{"orpha":506075,"name":"Non-functioning neuroendocrine tumor of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Non-functioning PNET","Non-functioning pancreatic NET","Non-functioning pancreatic neuroendocrine tumor","Non-functioning well-differentiated NEN of pancreas","Non-functioning well-differentiated neuroendocrine neoplasm of pancreas","Non-functioning well-differentiated pancreatic NEN","Non-functioning well-differentiated pancreatic neuroendocrine neoplasm"],"definition":"A rare neuroendocrine tumor of pancreas characterized by a well-differentiated epithelial pancreatic neuroendocrine neoplasm measuring at least 0.5 cm, without distinct hormonal syndrome. Tumors <0.5 cm are called microadenomas. Microadenomatosis is the multifocal occurrence of microadenomas. Histopathologic examination shows an organoid growth pattern and expression of synaptophysin and chromogranin A on immunohistochemistry. Tumors are often discovered incidentally, or patients may present with symptoms related to local or metastatic tumor spread. Microadenomas are considered benign, while larger tumors may behave in a malignant manner with extrapancreatic spread, metastasis, or recurrence.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-506075/","orphanet_url":"https://www.orpha.net/en/disease/detail/506075","names":{"fr":{"name":"Tumeur neuroendocrine non fonctionnelle du pancréas","synonyms":["NEN du pancréas bien différencié non fonctionnel","NEN pancréatique bien différencié non fonctionnel","Néoplasme neuroendocrine du pancréas bien différencié non fonctionnel","Néoplasme neuroendocrine pancréatique bien différencié non fonctionnel","PNET non fonctionnelle"],"definition":"Tumeur neuroendocrine rare du pancréas caractérisée par un néoplasme neuroendocrine pancréatique épithélial très différencié mesurant au moins 0,5 cm, en absence de syndrome hormonal distinct. Les tumeurs de moins de 0,5 cm sont appelées microadénomes. La microadénomatose est l'apparition multifocale de microadénomes. L'examen histopathologique révèle un mode de croissance organoïde et l'expression de la synaptophysine et de la chromogranine A via l'immunohistochimie. Les tumeurs sont souvent découvertes fortuitement, ou les patients peuvent manifester des symptômes liés à une propagation locale ou métastatique de la tumeur. Les microadénomes sont considérés comme bénins, tandis que les tumeurs plus larges peuvent être malignes avec une propagation extrapancréatique, des métastases ou une récidive."},"de":{"name":"Nicht-funktioneller neuroendokriner Tumor des Pankreas","synonyms":["Nicht-funktionelle hoch-differenzierte neuroendokrine Neoplasie des Pankreas","Nicht-funktioneller PNET","Nicht-funktioneller hoch-differenzierter NET des Pankreas"],"definition":"Ein neuroendokriner Tumor der Bauchspeicheldrüse, der durch eine gut differenzierte epitheliale neuroendokrine Neoplasie der Bauchspeicheldrüse mit einer Größe von mindestens 0,5 cm gekennzeichnet ist, ohne ausgeprägtes hormonelles Syndrom. Tumore <0,5 cm werden als Mikroadenome bezeichnet. Unter Mikroadenomatose versteht man das multifokale Auftreten von Mikroadenomen. Die histopathologische Untersuchung zeigt ein organoides Wachstumsmuster und die Expression von Synaptophysin und Chromogranin A in der Immunhistochemie. Die Tumoren werden häufig zufällig entdeckt, oder die Patienten stellen sich mit Symptomen vor, die auf eine lokale oder metastatische Tumorausbreitung zurückzuführen sind. Mikroadenome gelten als gutartig, während sich größere Tumore bösartig verhalten können und sich extrapankreatisch ausbreiten, metastasieren oder rezidivieren."},"es":{"name":"Tumor neuroendocrino de páncreas no funcionante","synonyms":["NEN de páncreas no funcionante bien diferenciada","NEN pancreática no funcionante bien diferenciada","Neoplasia neuroendocrina de páncreas no funcionante bien diferenciada","Neoplasia neuroendocrina pancreática no funcionante bien diferenciada","TNE pancreático no funcionante","TNEP no funcionante","Tumor neuroendocrino pancreático no funcionante"],"definition":"Es un tumor neuroendocrino pancreático poco frecuente caracterizado por una neoplasia neuroendocrina pancreática epitelial bien diferenciada, con un tamaño superior a 0,5 cm, sin un síndrome hormonal distintivo. Los tumores menores de 0,5 cm se denominan microadenomas. La microadenomatosis es la aparición multifocal de microadenomas. El examen histopatológico revela un patrón de crecimiento organoide y expresión de sinaptofisina y de cromogranina A en la inmunohistoquímica. Los tumores a menudo se detectan de manera incidental o bien los pacientes pueden presentarse con síntomas asociados a la diseminación local o metastásica del tumor. Los microadenomas se consideran benignos, mientras que los tumores de mayor tamaño pueden presentar un comportamiento maligno con diseminación extrapancreática, metástasis o recidiva."},"it":{"name":"Tumore neuroendocrino pancreatico non funzionante","definition":"Si tratta di tumore neuroendocrino raro del pancreas, caratterizzato da una neoplasia neuroendocrina epiteliale pancreatica ben differenziata, che misura almeno 0,5 cm, in assenza della sindrome ormonale. I tumori di dimensioni inferiori a 0,5 cm sono detti microadenomi. La microadenomatosi consiste nella presenza di microadenomi multifocali. L'esame istopatologico evidenzia una crescita organoide, mentre l'esame immunoistochimico mostra l'espressione della sinaptofisina e della cromogranina A. I tumori vengono spesso scoperti casualmente, oppure quando i pazienti presentano i sintomi correlati alla diffusione locale o metastatica del tumore. I microadenomi sono considerati benigni, mentre i tumori di dimensioni maggiori possono essere maligni, con una diffusione extrapancreatica, metastasi o recidive."},"nl":{"name":"Niet-functionele neuro-endocriene tumor van pancreas","synonyms":["Niet-functioneel goed gedifferentieerd NEN van pancreas","Niet-functioneel goed gedifferentieerd neuro-endocrien neoplasma van pancreas","Niet-functioneel goed gedifferentieerd pancreatisch NEN","Niet-functioneel goed gedifferentieerd pancreatisch neuro-endocrien neoplasma","Niet-functionele PNET","Niet-functionele neuro-endocriene tumor van alvleesklier","Niet-functionele pancreatische NET","Niet-functionele pancreatische neuro-endocriene tumor"],"definition":"Een zeldzame neuro-endocriene tumor van pancreas, gekarakteriseerd door een goed gedifferentieerd epitheliaal neuro-endocrien neoplasma van pancreas van minstens 0,5 cm groot, zonder duidelijk hormonaal syndroom. Tumoren die kleiner zijn dan 0,5 cm worden microadenomen genoemd. Microadenomatose is het multifocaal optreden van microadenomen. Histopathologisch onderzoek toont een organoïd groeipatroon en expressie van synaptofysine en chromogranine A bij immuunhistochemie. Tumoren worden vaak toevallig ontdekt, of patiënten presenteren zich met symptomen gerelateerd aan lokale of metastatische verspreiding van de tumor. Microadenomen worden als goedaardig beschouwd, terwijl grotere tumoren op maligne gedragingen kunnen vertonen, zoals verspreiding, metastase, of recidief buiten de pancreas."},"pt":{"name":"Tumor neuroendócrino não-funcionante do pâncreas"},"pl":{"name":"Rak neuroendokrynny trzustki nieczynny hormonalnie","synonyms":["Rak trzustki nieczynny neuroendokrynnie"]},"cs":{"name":"Nefunkční neuroendokrinní tumor pankreatu","synonyms":["Nefunkční PNET","Nefunkční pankreatický NET","Nefunkční pankreatický neuroendokrinní tumor","Nefunkční dobře diferencovaný pankreatický NEN","Nefunkční dobře diferencovaný NEN pankreatu","Nefunkční dobře diferencovaný pankreatický neuroendokrinní novotvar","Nefunkční dobře diferencovaný neuroendokrinní novotvar pankreatu"]},"uk":{"name":"Нефункціонуюча нейроендокринна пухлина підшлункової залози","synonyms":["Нефункціонуюча панкреатична NEТ","Нефункціонуюча нейроендокринна пухлина підшлункової залози","Непрацююча PNEТ","Нефункціонуючий добре диференційований NEN підшлункової залози","Нефункціонуюча добре диференційована нейроендокринна пухлина підшлункової залози","Нефункціонуючий добре диференційований панкреатичний NEN","Нефункціонуюча добре диференційована нейроендокринна пухлина підшлункової залози"],"definition":"A rare neuroendocrine tumor of pancreas characterized by a well-differentiated epithelial pancreatic neuroendocrine neoplasm measuring at least 0.5 cm, without distinct hormonal syndrome. Tumors <0.5 cm are called microadenomas. Microadenomatosis is the multifocal occurrence of microadenomas. Histopathologic examination shows an organoid growth pattern and expression of synaptophysin and chromogranin A on immunohistochemistry. Tumors are often discovered incidentally, or patients may present with symptoms related to local or metastatic tumor spread. Microadenomas are considered benign, while larger tumors may behave in a malignant manner with extrapancreatic spread, metastasis, or recurrence."},"zh":{"name":"非功能性胰腺神经内分泌肿瘤","synonyms":["非功能性高分化胰腺神经内分泌肿瘤"]},"tr":{"name":"Pankreas fonksiyonel olmayan nöroendokrin tümörü","synonyms":["Fonksiyonel olmayan PNET","Fonksiyonel olmayan PankreastikNET","Fonksiyonel olmayan pankreatik nöroendokrin tümörü","Fonksiyonel olmayan iyi ayırt edilmiş pankreatik NEN","Pankreasın fonksiyonel olmayan iyi ayırt edilmiş NEN'i","Fonksiyonel olmayan iyi ayırt edilmiş pankreatik nöroendokrin neoplazmı","Fonksiyonel olmayan iyi ayırt edilmiş pankreasın nöroendokrin neoplazmı"]}},"xrefs":[{"source":"ICD-10","code":"C25.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0004334","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C536126","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1334977","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":97253,"name":"Neuroendocrine tumor of pancreas","classifications":[152,193,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[],"phenotypes":null}