{"orpha":329977,"name":"Classic neuroendocrine tumor of appendix","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Classic appendiceal neuroendocrine tumor","Classic appendix neuroendocrine tumor"],"definition":"A rare endocrine tumor of the appendix, seen twice as frequently in females than in males, and usually presenting before the fifth decade of life. It is usually asymptomatic when located in the tip of the appendix (without obstruction), but acute appendicitis is often associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-329977/","orphanet_url":"https://www.orpha.net/en/disease/detail/329977","names":{"fr":{"name":"Tumeur neuroendocrine classique de l'appendice","synonyms":["Tumeur neuroendocrine appendiculaire classique"],"definition":"Type de tumeur neuroendocrine de l'appendice rare qui se manifeste habituellement avant la cinquième décennie et survient deux fois plus fréquemment chez les femmes que chez les hommes. Lorsqu'elle est située au niveau de la pointe de l'appendice (sans obstruction), elle est généralement asymptomatique, mais une appendicite aigüe est souvent associée."},"de":{"name":"Neuroendokriner Tumor des Apendix, klassischer","synonyms":["Appendix-NET, klassischer"],"definition":"Ein seltener endokriner Tumor des Blinddarms, der bei Frauen doppelt so häufig auftritt wie bei Männern und in der Regel vor dem fünften Lebensjahrzehnt auftritt. Er ist in der Regel asymptomatisch, wenn er sich in der Spitze des Blinddarms befindet (ohne Obstruktion), aber oft geht er mit einer akuten Blinddarmentzündung einher."},"es":{"name":"Tumor neuroendocrino clásico del apéndice","synonyms":["Tumor neuroendocrino apendicular clásico"],"definition":"Es un tumor endocrino poco frecuente del apéndice, que se observa con una frecuencia dos veces mayor en mujeres que en hombres, y que generalmente se presenta antes de la quinta década de la vida. Suele ser asintomático cuando se localiza en la punta del apéndice (sin obstrucción), pero suele asociarse a apendicitis aguda."},"it":{"name":"Tumore endocrino classico dell'appendice","synonyms":["Tumore endocrino appendicolare classico"]},"nl":{"name":"Klassieke neuro-endocriene tumor van appendix","synonyms":["Klassieke appendiculaire neuro-endocriene tumor"],"definition":"Een zeldzame endocriene tumor van appendix, die tweemaal zo vaak wordt waargenomen bij vrouwen dan bij mannen en zich meestal presenteert voor het vijfde levensdecennium. De tumor is doorgaans asymptomatisch wanneer deze is gesitueerd aan het uiteinde van appendix (zonder obstructie), maar vaak is acute appendicitis geassocieerd."},"pt":{"name":"Tumor endócrino clássico do apêndice","synonyms":["Tumor neuroendócrino clássico do apêndice"]},"pl":{"name":"Klasyczny guz neuroendokrynny wyrostka robaczkowego","synonyms":["Classic appendiceal neuroendocrine tumor","Classic appendix neuroendocrine tumor"]},"cs":{"name":"Klasický endokrinní nádor apendixu","synonyms":["Classic appendiceal neuroendocrine tumor","Classic appendix neuroendocrine tumor"]},"uk":{"name":"Класична нейроендокринна пухлина апендикса","synonyms":["Класична нейроендокринна пухлина апендикса","Класична нейроендокринна пухлина апендикса"],"definition":"A rare endocrine tumor of the appendix, seen twice as frequently in females than in males, and usually presenting before the fifth decade of life. It is usually asymptomatic when located in the tip of the appendix (without obstruction), but acute appendicitis is often associated."},"zh":{"name":"经典阑尾神经内分泌肿瘤","synonyms":["Classic appendiceal neuroendocrine tumor","Classic appendix neuroendocrine tumor"],"definition":"典型阑尾内分泌肿瘤是阑尾内分泌肿瘤（见该术语）的一种类型，女性发病率是男性的两倍，通常出现在五岁以前。典型阑尾内分泌肿瘤位于阑尾顶端时通常无症状（无梗阻），但常伴发急性阑尾炎。"},"tr":{"name":"Apendiksin klasik nöroendokrin tümörü","synonyms":["Klasik apendiks nöroendokrin tümör","Klasik apendiks nöroendokrin tümörü"]}},"xrefs":[{"source":"ICD-10","code":"D37.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018016","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679854","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":100079,"name":"Neuroendocrine neoplasm of appendix","classifications":[152,193,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.25,"area":"Worldwide","status":"Not yet validated","source":"20524865[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}