermittent epistaxis. MRI revealed a large mass in the upper nasal vault. Biopsy of the mass revealed an olfactory neuroblastoma. While waiting resection, she acutely developed severe proximal muscle weakness, lethargy and lower extremity oedema. Blood glucose was elevated, and hypokalaemic metabolic alkalosis was noted. Elevated serum cortisol level of 95.7 µg/dL (8.7-22.4 µg/dL) and markedly elevated 24-hour urinary cortisol level of 6962...
March 13, 2018: BMJ Case Reports
Alejandro López-Hernández, Blanca Vivanco, Alessandro Franchi, Elisabeth Bloemena, Virginia N Cabal, Sira Potes, Cristina Riobello, Cristina García-Inclán, Fernando López, José L Llorente, Mario Hermsen
The sinonasal cavities harbour a variety of rare tumour types. Many carry a poor prognosis while therapeutic options are limited. Histopathological classification can be difficult, especially for poorly differentiated tumours such as olfactory neuroblastoma (ONB), sinonasal neuroendocrine carcinoma (SNEC) and sinonasal undifferentiated carcinoma (SNUC). We analysed Affymetrix OncoScan genome-wide copy number profiles of these three tumour types, both as originally diagnosed and as regrouped by their cytokeratin (Ck) and neuroendocrine (Ne) expression pattern, aiming to find a relation between phenotype and genotype...
March 5, 2018: Scientific Reports
Hiroaki Suefuji, Masashi Koto, Yusuke Demizu, Jun-Ichi Saitoh, Yoshiyuki Shioyama, Hiroshi Tsuji, Tomoaki Okimoto, Tatsuya Ohno, Kenji Nemoto, Takashi Nakano, Tadashi Kamada
The purpose was to evaluate efficacy and safety of carbon ion radiotherapy (C-ion RT) in patients with locally advanced olfactory neuroblastomas (ONBs). This study was a sub-analysis of the Japan Carbon-Ion Radiation Oncology Study Group Study (1402 HN, UMIN000024473). Clinical data of T4 ONBs treated with C-ion RT at four Institutions between November 2003 and December 2014 were retrospectively reviewed. Twenty-one patients underwent C-ion RT. Seven patients had T4a and 14 had T4b tumours without cervical node metastases...
March 2018: Anticancer Research
Giandomenico Maggiore, Maria Silvia Lazio, Oreste Gallo
Olfactory neuroblastoma is a rare malignant tumor of neuroectodermal origin and represents the most common cancer of the nasal cavity in pediatric age. The gold standard of treatment consists of en bloc resection, numerous studies have shown as the endoscopic approaches permit good control of the disease improving the quality of life after the treatment. Herein we describe the case of a 13-year-old patient referred to our outpatient clinic with a polypoid multi-lobed lesion occupying the left nasal cavity and imaging that confirmed a left-sided nasal mass without cribriform plate involvement (Kadish B)...
February 20, 2018: Auris, Nasus, Larynx
Lisa M Rooper, Justin A Bishop, William H Westra
The head and neck is the site of a wide and sometimes bewildering array of neuroendocrine (NE) tumors. Although recognition of NE differentiation may be necessary for appropriate tumor classification and treatment, traditional NE markers such as synaptophysin, chromogranin, and CD56 are not always sufficiently sensitive or specific to make this distinction. Insulinoma-associated protein 1 (INSM1) is a novel transcription factor that has recently demonstrated excellent sensitivity and specificity for NE differentiation in various anatomic sites, but has not yet been extensively evaluated in tumors of the head and neck...
February 12, 2018: American Journal of Surgical Pathology
Diana Bell
Sinonasal tumors with neuroendocrine differentiation form a group of rare heterogeneous neoplasms of neuroectodermal and epithelial origin, consisting of olfactory neuroblastomas and neuroendocrine carcinomas. Because the natural history and biological behavior of this group of tumors vary, the morphological diagnosis coupled with grading/staging is important for prognostication, and the approach to treatment and rehabilitation is multidisciplinary. The identification of molecular abnormalities underlying these tumors is critical to the development of specific targeted therapies and the design of clinical trials...
February 9, 2018: Head and Neck Pathology
Ahmed S Abdelmeguid
PURPOSE OF REVIEW: To provide a comprehensive review of the literature focusing on the recent advances in the diagnosis and management of olfactory neuroblastoma. RECENT FINDINGS: Multimodality treatment is usually recommended for the majority of ONB cases. Recent advances in surgical approaches include the evolving role of endonasal endoscopic surgical resection and reconstruction. The introduction of new conformal radiation techniques has improved the outcomes and reduced treatment-related toxicity to important structures such as the eye and the brain...
February 7, 2018: Current Oncology Reports
B Sipos
Neuroendocrine neoplasms (NENs) are infrequent in sensory organs. There are well-differentiated neuroendocrine neoplasms that should be classified as neuroendocrine tumors, in analogy to their gastrointestinal counterparts, however the nomenclature is inconsistent. The best defined entities are neuroendocrine tumors in the middle ear and ectopic pituitary adenoma in the sphenoid region. Poorly differentiated NENs most often arise in the olfactory organ and nasal cavity that are represented by olfactory neuroblastomas and poorly differentiated neuroendocrine carcinomas...
February 1, 2018: Der Pathologe
Ludovic de Gabory, Benjamin Verillaud, Cécile Rumeau, Philippe Herman, Roger Jankowski, Justin Michel, Héloïse de Kermadec, André Coste, Geoffrey Mortuaire, Christian Righini, Emile Reyt, Olivier Choussy, Vincent Trévillot, Louis Crampette, Elie Serrano, Anjarasoa Tsaranazy, Pierre-Louis Bastier, Sebastien Vergez
BACKGROUND: Given the particularities of olfactory neuroblastoma (ONB) and the lack of studies on the subject, a multicenter collaborative study was conducted to assess treatment strategy. METHODS: Fifty-three patients with ONB were included from the French Rare Head and Neck Cancer Expert Network (REFCOR) database: 16T1, 8T2, 19T3, and 10T4. All cases were treated endoscopically with skull base removal and repair in 26 cases (49%) and without external craniotomy...
January 22, 2018: Head & Neck
Kenny Yu, Federico Roncaroli, Tara Kearney, David Ewins, Deepa Beeharry, Thomas Naylor, David Ray, Rajiv Bhalla, Kanna Gnanalingham
We present the case of a patient with Cushing's syndrome secondary to ectopic ACTH secretion. A MR of the head showed a left-sided nasal mass extending down from the cribriform plate. The patient underwent endoscopic resection with nearly complete removal of the mass. Histological examination showed an ACTH-secreting olfactory neuroblastoma (ONB). The patient's cortisol levels returned to normal range after surgery and have remained normal for over a year. ONB is a rare cause for ectopic ACTH secretion. This case highlights the diagnostic and management difficulties in patients with ectopic ACTH secretion, and provides a brief review of ONB...
January 17, 2018: Acta Neurochirurgica
Jasmina Topcagic, Rebecca Feldman, Anatole Ghazalpour, Jeffrey Swensen, Zoran Gatalica, Semir Vranic
Olfactory neuroblastoma (ONB) is a rare, locally aggressive, malignant neoplasm originating in the olfactory epithelium in the nasal vault. The recurrence rate of ONB remains high and there are no specific treatment guidelines for recurrent/metastatic ONBs. This study retrospectively evaluated 23 ONB samples profiled at Caris Life Sciences (Phoenix, Arizona) using DNA sequencing (Sanger/NGS [Illumina], n = 15) and gene fusions (Archer FusionPlex, n = 6), whole genome RNA microarray (HumanHT-12 v4 beadChip, Illumina, n = 4), gene copy number assays (chromogenic and fluorescent in situ hybridization), and immunohistochemistry...
2018: PloS One
Alberto Schreiber, Vittorio Rampinelli, Marco Ferrari, Davide Mattavelli, Davide Farina, Simonetta Battocchio, Piero Nicolai
OBJECTIVES/HYPOTHESIS: Pretreatment biopsy is crucial in histology-based management of malignant nasoethmoidal tumors, because misdiagnosis can lead to inadequate therapeutic planning. Factors influencing the reliability of biopsy were retrospectively investigated in a tertiary referral center. STUDY DESIGN: Retrospective case series. METHODS: All patients who underwent biopsy, staging magnetic resonance, surgery, and histologic diagnosis for nasoethmoidal tumors in our hospital were retrospectively evaluated...
January 4, 2018: Laryngoscope
B Konukiewitz, A Agaimy, W Weichert, G Klöppel
Common to all neuroendocrine neoplasms (NENs), irrespective of their site of origin, is the expression of synaptophysin and chromogranin A. NENs of the head and neck region derive either from epithelial or neural/neuroectodermal tissues. The epithelial-type NENs express cytokeratins and include the well-differentiated typical and atypical carcinoids (also called low- and intermediate-grade neuroendocrine carcinomas by WHO), the poorly differentiated high-grade neuroendocrine carcinomas of small and large cell type and the mixed neuroendocrine-nonneuroendocrine neoplasms...
February 2018: Der Pathologe
Ashley C Mays, Diana Bell, Renata Ferrarotto, Jack Phan, Dianna Roberts, Clifton D Fuller, Steven J Frank, Shaan M Raza, Michael E Kupferman, Franco DeMonte, Ehab Y Hanna, Shirley Y Su
OBJECTIVE: Compare outcomes of patients with olfactory neuroblastoma (ONB) without skull base involvement treated with and without resection of the dura and olfactory bulb. METHODS: Retrospective review of ONB patients treated from 1992 to 2013 at the MD Anderson Cancer Center (The University of Texas, Houston, Texas, U.S.A.). Primary outcomes were overall and disease-free survival. RESULTS: Thirty-five patients were identified. Most patients had Kadish A/B...
December 11, 2017: Laryngoscope
Mario Turri-Zanoni, Roberta Maragliano, Paolo Battaglia, Marta Giovannardi, Paolo Antognoni, Davide Lombardi, Maria Laura Morassi, Ernesto Pasquini, Paolo Tarchini, Sofia Asioli, Maria Pia Foschini, Fausto Sessa, Piero Nicolai, Paolo Castelnuovo, Stefano La Rosa
OBJECTIVES: To provide a comprehensive review of the clinical and histopathological features of olfactory neuroblastoma (ONB) and other sinonasal neuroendocrine neoplasms (NENs), in order to refine diagnostic criteria, analyze treatment outcomes, and identify prognostic factors. METHODS: Data from an Italian multi-institutional database were analyzed. Patients were treated surgically via a minimally-invasive endoscopic approach followed by adjuvant radiotherapy or radiochemotherapy...
November 2017: Oral Oncology
R Jankowski, C Rumeau, P Gallet, D T Nguyen, A Russel, B Toussaint
The olfactory cleft is the specific site of development of many tumours (respiratory epithelial adenomatoid hamartoma, intestinal-type adenocarcinoma, neuroblastoma, inverted papilloma, glomangiopericytoma, etc.) and is also the site of CSF rhinorrhoea via the cribriform plate (cribri-rhinorrhoea). Olfactory cleft surgery must therefore be considered to be a specific type of surgery, complementary to ethmoidal labyrinth surgery and anterior skull base surgery. Olfactory cleft tumours can be resected according to five different surgical procedures: olfactory cleft mucosal resection, partial resection of the olfactory cleft, total resection of the olfactory cleft, unilateral endoscopic anterior skull base resection, and bilateral endoscopic anterior skull base resection...
October 23, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
Ricardo Bartel, Xavier Gonzalez-Compta, Enric Cisa, Francesc Cruellas, Alberto Torres, Aleix Rovira, Manel Manos
INTRODUCTION AND OBJECTIVES: Olfactory neuroblastoma (ONB) is a rare entity that constitutes less than 5% of nasosinusal malignancies. Mainstream treatment consists in surgical resection+/-adjuvant radiotherapy. By exposing results observed with apparition of new therapeutic options as neoadjuvant chemotherapy, the objective is to evaluate a series and a review of the current literature. METHODS: A retrospective review was conducted including patients diagnosed and followed-up for ONB from 2008 to 2015 in our institution...
October 20, 2017: Acta Otorrinolaringológica Española
Dario Candini, Ilaria Biasato, Paulo Ricardo Dell'Armelina Rocha, Elena Grego, Maria Teresa Capucchio, Cristina Vercelli
Behavioral alterations in dogs are not easy to understand and cure. The situation is more difficult when an encephalitis due to Canine Distemper Virus (CDV) and a concomitant olfactory neuroblastoma are present. This case report deals with the story of a 5-year-old Swiss shepherd dog with behavioral changes, seizures, epistaxis and ataxia. Following clinical and laboratory exams, a suspected diagnosis of CDV infection was hypothesized, and a therapy based on Ω-interferon was administered. Every supporting therapy failed and the worsening of the clinical conditions led to the euthanasia of the patient...
August 28, 2017: Veterinary Sciences
Gabriela G Chavez, Gabriella Taylor, Jekaterina Garaliene, Guy P Richardson, Sergei A Korneev
Experimental work over the past several years has revealed an unexpected abundance of long natural antisense transcripts (NATs) in eukaryotic species. In light of the proposed role of such RNA molecules in the regulation of gene expression in the brain, attention is now focused on specific examples of neuronal NATs. Of particular interest are NATs that are complementary to mRNAs encoding nitric oxide synthase (NOS), the enzyme responsible for production of the important gaseous neurotransmitter nitric oxide (NO)...
December 1, 2017: Nitric Oxide: Biology and Chemistry
Richard J Harvey, Sunny Nalavenkata, Raymond Sacks, Nithin D Adappa, James N Palmer, Michael T Purkey, Rodney J Schlosser, Carl Snyderman, Eric W Wang, Bradford A Woodworth, Robert Smee, Tom Havas, Richard Gallagher
BACKGROUND: Advanced-stage olfactory neuroblastoma requires multimodal therapy for optimal outcomes. Debate exists over endoscopic endonasal surgery in this situation. Stage-matched open and endoscopic surgical therapy were compared. METHODS: Patients from 6 cancer institutions were assessed. Stratification included dural involvement, Kadish stage, nodal disease, Hyams' grade, approach, and margin status. At follow-up, local control, nodal status, and evidence of distant metastases were recorded with any subsequent therapy...
September 25, 2017: Head & Neck
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