{"orpha":86879,"name":"Extranodal nasal NK/T cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Angiocentric T-cell lymphoma","Lethal midline granuloma","NK/T-cell lymphoma","NKTCL","Nasal T/natural killer-cell lymphoma"],"definition":"Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare, malignant neoplasm mainly affecting men in the fifth decade of life, that usually arises in the nose, paranasal sinuses, orbits or upper airway, and that can present with a nasal mass, nasal bleeding, nasal obstruction, palate perforation (i.e. midline perforation of the hard palate), and mid-facial and/or upper airway destructive lesions. In advanced disease stages, which are associated with a poor prognosis, NKTCL may disseminate to other organs. A few cases of NKTCL presenting primarily in the lymph nodes have also been described.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86879/","orphanet_url":"https://www.orpha.net/en/disease/detail/86879","names":{"fr":{"name":"Lymphome NK/T extranodulaire type nasal","synonyms":["NKTCL","Granulome létal de la ligne médiane","Lymphome NK/T","Lymphome T angiocentrique","Lymphome à cellules natural killer/T nasal"],"definition":"Néoplasie maligne rare touchant principalement les hommes dans la cinquantaine. Elle se développe généralement dans le nez, les sinus paranasaux, les orbites ou les voies aériennes supérieures et peut se présenter sous la forme d'une masse nasale, d'un saignement nasal, d'une obstruction nasale, d'une perforation du palais (perforation de la ligne médiane du palais dur) et de lésions destructrices des voies aériennes moyennes et/ou supérieures. À un stade avancé de la maladie, qui est associé à un mauvais pronostic, le lymphome NK/T extranodulaire type nasal peut se propager à d'autres organes. Quelques cas se manifestant principalement dans les ganglions lymphatiques ont également été décrits."},"de":{"name":"Extranodales NK/T-Zell-Lymphom","synonyms":["Mittelliniengranulom, letales","NK/T-Zell-Lymphom","NKTCL","Nasales T-Zell-/Natürliche-Killer-Zell-Lymphom","T-Zell-Lymphom, angiozentrisches"]},"es":{"name":"Linfoma extranodal nasal de células NK/T","synonyms":["Granuloma letal de la línea media","Linfoma angiocéntrico de células T","Linfoma de células NK/T","Linfoma nasal de células T/natural killer","NKTCL"],"definition":"Es una neoplasia maligna poco frecuente que afecta principalmente a los varones en la quinta década de vida. Por lo general, aparece en la nariz, los senos paranasales, las órbitas o vías respiratorias superiores, y puede presentarse con una masa / sangrado / obstrucción nasal, perforación del paladar (por ej., perforación de la línea media del paladar duro) y lesiones destructivas a nivel mediofacial y/o de las vías aéreas superiores. En estadios avanzados de la enfermedad, asociados a un mal pronóstico, esta neoplasia puede diseminarse a otros órganos. También se han descrito algunos casos de este tipo de linfomas que se presentan en los ganglios linfáticos de forma primaria."},"it":{"name":"Linfoma nasale extranodale a cellule NK/T","synonyms":["Granuloma letale della linea mediana","Linfoma angiocentrico a cellule T","Linfoma nasale a cellule T/natural killer","NKTCL"],"definition":"Il linfoma nasale extranodale a cellule NK/T (NKTCL) è una neoplasia maligna rara, che colpisce di solito i maschi nella quinta decade di vita. In genere, origina dal naso, dai seni paranasali, dalle orbite o dalle vie aeree superiori; si presenta come una massa nel naso, con emorragie nasali, ostruzione del naso, perforazione del palato (perforazione della linea mediana del palato duro) e lesioni distruttive delle vie aeree medio-facciali e/o superiori. Negli stadi avanzati, che comportano una prognosi sfavorevole, la malattia può diffondersi ad altri organi. Sono stati descritti alcuni casi di NKTCL con localizzazione primaria nei linfonodi."},"nl":{"name":"Extranodaal nasaal NK/T-cellymfoom","synonyms":["Angiocentrisch T-cellymfoom","Letaal middellijngranuloom","NK/T-cellymfoom","NKTCL","Nasaal T-cel-/natuurlijke killercellymfoom"],"definition":"Extranodaal nasaal NK/T-cellymfoom (NKTCL) is een zeldzaam, maligne neoplasma dat vooral mannen in hun vijfde levensdecennium treft, dat meestal ontstaat in de neus, paranasale sinussen, oogkassen of bovenste luchtwegen, en dat zich kan manifesteren met een gezwel in de neus, nasale bloeding, obstructie van de neus, perforatie van het verhemelte (i.e. perforatie ter hoogte van de middellijn van het harde verhemelte), en destructieve laesies aan het middengezicht en/of de bovenste luchtwegen. In de gevorderde stadia, die zijn geassocieerd met een slechte prognose, kan NKTCL zich verspreiden naar andere organen. Er werden ook enkele gevallen beschreven waarbij NKTCL zich voornamelijk voordeed in de lymfeklieren."},"pt":{"name":"Linfoma extranodal das células NK/T, tipo nasal"},"pl":{"name":"Pozawęzłowy chłoniak z komórek NK/T typu nosowego","synonyms":["Angiocentryczny chłoniak z komórek T","Chłoniak z komórek NK/T","NKTCL","Nosowy chłoniak z komórek T/NK","Ziarniniak pośrodkowy"]},"cs":{"name":"Extranodální nazální NK/T-buněčný lymfom","synonyms":["Angiocentrický T-lymfom","NKTCL","Letální granulom střední čáry","NK/T-buněčný lymfom","Nazální T/natural killer-buněčný lymfom"]},"uk":{"name":"Екстранодальна назальна NK/T-клітинна лімфома","synonyms":["Ангіоцентрична Т-клітинна лімфома","Смертельна гранульома серединної лінії","Т-лімфома носа/природна кілерна лімфома","NK/T-клітинна лімфома","NKTCL"],"definition":"Extranodal nasal NK/T cell lymphoma (NKTCL) is a rare, malignant neoplasm mainly affecting men in the fifth decade of life, that usually arises in the nose, paranasal sinuses, orbits or upper airway, and that can present with a nasal mass, nasal bleeding, nasal obstruction, palate perforation (i.e. midline perforation of the hard palate), and mid-facial and/or upper airway destructive lesions. In advanced disease stages, which are associated with a poor prognosis, NKTCL may disseminate to other organs. A few cases of NKTCL presenting primarily in the lymph nodes have also been described."},"zh":{"name":"结外鼻型NK/T细胞淋巴瘤","synonyms":["鼻型T/自然杀伤细胞淋巴瘤"]},"tr":{"name":"Ekstranodal nazal NK / T-hücreli lenfoma","synonyms":["NKTCL","NK/T hücreli lenfoma","Letal orta hat granülomu","Anjiyosentrik T hücreli lenfoma","Nazal T/doğal öldürücü hücreli lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C86.0","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A90.6","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"684005900"},{"source":"MONDO","code":"0019472","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D054391","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0392788","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7041","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10065855","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":178551,"name":"Aggressive primary cutaneous T-cell lymphoma","classifications":[187,194,202,233]},{"orpha":289644,"name":"Epstein-Barr virus-associated malignant lymphoproliferative disorder","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.25,"area":"France","status":"Validated","source":"OTHERitut National du Cancer 2013[OTHER]"}],"phenotypes":null}