{"orpha":506112,"name":"Mixed neuroendocrine and non-neuroendocrine neoplasm of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["MiNEN of pancreas","Pancreatic MiNEN","Pancreatic mixed neuroendocrine-nonneuroendocrine neoplasm"],"definition":"A rare neuroendocrine neoplasm of pancreas characterized by morphologically recognizable neuroendocrine and non-neuroendocrine components, each constituting at least 30% of the tumor volume. Based on histopathology, mixed ductal- and mixed acinar-neuroendocrine carcinomas are distinguished. Patients usually present with unspecific symptoms related to tumor growth and/or metastasis, although occurrence of Zollinger-Ellison syndrome has been reported. Resectability of the tumor is the most important prognostic factor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-506112/","orphanet_url":"https://www.orpha.net/en/disease/detail/506112","names":{"fr":{"name":"Néoplasme neuroendocrine-non neuroendocrine mixte du pancréas","synonyms":["MiNEN","MiNEN du pancréas","MiNEN pancréatique","Néoplasme neuroendocrine-nonneuroendocrine mixte pancréatique"],"definition":"Néoplasme neuroendocrine rare du pancréas caractérisé par des tissus neuroendocriniens et non neuroendocriniens identifiables par imagerie morphologique, chacun constituant au moins 30 % du volume de la tumeur. L'examen histologique permet de distinguer des carcinomes mixtes, canalaires- et mixtes à cellules acineuses -neuroendocriniens. Bien que le syndrome de Zollinger-Ellison ait été rapporté, les patients présentent généralement des symptômes non spécifiques liés à la croissance et/ou aux métastases de la tumeur. Le caractère résécable ou non de la tumeur est le facteur le plus important pour le pronostic."},"de":{"name":"Gemischte neuroendokrine/nicht neuroendokrine Neoplasie des Pankreas","synonyms":["MiNEN des Pankreas","Pankreatische MiNEN","Pankreatische gemischte neuroendokrine-nicht-neuroendokrine Neoplasie"],"definition":"Eine neuroendokrine Neoplasie des Pankreas, die durch morphologisch erkennbare neuroendokrine und nicht-neuroendokrine Komponenten gekennzeichnet ist, die jeweils mindestens 30 % des Tumorvolumens ausmachen. Aufgrund der Histopathologie werden gemischte duktale und gemischt azinär-neuroendokrine Karzinome unterschieden. Die Patienten weisen in der Regel unspezifische Symptome im Zusammenhang mit dem Tumorwachstum und/oder der Metastasierung auf, obwohl auch über das Auftreten des Zollinger-Ellison-Syndroms berichtet wurde. Die Resektabilität des Tumors ist der wichtigste prognostische Faktor."},"es":{"name":"Neoplasia mixta neuroendocrina y no neuroendocrina de páncreas","synonyms":["MiNEN de páncreas","MiNEN pancreática","Neoplasia neuroendocrina y no neuroendocrina pancreática mixta"],"definition":"Es una neoplasia neuroendocrina pancreática poco frecuente caracterizada por unos componentes neuroendocrinos y no neuroendocrinos morfológicamente reconocibles, constituyendo cada uno de ellos, al menos, el 30% del volumen tumoral. En base a la histopatología, se distinguen los carcinomas ductales- neuroendocrinos mixtos y los acinares-neuroendocrinos mixtos. Los pacientes se suelen presentar con síntomas inespecíficos asociados al crecimiento tumoral y/o metástasis, aunque se ha descrito la aparición del síndrome de Zollinger-Ellison. La resección tumoral es el factor pronóstico más importante."},"it":{"name":"Neoplasia mista neuroendocrina-non neuroendocrina del pancreas","synonyms":["MiNEN del pancreas","MiNEN pancreatica","Neoplasia mista neuroendocrina-non neuroendocrina pancreatica"],"definition":"Si tratta di una neoplasia neuroendocrina rara del pancreas, caratterizzata da componenti neuroendocrine e non-neuroendocrine morfologicamente riconoscibili, ciascuno delle quali costituisce almeno il 30% del volume del tumore. L'esame istologico permette di differenziare i carcinomi misti duttali-neuroendocrini e misti acinari-neuroendocrini. Di solito i pazienti presentano sintomi aspecifici correlati alla crescita del tumore e/o alle metastasi, mentre alcuni presentano la sindrome di Zollinger-Ellison. La resecabilità del tumore costituisce il fattore prognostico più importante."},"nl":{"name":"Gemengd neuro-endocrien en niet-neuro-endocrien neoplasma van pancreas","synonyms":["Gemengd neuro-endocrien en niet-neuro-endocrien neoplasma van alvleesklier","MiNEN van pancreas","Pancreatisch MiNEN","Pancreatisch gemengd neuro-endocrien - niet-neuro-endocrien neoplasma"],"definition":"Een zeldzaam neuro-endocrien neoplasma van pancreas, gekarakteriseerd door morfologisch herkenbare neuro-endocriene en niet-neuro-endocriene componenten, die elk minstens 30% van het volume van de tumor uitmaken. Op basis van histopathologie worden gemengd ductaal en neuro-endocrien carcinoom en gemengd acinair en neuro-endocrien carcinoom onderscheiden. Patiënten presenteren zich meestal met aspecifieke symptomen gerelateerd aan groei en/of metastase van de tumor, hoewel optreden van syndroom van Zollinger-Ellison ook reeds werd gerapporteerd. Resectabiliteit van de tumor is de belangrijkste prognostische factor."},"pt":{"name":"Neoplasia neuroendócrina e não-neuroendócrina mista do pâncreas"},"pl":{"name":"Nowotwór trzustki mieszany neuroendokrynny i nie-neuroendokrynny","synonyms":["MiNEN of pancreas","Pancreatic MiNEN","Pancreatic mixed neuroendocrine-nonneuroendocrine neoplasm"]},"cs":{"name":"Smíšený neuroendokrinní a nonneuroendokrinní novotvar pankreatu","synonyms":["Pankreatický MiNEN","MiNEN pankreatu","Pankreatický smíšený neuroendokrinní a nonneuroendokrinní novotvar"]},"uk":{"name":"Змішана нейроендокринна та ненейроендокринна пухлина підшлункової залози","synonyms":["МіНен підшлункової залози","Підшлункова залоза MiNEN","Змішана нейроендокринно-нейроендокринна пухлина підшлункової залози"],"definition":"A rare neuroendocrine neoplasm of pancreas characterized by morphologically recognizable neuroendocrine and non-neuroendocrine components, each constituting at least 30% of the tumor volume. Based on histopathology, mixed ductal- and mixed acinar-neuroendocrine carcinomas are distinguished. Patients usually present with unspecific symptoms related to tumor growth and/or metastasis, although occurrence of Zollinger-Ellison syndrome has been reported. Resectability of the tumor is the most important prognostic factor."},"zh":{"name":"胰腺混合性神经内分泌-非神经内分泌肿瘤","synonyms":["MiNEN of pancreas","Pancreatic MiNEN","Pancreatic mixed neuroendocrine-nonneuroendocrine neoplasm"]},"tr":{"name":"Pankreasın karma nöroendokrin-nöroendokrin olmayan neoplazmı","synonyms":["Pankreatik MiNEN","MiNEN pankreas","Pankreatik karma nöroendokrin-nöroendokrin olmayan neoplazm"]}},"xrefs":[{"source":"ICD-10","code":"C25.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0044727","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"44727","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5568577","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":506052,"name":"Neuroendocrine neoplasm of pancreas","classifications":[152,193,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}