{"orpha":877,"name":"Neuroendocrine neoplasm","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"A group of rare tumors characterized by predominantly neuroendocrine differentiation, potentially arising in most organs of the body, including the central nervous system, respiratory tract, larynx, gastrointestinal tract, thyroid, skin, breast, and urogenital system. The gastrointestinal tract and lungs are the most common primary tumor sites. Based on clinical behavior, histology, and proliferation rate, the tumors may be categorized as well differentiated (low grade to intermediate grade) neuroendocrine tumors and poorly differentiated (high grade) neuroendocrine carcinomas. They may or may not be associated with clinical hormone hypersecretion syndromes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-877/","orphanet_url":"https://www.orpha.net/en/disease/detail/877","names":{"fr":{"name":"Néoplasme neuroendocrine","definition":"Groupe de tumeurs rares caractérisées par une différenciation neuroendocrinienne prédominante, pouvant apparaître dans la plupart des organes de l'organisme, y compris le système nerveux central, les voies respiratoires, le larynx, le tractus gastro-intestinal, la thyroïde, la peau, les seins et le système urogénital. Le tractus gastro-intestinal et les poumons sont les zones tumorales primaires les plus fréquentes. Selon les signes cliniques, l'histologie et le taux de prolifération, les tumeurs peuvent être classées en deux catégories : les tumeurs neuroendocrines différenciées (de grade faible à intermédiaire) et les carcinomes neuroendocrines peu différenciés (de grade élevé). Ils peuvent ou non être associés à des syndromes cliniques d'hypersécrétion hormonale."},"de":{"name":"Neuroendokrine Neoplasie","definition":"Eine Gruppe seltener Tumoren, die sich durch eine vorwiegend neuroendokrine Differenzierung auszeichnen und in den meisten Organen des Körpers auftreten können, einschließlich des zentralen Nervensystems, der Atemwege, des Kehlkopfs, des Magen-Darm-Trakts, der Schilddrüse, der Haut, der Brust und des Urogenitalsystems. Der Gastrointestinaltrakt und die Lunge sind die häufigsten Primärtumorstandorte. Auf der Grundlage des klinischen Verhaltens, der Histologie und der Proliferationsrate können die Tumoren in gut differenzierte (niedriggradige bis mittelgradige) neuroendokrine Tumoren und schlecht differenzierte (hochgradige) neuroendokrine Karzinome eingeteilt werden. Sie können mit klinischen Hormonhypersekretionssyndromen verbunden sein oder auch nicht."},"es":{"name":"Tumor neuroendocrino","definition":"Es un grupo de tumores poco frecuentes caracterizados por una diferenciación predominantemente neuroendocrina, que se desarrolla potencialmente en la mayoría de los órganos del cuerpo, incluido el sistema nervioso central, el tracto respiratorio, la laringe, el tracto digestivo, la tiroides, la piel, la mama y el sistema urogenital. El tracto digestivo y los pulmones son los sitios de tumores primarios más comunes. Según el comportamiento clínico, la histología y la tasa de proliferación, los tumores pueden clasificarse como tumores neuroendocrinos bien diferenciados (bajo grado bajo a grado intermedio) y carcinomas neuroendocrinos poco diferenciados (alto grado). Pueden o no estar asociados a síndromes clínicos de hipersecreción hormonal."},"it":{"name":"Neoplasia neuroendocrina","definition":"Si tratta di un gruppo di tumori rari caratterizzati da una differenziazione prevalentemente neuroendocrina, che possono colpire la maggior parte degli organi, tra i quali il sistema nervoso centrale, le vie respiratorie, la laringe, il tratto gastrointestinale, la tiroide, la cute, le mammelle e l'apparato urogenitale. Di solito i tumori primari si localizzano nel tratto gastrointestinale e nei polmoni. In base al decorso clinico, all'istologia e al tasso di proliferazione, i tumori vengono classificati in tumori neuroendocrini ben differenziati (di grado basso o intermedio) e carcinomi neuroendocrini scarsamente differenziati (di grado alto). Queste neoplasie possono associarsi alle sindromi cliniche da ipersecrezione ormonale."},"nl":{"name":"Neuro-endocrien neoplasma","definition":"Een groep van zeldzame tumoren, gekarakteriseerd door hoofdzakelijk neuro-endocriene differentiatie, die mogelijk kunnen ontstaan in de meeste organen van het lichaam, waaronder centraal zenuwstelsel, ademhalingsorganen, larynx, maag-darmstelsel, schildklier, huid, borst, en urogenitaal stelsel. Maag-darmstelsel en long zijn de meest gangbare plaatsen van primaire tumoren. Op basis van klinisch gedrag, histologie, en snelheid van proliferatie kunnen de tumoren gecategoriseerd worden als goed gedifferentieerde (laaggradige tot intermediaire) neuro-endocriene tumoren en slecht gedifferentieerde (hooggradige) neuro-endocriene carcinomen. Ze kunnen al dan niet geassocieerd zijn met syndromen van klinische hypersecretie van hormoon."},"pt":{"name":"Tumor neuro-endócrino"},"pl":{"name":"Guz endokrynowy","synonyms":["APUDoma"]},"cs":{"name":"Endokrinní tumor","synonyms":["APUDom","Neuroendokrinní tumor"]},"uk":{"name":"Нейроендокринне новоутворення","definition":"A group of rare tumors characterized by predominantly neuroendocrine differentiation, potentially arising in most organs of the body, including the central nervous system, respiratory tract, larynx, gastrointestinal tract, thyroid, skin, breast, and urogenital system. The gastrointestinal tract and lungs are the most common primary tumor sites. Based on clinical behavior, histology, and proliferation rate, the tumors may be categorized as well differentiated (low grade to intermediate grade) neuroendocrine tumors and poorly differentiated (high grade) neuroendocrine carcinomas. They may or may not be associated with clinical hormone hypersecretion syndromes."},"zh":{"name":"神经内分泌瘤"},"tr":{"name":"Nöroendokrin neoplazm"}},"xrefs":[{"source":"MONDO","code":"0019496","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018358","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206754","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9316","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10052399","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":97978,"name":"Rare endocrine disease","classifications":[193]},{"orpha":182130,"name":"Tumor of endocrine glands","classifications":[202]}],"children":[{"orpha":100093,"name":"Carcinoid syndrome","type":"Clinical syndrome"},{"orpha":100092,"name":"Gastroenteropancreatic neuroendocrine neoplasm","type":"Category"},{"orpha":100101,"name":"Neuroendocrine tumor with other location","type":"Category"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":3.2,"area":"Norway","status":"Validated","source":"18853416[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":5.25,"area":"United States","status":"Validated","source":"18565894[PMID]_National Cancer OTHERitute[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.53,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":35.0,"area":"United States","status":"Validated","source":"18565894[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}