{"orpha":580572,"name":"Intraductal tubulopapillary neoplasm of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["ITPN"],"definition":"A rare epithelial tumor of pancreas characterized by a solid, nodular mass growing within dilated pancreatic ducts, histologically composed of nodules of back-to-back tubular glands forming large cribriform structures, with high-grade dysplasia and ductal differentiation. There is no overt production of mucin. About half of the tumors occur in the head of the pancreas, one third involve the gland diffusely. Patients present with nonspecific symptoms including abdominal pain, vomiting, weight loss, steatorrhea, and diabetes mellitus, while obstructive jaundice is uncommon. This tumor type accounts for less than 1% of exocrine neoplasms and 3% of intraductal neoplasms of the pancreas.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-580572/","orphanet_url":"https://www.orpha.net/en/disease/detail/580572","names":{"fr":{"name":"Carcinome intracanalaire tubulopapillaire du pancréas","synonyms":["ITPN"],"definition":"Tumeur épithéliale rare du pancréas caractérisée par la présence d'une masse nodulaire solide dans les canaux pancréatiques, histologiquement composée de nodules de glandes tubuleuses fortement convergentes (« dos à dos ») à l'architecture cribriforme, ainsi que par une dysplasie de haut grade et une différenciation canalaire. Aucune production manifeste de mucine n'est observée. Près de la moitié des tumeurs se situent dans la tête du pancréas, et un tiers sont diffuses. Les patients présentent des symptômes non spécifiques, telles que des douleurs abdominales, des vomissements, une perte de poids, une stéatorrhée et un diabète sucré. L'ictère obstructif est peu courant. Ce type de tumeur représente moins de 1 % des tumeurs exocrines et 3 % des tumeurs intracanalaires du pancréas."},"de":{"name":"Intraduktale tubulopapilläre Neoplasie des Pankreas","synonyms":["ITPN"],"definition":"Ein seltener epithelialer Tumor der Bauchspeicheldrüse, der durch eine solide, knotige Gewebeverdichtung innerhalb der erweiterten Pankreasgänge gekennzeichnet ist. Histologisch besteht er aus Knötchen von röhrenförmigen Drüsen, die sich Rücken an Rücken befinden und große kribriforme Strukturen bilden, mit hochgradiger Dysplasie und duktaler Differenzierung. Es gibt keine offensichtliche Muzinproduktion. Etwa die Hälfte der Tumoren tritt im Kopf der Bauchspeicheldrüse auf, ein Drittel betrifft die Drüse in diffuser Form. Die Patienten zeigen unspezifische Symptome wie Bauchschmerzen, Erbrechen, Gewichtsverlust, Steatorrhoe und Diabetes mellitus, während eine obstruktive Gelbsucht eher selten ist. Diese Tumorart macht weniger als 1 % der exokrinen Neoplasien und 3 % der intraduktalen Neoplasien des Pankreas aus."},"es":{"name":"Neoplasia tubulopapilar intraductal de páncreas","definition":"Es un tumor epitelial de páncreas poco frecuente caracterizado por una masa sólida y nodular que crece dentro de los conductos pancreáticos dilatados. Histológicamente se compone de nódulos de glándulas tubulares adosadas entre sí formando grandes estructuras cribiformes, con displasia de alto grado y diferenciación ductal. No hay producción manifiesta de mucina. Alrededor de la mitad de los tumores se produce en la cabeza del páncreas, y un tercio afecta a la glándula de forma difusa. Los pacientes presentan síntomas inespecíficos, como dolor abdominal, vómitos, pérdida de peso, esteatorrea y diabetes mellitus, mientras que la ictericia obstructiva es poco frecuente. Este tipo de tumor representa menos del 1% de las neoplasias exocrinas y el 3% de las neoplasias intraductales de páncreas."},"it":{"name":"Neoplasia tubulopapillare intraduttale del pancreas","synonyms":["ITPN"],"definition":"Si tratta di una neoplasia epiteliale rara del pancreas, caratterizzata da una massa solida e nodulare che si sviluppa all'interno dei dotti pancreatici dilatati; dal punto di vista istologico, è composta da noduli giustapposti alle consecutivi di ghiandole tubulari, che formano ampie strutture cribriformi, con displasia di grado elevato e differenziazione duttale. Non si associa alla produzione di mucina. Circa la metà dei tumori si sviluppa nella testa del pancreas, mentre un terzo interessa la ghiandola in maniera diffusa. I pazienti presentano sintomi aspecifici, che comprendono il dolore addominale, il vomito, la perdita di peso, la steatorrea e il diabete mellito, mentre l'ittero ostruttivo è raro. Questo tipo di tumore rappresenta meno dell'1% delle neoplasie esocrine, e il 3% delle neoplasie intraduttali del pancreas."},"nl":{"name":"Intraductaal tubulopapillair neoplasma van pancreas","synonyms":["ITPN"],"definition":"Een zeldzame epitheliale tumor van pancreas, gekenmerkt door een solide, nodulaire massa die groeit in gedilateerde pancreasgangen, en histologisch bestaat uit noduli van ruggelings tegen elkaar gelegen tubulaire klieren die grote cribriforme structuren vormen, met hooggradige dysplasie en ductale differentiatie. Er is geen overduidelijke productie van mucine. Ongeveer de helft van de tumoren komt voor in de pancreaskop, terwijl een derde diffuus verdeeld is over de klier. Patiënten presenteren zich met aspecifieke symptomen, waaronder abdominale pijn, braken, gewichtsverlies, steatorroe, en diabetes mellitus, terwijl obstructieve geelzucht niet vaak voorkomt. Dit type van tumor vertegenwoordigt minder dan 1% van de exocriene neoplasmata en 3% van de intraductale neoplasmata van pancreas."},"pt":{"name":"Neoplasia do pâncreas intraductal tubulopapilar","definition":"Tumor epitelial raro do pâncreas caracterizado por uma massa nodular sólida que surge dentro dos ductos pancreáticos dilatados, histologicamente composta por nódulos de glândulas tubulares \"costas com costas\" formando grandes estruturas cribriformes, com displasia de alto grau e diferenciação ductal. Não há produção evidente de mucina. Cerca de metade dos tumores ocorrem na cabeça do pâncreas, um terço envolve a glândula de forma difusa. Os doentes apresentam sintomas inespecíficos, incluindo dor abdominal, vómitos, perda de peso, esteatorreia e diabetes mellitus, enquanto a icterícia obstrutiva é incomum. Este tipo de tumor representa menos de 1% das neoplasias exócrinas e 3% das neoplasias intraductais do pâncreas."},"pl":{"name":"Nowotwór trzustki śródprzewodowy kanalikowo- brodawkowaty","synonyms":["ITPN"]},"cs":{"name":"Intraduktální tubulopapilární novotvar slinivky břišní","synonyms":["ITPN"]},"uk":{"name":"Внутрішньопротокова тубулопапілярна пухлина підшлункової залози","synonyms":["ITPN"],"definition":"A rare epithelial tumor of pancreas characterized by a solid, nodular mass growing within dilated pancreatic ducts, histologically composed of nodules of back-to-back tubular glands forming large cribriform structures, with high-grade dysplasia and ductal differentiation. There is no overt production of mucin. About half of the tumors occur in the head of the pancreas, one third involve the gland diffusely. Patients present with nonspecific symptoms including abdominal pain, vomiting, weight loss, steatorrhea, and diabetes mellitus, while obstructive jaundice is uncommon. This tumor type accounts for less than 1% of exocrine neoplasms and 3% of intraductal neoplasms of the pancreas."},"zh":{"name":"胰腺导管内管状乳头状肿瘤","synonyms":["ITPN"]},"tr":{"name":"Pankreasın intraduktal tübülopapiller neoplazmı","synonyms":["ITPN"]}},"xrefs":[{"source":"ICD-10","code":"C25.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0034987","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2987189","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":424033,"name":"Rare epithelial tumor of pancreas","classifications":[152,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"28529742[PMID]_26464736[PMID]"}],"phenotypes":null}