{"orpha":289596,"name":"Juvenile nasopharyngeal angiofibroma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["JNA"],"definition":"Juvenile nasopharyngeal angiofibroma (JNA) is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the nasopharynx, which affects mainly young and adolescent males (onset usually occurring between 7-19 years of age) and that presents as a mass in the nasopharynx and nasal cavity, leading to manifestations such as nasal obstruction, epistaxis, profound facial swelling, proptosis or diplopia. Although slowly progressive, it has a high rate of recurrence and sometimes invades adjacent structures.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-289596/","orphanet_url":"https://www.orpha.net/en/disease/detail/289596","names":{"fr":{"name":"Angiofibrome nasopharyngé juvénile","synonyms":["JNA"],"definition":"Tumeur fibrovasculaire rare et bénigne, mais localement agressive, qui se développe à partir de la paroi postéro-latérale du nasopharynx. Elle touche principalement les jeunes hommes et les adolescents (son apparition se situe généralement entre l'âge de 7 et 19&nbsp;ans) et se présente sous la forme d'une masse dans le nasopharynx et la cavité nasale, entraînant des manifestations telles qu'une obstruction nasale, une épistaxis, un gonflement prononcé du visage, une proptose ou une diplopie. Bien que la maladie évolue lentement, le taux de récidive est élevé et l'invasion des structures adjacentes est parfois observée."},"de":{"name":"Nasopharyngeales Angiofibrom, juveniles","synonyms":["JNA"]},"es":{"name":"Angiofibroma nasofaríngeo juvenil","synonyms":["JNA"],"definition":"Es un tumor fibrovascular poco frecuente y benigno, aunque localmente agresivo, que se origina en la pared posterolateral de la nasofaringe. Afecta principalmente a varones jóvenes y adolescentes (por lo habitual, entre los 7-19 años de edad) y que se presenta como una masa en la nasofaringe y en la cavidad nasal, conllevando manifestaciones tales como obstrucción nasal, epistaxis, notable hinchazón facial, proptosis o diplopía. Aunque lentamente progresivo, tiene una elevada tasa de recurrencia, invadiendo ocasionalmente estructuras adyacentes."},"it":{"name":"Angiofibroma nasofaringeo giovanile","synonyms":["JNA"],"definition":"L'angiofibroma nasofaringeo giovanile (JNA) è un tumore raro fibrovascolare benigno, localmente aggressivo, che origina dalla parete posterolaterale del rinofaringe, interessando prevalentemente maschi durante la tarda infanzia e l'adolescenza (la malattia esordisce di solito tra i 7 e i 19 anni) e si presenta sotto forma di massa nella cavità nasale e nel rinofaringe, provocando ostruzione nasale, epistassi, edema facciale importante, proptosi, diplopia. Sebbene la progressione sia lenta, la malattia presenta un elevato tasso di recidive e talvolta invade le strutture adiacenti."},"nl":{"name":"Juveniel nasofaryngeaal angiofibroom","synonyms":["JNA"],"definition":"Juveniel nasofaryngeaal angiofibroom (JNA) is een zeldzame en goedaardige maar lokaal agressieve fibrovasculaire tumor die ontstaat uit de posterolaterale wand van de nasofarynx, die voornamelijk jonge en adolescente mannen treft (aanvang vindt meestal plaats tussen de leeftijd van 7-19 jaar), en die tot uiting komt als een gezwel in de nasofarynx en de neusholte, met als gevolg manifestaties zoals obstructie van de neus, epistaxis (bloedneus), uitgesproken zwelling van het gelaat, proptosis of diplopie. Hoewel de ziekte traag progressief is, is er een grote kans op recidief en treedt soms invasie op van aangrenzende structuren."},"pt":{"name":"Angiofibroma nasofaríngeo juvenil","synonyms":["JNA"]},"pl":{"name":"Naczyniakowłókniak młodzieńczy nosogardła","synonyms":["JNA"]},"cs":{"name":"Juvenilní nasofaryngeální angiofibrom","synonyms":["JNA"]},"uk":{"name":"Ювенільна ангіофіброма носоглотки","synonyms":["JNA"],"definition":"Juvenile nasopharyngeal angiofibroma (JNA) is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the nasopharynx, which affects mainly young and adolescent males (onset usually occurring between 7-19 years of age) and that presents as a mass in the nasopharynx and nasal cavity, leading to manifestations such as nasal obstruction, epistaxis, profound facial swelling, proptosis or diplopia. Although slowly progressive, it has a high rate of recurrence and sometimes invades adjacent structures."},"zh":{"name":"青少年鼻咽血管纤维瘤","synonyms":["青少年鼻咽血管纤维瘤"],"definition":"青少年型鼻咽血管纤维瘤（JNA）是一种罕见的良性局灶侵袭性纤维血管瘤，主要累及青少年男性（发病年龄通常在7-19岁），表现为鼻咽和鼻腔肿块，导致鼻塞、鼻出血、严重的面部肿胀、突眼突或复视等。虽然进展缓慢，但复发率高，有时侵犯邻近结构。"},"tr":{"name":"Jüvenil nazofarengeal anjiyofibrom","synonyms":["JNA"]}},"xrefs":[{"source":"ICD-10","code":"D10.6","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2E90.6","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1862070581"},{"source":"MONDO","code":"0017340","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1367536","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98036,"name":"Rare otorhinolaryngologic disease"},"parents":[{"orpha":98061,"name":"Rare otorhinolaryngologic tumor","classifications":[200,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.6666,"area":"Worldwide","status":"Validated","source":"28199045[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-07-24","source":"30345941[PMID]_29752322[PMID]_4284864[PMID]_15951557[PMID]_19626637[PMID]","terms":[{"hpo":"HP:0030429","label":"Juvenile nasopharyngeal angiofibroma","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000421","label":"Epistaxis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000282","label":"Facial edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001742","label":"Nasal congestion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000520","label":"Proptosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002516","label":"Increased intracranial pressure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012198","label":"Juvenile colonic polyposis","frequency":"Very rare (<4-1%)","criteria":null}]}}