{"orpha":1957,"name":"Esthesioneuroblastoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Olfactory neuroblastoma"],"definition":"Esthesioneuroblastoma (ENB) is a rare malignant neoplasm of the sinonasal cavity, arising from the basal layers of olfactory neuroepithelial cells in the superior nasal vault, which usually occurs in the 5th to 6th decades of life and is characterized clinically by non-specific symptoms such as progressive ipsilateral nasal block, sinusitis, facial pain, intermittent headaches, hyposmia/dysosmia, rhinorrhea and epistaxis as well as proptosis, diplopia and excessive lacrimation due to orbital extension. With early treatment and in the absence of distant metastases, ENB appears to have a good prognosis (compared to other superior nasal malignancies), despite a high rate of cervical metastases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-1957/","orphanet_url":"https://www.orpha.net/en/disease/detail/1957","names":{"fr":{"name":"Esthésioneuroblastome","synonyms":["Neuroblastome olfactif"],"definition":"Tumeur maligne rare de la cavité nasale, prenant naissance dans les couches basales des cellules neuroépithéliales olfactives de la voûte nasale, survenant généralement entre la 5<sup>e</sup> et la 6<sup>e</sup> décennie de vie. Elle se caractérise par des signes cliniques non spécifiques, à savoir obstruction nasale ipsilatérale progressive, sinusite, douleur faciale, maux de tête intermittents, hyposmie/dysosmie, rhinorrhée et épistaxis ainsi que proptose, diplopie et larmoiement excessif en raison de l'atteinte de l'orbite. Malgré le taux élevé de métastases cervicales, avec un traitement précoce et en l'absence de métastases à distance, le pronostic semble favorable (comparé à d'autres tumeurs malignes de la voûte nasale)."},"de":{"name":"Ästhesioneuroblastom","synonyms":["Olfaktorisches Neuroblastom"],"definition":"Das Ästhesioneuroblastom (ENB) ist ein seltenes bösartiges Neoplasma der Sinonasalhöhle, das aus den Grundschichten der olfaktorischen neuroepithelialen Zellen im oberen Nasengewölbe entsteht. Es tritt in der Regel im 5. bis 6. Lebensjahrzehnt auf und ist klinisch gekennzeichnet durch unspezifische Symptome wie fortschreitende ipsilaterale Nasenblockade, Sinusitis, Gesichtsschmerzen, intermittierende Kopfschmerzen, Hyposmie/Dysosmie, Rhinorrhö und Epistaxis sowie Proptose, Diplopie und übermäßige Tränenfluss durch Orbitalausdehnung. Bei frühzeitiger Behandlung und ohne Fernmetastasen scheint ENB trotz einer hohen Rate an Halsmetastasen eine gute Prognose zu haben (im Vergleich zu anderen superior-nasalen Malignomen)."},"es":{"name":"Estesioneuroblastoma","synonyms":["Neuroblastoma olfativo"],"definition":"El esterineuroblastoma (ENB) es un tumor raro maligno de la cavidad nasal, que se origina a partir de las capas basales de las células olfatorias neuroepiteliales de la cavidad nasal. Por lo general se presenta entre la quinta y la sexta década de vida y se caracteriza clínicamente por síntomas inespecíficos tales como obstrucción nasal ipsilateral progresiva sinusitis, dolor facial, dolores de cabeza intermitentes, hiposmia/disosmia, rinorrea y epistaxis así como proptosis, diplopía y abundante lagrimeo debido a la extensión orbital. Con un tratamiento temprano y en ausencia de metástasis a distancia, el ENB parece tener un buen pronóstico (comparado con otras neoplasias nasales superiores) a pesar de la elevada tasa de metástasis cervicales."},"it":{"name":"Estesioneuroblastoma","synonyms":["Neuroblastoma olfattivo"]},"nl":{"name":"Esthesioneuroblastoom","synonyms":["Olfactorisch neuroblastoom"],"definition":"Esthesioneuroblastoom (ENB) is een zeldzaam kwaadaardig neoplasma van de neusholte dat ontstaat in de basale lagen van olfactorische neuro-epitheelcellen in de bovenste neusholte, meestal opkomt tijdens het vijfde of zesde levensdecennium, en klinisch gekarakteriseerd wordt door niet-specifieke symptomen zoals progressieve ipsilaterale neusverstopping, sinusitis, pijn aan het gezicht, wederkerende hoofdpijn, hyposmie/dysosmie, loopneus en bloedneus, alsook exoftalmus, diplopie en overmatig tranen door een uitbreiding van de oogkas. Bij een vroege behandeling en afwezigheid van verre uitzaaiingen lijkt EBN een gunstige prognose te hebben (in vergelijking met andere aandoeningen bovenin de neus), ondanks een hoog aantal cervicale uitzaaiingen."},"pt":{"name":"Estesioneuroblastoma"},"pl":{"name":"Nerwiak węchowy zarodkowy","synonyms":["Nerwiak węchowy zarodkowy"]},"cs":{"name":"Estezioneuroblastom","synonyms":["Olfaktoriální neuroblastom"]},"uk":{"name":"Естезионейробластома","synonyms":["Олфакторна нейробластома"],"definition":"Esthesioneuroblastoma (ENB) is a rare malignant neoplasm of the sinonasal cavity, arising from the basal layers of olfactory neuroepithelial cells in the superior nasal vault, which usually occurs in the 5th to 6th decades of life and is characterized clinically by non-specific symptoms such as progressive ipsilateral nasal block, sinusitis, facial pain, intermittent headaches, hyposmia/dysosmia, rhinorrhea and epistaxis as well as proptosis, diplopia and excessive lacrimation due to orbital extension. With early treatment and in the absence of distant metastases, ENB appears to have a good prognosis (compared to other superior nasal malignancies), despite a high rate of cervical metastases."},"zh":{"name":"鼻腔神经胶质瘤","synonyms":["嗅神经母细胞瘤"]},"tr":{"name":"Estezionöroblastom","synonyms":["Olfaktör nöroblastoma"]}},"xrefs":[{"source":"ICD-10","code":"C30.0","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2C20.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2007774165"},{"source":"ICD-11","code":"XH50L1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"53002801"},{"source":"MONDO","code":"0016029","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018304","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206717","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2197","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251870,"name":"Central nervous system embryonal tumor","classifications":[181,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.02,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET"}],"phenotypes":null}