{"orpha":506090,"name":"Serotonin-producing neuroendocrine tumor of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Serotonin-producing PNET","Serotonin-producing pancreatic NET","Serotonin-producing pancreatic neuroendocrine tumor"],"definition":"A rare functioning neuroendocrine tumor of pancreas characterized by a typically well-differentiated neoplasm composed of cells expressing serotonin. Patients may present with atypical carcinoid syndrome with abdominal pain, diarrhea, weight loss, and/or flushing. Carcinoid syndrome is usually present only when there are liver metastases. The tumors tend to be larger than non-functioning tumors and are associated with a poorer prognosis because they are almost always metastatic.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-506090/","orphanet_url":"https://www.orpha.net/en/disease/detail/506090","names":{"fr":{"name":"Tumeur neuroendocrine du pancréas sécrétrice de sérotonine","synonyms":["PNET sécrétrice de sérotonine","Tumeur neuroendocrine pancréatique sécrétrice de sérotonine"],"definition":"Tumeur neuroendocrine fonctionnelle rare du pancréas, caractérisée par un néoplasme typiquement bien différencié composé de cellules exprimant la sérotonine. Les patients peuvent présenter un syndrome carcinoïde atypique avec des douleurs abdominales, des diarrhées, une perte de poids et/ou des bouffées vasomotrices. Généralement, le syndrome carcinoïde s'observe seulement en présence des métastases hépatiques. De taille plus importante par rapport aux tumeurs non fonctionnelles et étant presque toujours métastatiques, ces tumeurs sont associées à un pronostic moins favorable."},"de":{"name":"Serotonin-produzierender neuroendokriner Tumor des Pankreas","synonyms":["Serotonin-produzierender NET des Pankreas","Serotonin-produzierender PNET"],"definition":"Ein seltener funktionsfähiger neuroendokriner Tumor der Bauchspeicheldrüse, der sich durch eine typischerweise gut differenzierte Neoplasie aus serotoninexprimierenden Zellen auszeichnet. Die Patienten können ein atypisches Karzinoid-Syndrom mit Bauchschmerzen, Durchfall, Gewichtsverlust und/oder Flush aufweisen. Das Karzinoid-Syndrom tritt in der Regel nur auf, wenn Lebermetastasen vorhanden sind. Die Tumore sind in der Regel größer als nicht funktionierende Tumore und haben eine schlechtere Prognose, da sie fast immer metastasieren."},"es":{"name":"Tumor neuroendocrino de páncreas productor de serotonina","synonyms":["TNE pancreático productor de serotonina","TNEP productor de serotonina","Tumor neuroendocrino pancreático productor de serotonina"],"definition":"Es un tumor neuroendocrino pancreático funcionante poco frecuente caracterizado por una neoplasia típicamente bien diferenciada compuesta por células que expresan serotonina. Los pacientes pueden presentarse con un síndrome carcinoide atípico con dolor abdominal, diarrea, pérdida de peso y/o rubor. El síndrome carcinoide suele presentarse únicamente cuando hay metástasis hepáticas. Los tumores tienden a ser de mayor tamaño que los no funcionantes y están asociados a un peor pronóstico debido a que suelen ser metastásicos."},"it":{"name":"Tumore neuroendocrino pancreatico secernente serotonina","definition":"Si tratta di un tumore neuroendocrino funzionante raro del pancreas, caratterizzato da una neoplasia in genere bene differenziata, composta da cellule secernenti serotonina. I pazienti possono presentare una sindrome del carcinoide atipica con dolore addominale, diarrea, perdita di peso e/o vampate. Di solito la sindrome del carcinoide si manifesta solo in presenza di metastasi epatiche. Questi tumori tendono ad essere di dimensioni maggiori rispetto ai tumori non funzionanti, e si associano ad una prognosi peggiore in quanto sono quasi sempre metastatici."},"nl":{"name":"Serotonine-producerende neuro-endocriene tumor van pancreas","synonyms":["Serotonine-producerende PNET","Serotonine-producerende neuro-endocriene tumor van alvleesklier","Serotonine-producerende pancreatische NET","Serotonine-producerende pancreatische neuro-endocriene tumor"],"definition":"Een zeldzame functionele neuro-endocriene tumor van pancreas, gekarakteriseerd door een doorgaans goed gedifferentieerd neoplasma bestaande uit cellen die serotonine tot expressie brengen. Patiënten kunnen zich presenteren met atypisch carcinoïdsyndroom met abdominale pijn, diarree, gewichtsverlies, en/of blozen. Carcinoïdsyndroom is meestal enkel aanwezig wanneer er uitzaaiingen naar lever zijn. De tumoren zijn eerder groter dan niet-functionele tumoren, en zijn geassocieerd met een slechtere prognose aangezien ze nagenoeg altijd metastatisch zijn."},"pt":{"name":"Tumor neuroendócrino do pâncreas secretor de seretonina"},"pl":{"name":"Guz trzustki neuroendokrynny produkujący serotoninę","synonyms":["Serotonin-producing PNET","Serotonin-producing pancreatic NET","Serotonin-producing pancreatic neuroendocrine tumor"]},"cs":{"name":"Neuroendokrinní tumor pankreatu produkující serotonin","synonyms":["PNET produkující serotonin","Pankreatický NET produkující serotonin","Pankreatický neuroendokrinní tumor produkující serotonin"]},"uk":{"name":"Серотонінпродукуюча нейроендокринна пухлина підшлункової залози","synonyms":["Серотонінпродукуюча підшлункова залоза NET","Нейроендокринна пухлина підшлункової залози, що продукує серотонін","PNET, що виробляє серотонін"],"definition":"A rare functioning neuroendocrine tumor of pancreas characterized by a typically well-differentiated neoplasm composed of cells expressing serotonin. Patients may present with atypical carcinoid syndrome with abdominal pain, diarrhea, weight loss, and/or flushing. Carcinoid syndrome is usually present only when there are liver metastases. The tumors tend to be larger than non-functioning tumors and are associated with a poorer prognosis because they are almost always metastatic."},"zh":{"name":"5-羟色胺分泌型胰腺神经内分泌肿瘤","synonyms":["Serotonin-producing PNET","Serotonin-producing pancreatic NET","Serotonin-producing pancreatic neuroendocrine tumor"]},"tr":{"name":"Pankreas serotonin üreten nöroendokrin tümörü","synonyms":["Serotonin üreten PNET","Serotonin üreten pankreatik PNET","Serotonin üreten pankreatik nöroendokrin tümör"]}},"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":"24504","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0345933","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":506060,"name":"Functioning neuroendocrine tumor of pancreas","classifications":[152,193,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}