{"orpha":97253,"name":"Neuroendocrine tumor of pancreas","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["PNET","Pancreatic NET","Pancreatic neuroendocrine tumor","Well-differentiated NEN of pancreas","Well-differentiated neuroendocrine neoplasm of pancreas","Well-differentiated pancreatic NEN","Well-differentiated pancreatic neuroendocrine neoplasm"],"definition":"Pancreatic endocrine tumor, also known as pancreatic neuroendocrine tumor (PNET), describes a group of endocrine tumors originating in the pancreas that are usually indolent and benign, but may have the potential to be malignant. They can be functional, exhibiting a hormonal hypersecretion syndrome, but can be non-functional presenting with non-specific symptoms and include insulinoma, glucagonoma, VIPoma, somatostatinoma (SSoma), PPoma and Zollinger-Ellison syndrome (ZES, or gastrinoma) and other ectopic hormone producing tumors (such as GRFoma).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97253/","orphanet_url":"https://www.orpha.net/en/disease/detail/97253","names":{"fr":{"name":"Tumeur neuroendocrine du pancréas","synonyms":["NEN du pancréas bien différencié","NEN pancréatique bien différencié","Néoplasme neuroendocrine du pancréas bien différencié","Néoplasme neuroendocrine pancréatique bien différencié","PNET"],"definition":"Également connue sous le nom de tumeur neuroendocrine pancréatique (PNET pour pancreatic neuroendocrine tumor), l'entité fait partie d'un groupe de tumeurs endocrines prenant naissance dans le pancréas, généralement indolentes et bénignes, mais qui peuvent être à potentiel malin. Elles peuvent être fonctionnelles et associées à un syndrome d'hypersécrétion hormonale, mais aussi non fonctionnelles et associées à des symptômes non spécifiques. Parmi ces tumeurs figurent l'insulinome, le glucagonome, le VIPome, le somatostatinome (SSome), le PPome et le syndrome de Zollinger-Ellison (ZES, ou gastrinome) et d'autres tumeurs productrices d'hormones ectopiques (telles que le GRFome)."},"de":{"name":"Neuroendokriner Tumor des Pankreas","synonyms":["Hoch-differenzierte NEN des Pankreas","Hoch-differenzierte neuroendokrine Neoplasie des Pankreas","Hoch-differenzierte pankreatische neuroendokrine Neoplasie","NET des Pankreas","Neuroendokrine Neoplasie des Pankreas","PNET"]},"es":{"name":"Tumor neuroendocrino del páncreas","synonyms":["Neoplasia neuroendocrina del páncreas bien diferenciada","Neoplasia neuroendocrina pancreática bien diferenciada","PNET","TNE bien diferenciado del páncreas","TNE bien diferenciado pancreático","TNE pancreático","Tumor neuroendocrino pancreático"],"definition":"El tumor neuroendocrino pancreático (pNET), describe un grupo de tumores endocrinos que se originan en el páncreas y que, por lo general, su comportamiento es poco agresivo, aunque pueden resultar potencialmente malignos. Pueden ser funcionantes, exhibiendo un síndrome de hipersecreción hormonal, o no funcionantes, presentándose con síntomas no específicos e incluyen el insulinoma, el glucagonoma, el VIPoma, el somatostatinoma (SSoma), el PPoma y el síndrome de Zollinger-Ellison (ZES, o gastrinoma) y otros tumores producidos por hormonas ectópicas (tales como el GRFoma) (consulte estos términos)."},"it":{"name":"Tumore neuroendocrino del pancreas","synonyms":["Tumore neuroendocrino pancreatico","NEN ben differenziato del pancreas","NET pancreatico","PNET","Tumore neuroendocrino ben differenziato del pancreas"],"definition":"Il tumore neuroendocrino pancreatico (PNET), comprende un gruppo di tumori endocrini che si sviluppano nel pancreas. Questi tumori di solito sono silenti e benigni, ma possono anche essere maligni, possono essere funzionanti e manifestarsi con una sindrome da ipersecrezione ormonale, oppure non funzionanti e manifestarsi con sintomi aspecifici. Comprendono l'insulinoma, il glucagonoma, il VIPoma, il somatostatinoma (SSoma), il PPoma, la sindrome di Zollinger-Ellison (ZES o gastrinoma) e altri tumori che secernono ormoni ectopici (come il GRFoma) (si vedano questi termini)."},"nl":{"name":"Neuro-endocriene tumor van pancreas","synonyms":["Goed gedifferentieerd NEN van pancreas","Goed gedifferentieerd neuro-endocrien neoplasma van pancreas","Goed gedifferentieerd pancreatisch NEN","Goed gedifferentieerd pancreatisch neuro-endocrien neoplasma","Neuro-endocriene pancreastumor","Neuro-endocriene tumor van alvleesklier","PNET","Pancreatische NET","Pancreatische neuro-endocriene tumor"],"definition":"Een groep van endocriene tumoren die ontstaan in pancreas en die meestal indolent en goedaardig zijn, maar met de potentie kwaadaardig te zijn. De tumoren kunnen functioneel zijn, waarbij ze een hormonaal hypersecretiesyndroom vertonen, of niet-functioneel, waarbij ze niet-specifieke symptomen vertonen, en omvatten insulinoom, glucagonoom, VIPoom, somatostatinoom (SSoom), PPoom en syndroom van Zollinger-Ellison (ZES of gastrinoom) en andere ectopische hormoonproducerende tumoren (zoals GRFoom)."},"pt":{"name":"Tumor pancreático endócrino"},"pl":{"name":"Guz neuroendokrynny trzustki","synonyms":["PNET","Pancreatic NET","Pancreatic neuroendocrine tumor","Well-differentiated NEN of pancreas","Well-differentiated neuroendocrine neoplasm of pancreas","Well-differentiated pancreatic NEN","Well-differentiated pancreatic neuroendocrine neoplasm"]},"cs":{"name":"Endokrinní nádory pankreatu","synonyms":["Neuroendokrinní nádory pankreatu"]},"uk":{"name":"Нейроендокринна пухлина підшлункової залози","synonyms":["Панкреатична NET","Нейроендокринна пухлина підшлункової залози","PNET","Добре диференційований НЕН підшлункової залози","Добре диференційована нейроендокринна пухлина підшлункової залози","Добре диференційований панкреатичний НЕН","Добре диференційована нейроендокринна пухлина підшлункової залози"],"definition":"Pancreatic endocrine tumor, also known as pancreatic neuroendocrine tumor (PNET), describes a group of endocrine tumors originating in the pancreas that are usually indolent and benign, but may have the potential to be malignant. They can be functional, exhibiting a hormonal hypersecretion syndrome, but can be non-functional presenting with non-specific symptoms and include insulinoma, glucagonoma, VIPoma, somatostatinoma (SSoma), PPoma and Zollinger-Ellison syndrome (ZES, or gastrinoma) and other ectopic hormone producing tumors (such as GRFoma) (see these terms)."},"zh":{"name":"胰腺神经内分泌肿瘤","synonyms":["高分化胰腺神经内分泌肿瘤"],"definition":"胰腺内分泌肿瘤，又称胰腺神经内分泌肿瘤（PNET），是指起源于胰腺的一组内分泌肿瘤，通常是良性的，但可能是恶性的。它们可以是功能性的，表现为激素分泌亢进综合征，但也可以是非功能性的，表现为非特异性症状，包括胰岛素瘤、胰高血糖素瘤、血管活性肠肽瘤(VIPoma)、生长抑素瘤（SSoma）、胰多肽瘤(Ppoma)和Zollinger-Ellison综合征（ZES，或胃泌素瘤），以及其他异位激素分泌肿瘤（如GRFoma）（见该类词条）。"},"tr":{"name":"Pankreasın nöroendokrin tümörü","synonyms":["PNET","Pankreatik NET","Pankreatik nöroendokrin tümörü","İyi-diferansiye pankreatik NEN","Pankreasın iyi-diferansiye NEN'i","İyi-diferansiye pankreatik nöroendokrin neoplazmı","İyi-diferansiye pankreasın nöroendokrin neoplazmı"]}},"xrefs":[{"source":"MONDO","code":"0019954","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4305467","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"13034","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10067517","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":506052,"name":"Neuroendocrine neoplasm of pancreas","classifications":[152,193,202]}],"children":[{"orpha":506060,"name":"Functioning neuroendocrine tumor of pancreas","type":"Category"},{"orpha":506075,"name":"Non-functioning neuroendocrine tumor of pancreas","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.22,"area":"United States","status":"Validated","source":"18515795[PMID]_National Cancer OTHERitute[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.27,"area":"Japan","status":"Validated","source":"24499825[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.69,"area":"Japan","status":"Validated","source":"24499825[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.21,"area":"France","status":"Validated","source":"15016750[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.21,"area":"Europe","status":"Validated","source":"15016750[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":27.5,"area":"United States","status":"Validated","source":"23237225[PMID]_[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"1-5 / 10 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}