{"orpha":506098,"name":"Neuroendocrine carcinoma of pancreas","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pancreatic NEC","Pancreatic neuroendocrine carcinoma","Poorly-differentiated NEN of pancreas","Poorly-differentiated neuroendocrine neoplasm of pancreas","Poorly-differentiated pancreatic NEN","Poorly-differentiated pancreatic neuroendocrine neoplasm"],"definition":"A rare neuroendocrine neoplasm of pancreas characterized by a high-grade malignant epithelial tumor with neuroendocrine differentiation. Based on histopathologic appearance, a small cell (composed of diffuse sheets of cells) and a large cell type (showing a nesting/trabecular pattern) are distinguished. Synaptophysin and chromogranin are positive on immunohistochemistry. The Ki-67 proliferation index is typically very high (>60 - 80%). Patients present with back pain, jaundice, and/or non-specific abdominal symptoms. Serum hormone activity is unusual. The tumor is highly aggressive with poor prognosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-506098/","orphanet_url":"https://www.orpha.net/en/disease/detail/506098","names":{"fr":{"name":"Carcinome neuroendocrine du pancréas","synonyms":["Carcinome neuroendocrine pancréatique","NEN du pancréas peu différencié","NEN pancréatique peu différencié","Néoplasme neuroendocrine du pancréas peu différencié","Néoplasme neuroendocrine pancréatique peu différencié"],"definition":"Néoplasme neuroendocrine rare du pancréas caractérisé par une tumeur épithéliale maligne de haut grade avec une différenciation neuroendocrine. L'aspect histopathologique permet de distinguer de petites cellules (qui s'agencent en nappes diffuses) et de grandes cellules (sous forme de niche ou trabéculaire). L'analyse immunohistochimique utilisant la synaptophysine et la chromogranine est positive. L'indice de prolifération Ki-67 est généralement très élevé (> 60-80 %). Les patients manifestent des douleurs dorsales, une jaunisse et/ou des symptômes abdominaux non spécifiques. Le dosage hormonal sérique est anormal. La tumeur est très agressive et le pronostic est réservé."},"de":{"name":"Neuroendokrines Karzinom des Pankreas","synonyms":["Niedrig-differenzierte NEN des Pankreas","Niedrig-differenzierte PNEN","Niedrig-differenzierte neuroendokrine Neoplasie des Pankreas","PanNEC"],"definition":"Eine seltene neuroendokrine Neoplasie der Bauchspeicheldrüse, die durch einen hochgradig malignen epithelialen Tumor mit neuroendokriner Differenzierung gekennzeichnet ist. Anhand des histopathologischen Erscheinungsbildes lassen sich ein kleinzelliger (aus diffusen Zellblättern bestehender) und ein großzelliger Typ (mit verschachteltem/trabekulärem Muster) unterscheiden. Synaptophysin und Chromogranin sind in der Immunhistochemie positiv. Der Ki-67-Proliferationsindex ist in der Regel sehr hoch (>60-80 %). Die Patienten präsentieren sich mit Rückenschmerzen, Gelbsucht und/oder unspezifischen Unterleibssymptomen. Die Hormonaktivität im Serum ist ungewöhnlich. Der Tumor ist sehr aggressiv und hat eine schlechte Prognose."},"es":{"name":"Carcinoma neuroendocrino de páncreas","synonyms":["CNE pancreático","Carcinoma neuroendocrino pancreático","NEN de páncreas pobremente diferenciada","NEN pancreática pobremente diferenciada","Neoplasia neuroendocrina de páncreas pobremente diferenciada","Neoplasia neuroendocrina pancreática pobremente diferenciada"],"definition":"Es una neoplasia neuroendocrina pancreática poco frecuente caracterizada por un tumor epitelial maligno de alto grado con diferenciación neuroendocrina. Según el aspecto histopatológico, se distingue un tipo de células pequeñas (compuesto por láminas difusas de células) y otro de células grandes (que muestran un patrón trabecular/anidado). La inmunohistoquímica es positiva para la sinaptofisina y la cromogranina. El índice de proliferación de Ki-67 suele ser muy elevado (> 60 - 80%). Los pacientes se presentan con dolor de espalda, ictericia y/o síntomas abdominales inespecíficos. La actividad hormonal sérica es inusual. El tumor es muy agresivo y el pronóstico es desfavorable."},"it":{"name":"Carcinoma neuroendocrino del pancreas","definition":"Si tratta di una neoplasia neuroendocrina rara del pancreas, caratterizzata da un tumore epiteliale maligno ad alto grado con differenziazione neuroendocrina. Sulla base dell'esame istopatologico, il tumore viene differenziato nel tipo a piccole cellule (composto da diffusi foglietti di cellule) e nel tipo a grandi cellule (caratterizzato da un aspetto nidificato/trabecolare). L'esame immunoistochimico è positivo per la sinaptofisina e la cromogranina. Di solito l'indice di proliferazione Ki-67 è molto elevato (>60 - 80%). I pazienti presentano mal di schiena, ittero e/o sintomi addominali aspecifici. L'attività ormonale nel siero è atipica. Il tumore è molto aggressivo e la prognosi è sfavorevole."},"nl":{"name":"Neuro-endocrien carcinoom van pancreas","synonyms":["Slecht gedifferentieerd pancreatisch NEN","Slecht gedifferentieerd pancreatisch neuro-endocrien neoplasma","Neuro-endocrien carcinoom van alvleesklier","Pancreatisch NEC","Pancreatisch neuro-endocrien carcinoom","Slecht gedifferentieerd NEN van pancreas","Slecht gedifferentieerd neuro-endocrien neoplasma van pancreas"],"definition":"Een zeldzaam neuro-endocrien neoplasma van pancreas, gekarakteriseerd door een hooggradige maligne epitheliale tumor met neuro-endocriene differentiatie. Op basis van histopathologisch uiterlijk worden een kleincellig type (bestaande uit diffuse cellagen) en een grootcellig type (met een genest/trabeculair patroon) onderscheiden. Immuunhistochemie is positief voor synaptofysine en chromogranine. De Ki-67 proliferatie-index is doorgaans zeer hoog (> 60-80%). Patiënten presenteren zich met rugpijn geelzucht, en/of aspecifieke abdominale symptomen. Activiteit van hormonen in serum is ongewoon. De tumor is zeer agressief en heeft een slechte prognose."},"pt":{"name":"Carcinoma neuroendócrino do pâncreas"},"pl":{"name":"Rak neuroendokrynny trzustki","synonyms":["Pancreatic NEC","Pancreatic neuroendocrine carcinoma","Poorly-differentiated NEN of pancreas","Poorly-differentiated neuroendocrine neoplasm of pancreas","Poorly-differentiated pancreatic NEN","Poorly-differentiated pancreatic neuroendocrine neoplasm"]},"cs":{"name":"Neuroendokrinní karcinom pankreatu","synonyms":["Pankreatický NEC","Pankreatický neuroendokrinní karcinom","Špatně diferencovaný pankreatický NEN","Špatně diferencovaný pankreatický neuroendokrinní novotvar","Špatně diferencovaný neuroendokrinní novotvar pankreatu"]},"uk":{"name":"Нейроендокринна карцинома підшлункової залози","synonyms":["Панкреатична NEC","Нейроендокринна карцинома підшлункової залози","Погано диференційована NEN підшлункової залози","Погано диференційована нейроендокринна пухлина підшлункової залози","Погано диференційована панкреатична NEN","Погано диференційована нейроендокринна пухлина підшлункової залози"],"definition":"A rare neuroendocrine neoplasm of pancreas characterized by a high-grade malignant epithelial tumor with neuroendocrine differentiation. Based on histopathologic appearance, a small cell (composed of diffuse sheets of cells) and a large cell type (showing a nesting/trabecular pattern) are distinguished. Synaptophysin and chromogranin are positive on immunohistochemistry. The Ki-67 proliferation index is typically very high (>60 - 80%). Patients present with back pain, jaundice, and/or non-specific abdominal symptoms. Serum hormone activity is unusual. The tumor is highly aggressive with poor prognosis."},"zh":{"name":"胰腺神经内分泌癌","synonyms":["低分化胰腺神经内分泌肿瘤"]},"tr":{"name":"Pankreas nöroendokrin karsinomu","synonyms":["Pankreatik NEC","Pankreatik nöroendokrin karsinom","Kötü-diferansiye pankreatik NEN","Pankreasın kötü-diferansiye NEN'i","Kötü diferansiye pankreatik nöroendokrin neoplazmı","Pankreasın kötü diferansiye 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":"index","validated":true,"icd11_uri":"362948338"},{"source":"MONDO","code":"0005893","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"5893","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2205506","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}