{"orpha":424065,"name":"Pancreatic solid pseudopapillary neoplasm","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Frantz tumor","SPN","Solid pseudopapillary carcinoma of pancreas","Solid pseudopapillary neoplasm of the pancreas"],"definition":"A rare carcinoma of the pancreas characterized by a variable combination of nonspecific signs and symptoms, such as abdominal pain, jaundice, abdominal fullness, anorexia, nausea, vomiting, and weight loss. One-third of the patients are asymptomatic. The tumor has low malignant potential, but can invade locally.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424065/","orphanet_url":"https://www.orpha.net/en/disease/detail/424065","names":{"fr":{"name":"Tumeur pseudopapillaire et solide du pancréas","synonyms":["Carcinome pseudo-papillaire et solide du pancréas","Carcinome pseudo-papillaire et solide pancréatique","TPPSP","Tumeur de Frantz","Tumeur pseudo-papillaire et solide pancréatique"],"definition":"Carcinome rare du pancréas caractérisé par une association inconstante de signes et de symptômes non spécifiques, tels que douleurs abdominales, ictère, plénitude abdominale, anorexie, nausées, vomissements et perte de poids. Un tiers des patients sont asymptomatiques. La tumeur a un faible potentiel de malignité, mais peut s'étendre localement."},"de":{"name":"Solides pseudopapilläres Karzinom des Pankreas","synonyms":["Frantz-Tumor","Pankreatisches solides pseudopapilläres Karzinom","SPN","Solid-pseudopapilläre Neoplasie des Pankreas","Solide pseudopapilläre Neoplasie des Pankreas"],"definition":"Ein seltenes Karzinom der Bauchspeicheldrüse, das durch eine variable Kombination unspezifischer Anzeichen und Symptome wie Bauchschmerzen, Gelbsucht, Völlegefühl, Appetitlosigkeit, Übelkeit, Erbrechen und Gewichtsverlust gekennzeichnet ist. Ein Drittel der Patienten ist asymptomatisch. Der Tumor hat ein geringes malignes Potenzial, kann aber lokal infiltrieren."},"es":{"name":"Neoplasia sólida pseudopapilar pancreática","synonyms":["Carcinoma pancreático sólido pseudopapilar","Neoplasia sólida pseudopapilar de páncreas"],"definition":"Es un carcinoma poco frecuente del páncreas caracterizado por una combinación variable de signos y síntomas inespecíficos, tales como dolor abdominal, ictericia, plenitud abdominal, anorexia, náuseas, vómitos y pérdida de peso. Un tercio de los pacientes son asintomáticos. El tumor tiene un bajo potencial maligno, aunque con posible invasión local."},"it":{"name":"Carcinoma pancreatico pseudopapillare solido","definition":"Il carcinoma pseudopapillare solido del pancreas è un carcinoma pancreatico raro, caratterizzato da segni clinici aspecifici, che comprendono il dolore addominale, l'ittero, il senso di pienezza dell'addome, l'anoressia, la nausea, il vomito e la perdita di peso. Un terzo dei pazienti è asintomatico. Il tumore ha un basso potenziale di evoluzione maligna, ma può essere localmente invasivo."},"nl":{"name":"Solide pseudopapillair neoplasma van pancreas","synonyms":["Frantz-tumor","SPN","Solide pseudopapillair carcinoom van pancreas","Solide pseudopapillair neoplasma van alvleesklier"],"definition":"Een zeldzaam carcinoom van pancreas, gekenmerkt door een variabele combinatie van aspecifieke verschijnselen en symptomen, zoals abdominale pijn, geelzucht, vol gevoel van abdomen, anorexie, misselijkheid, braken en gewichtsverlies. Een derde van de patiënten is asymptomatisch. De tumor heeft een laag maligne potentieel, maar kan lokaal invasief zijn."},"pt":{"name":"Carcinoma sólido pseudopapilar do pâncreas","synonyms":["SPN","Tumor Frantz","Neoplasia sólida pseudopapilar do pâncreas"]},"pl":{"name":"Lity rzekomobrodawkowaty rak trzustki","synonyms":["Frantz tumor","SPN","Solid pseudopapillary carcinoma of pancreas","Solid pseudopapillary neoplasm of the pancreas"],"definition":"*Lity rzekomobrodawkowaty rak trzustki jest rzadkim nowotworem trzustki, który charakteryzuje się zmienną kombinacją niespecyficznych objawów przedmiotowych i podmiotowych, takich jak ból brzucha, żółtaczka, uczucie pełności brzucha, jadłowstręt, nudności, wymioty i utrata masy ciała. Jedna trzecia pacjentów nie wykazuje objawów. Guz jest potencjalnie mało złośliwy, ale może naciekać lokalne struktury."},"cs":{"name":"Solidní pseudopapilární karcinom pankreatu","synonyms":["Pankreatický solidní pseudopapilární karcinom"]},"uk":{"name":"Солідна псевдопапілярна карцинома підшлункової залози","synonyms":["Солідна псевдопапілярна карцинома підшлункової залози","Солідна псевдопапілярна пухлина підшлункової залози"],"definition":"A rare carcinoma of the pancreas characterized by a variable combination of nonspecific signs and symptoms, such as abdominal pain, jaundice, abdominal fullness, anorexia, nausea, vomiting, and weight loss. One-third of the patients are asymptomatic. The tumor has low malignant potential, but can invade locally."},"zh":{"name":"胰腺实性假乳头状癌","synonyms":["胰腺实性假乳头状癌"],"definition":"实体胰腺假乳头状癌是一种罕见的胰腺癌。其临床表现包括一种或多种非特异性体征和症状，例如腹痛，黄疸，腹胀，厌食，恶心，呕吐和体重减轻。约三分之一的患者无症状。该肿瘤具有恶性程度较低，但可局部侵袭。"},"tr":{"name":"Pankreasın katı psödopapiller karsinomu","synonyms":["Pankreatik solid psödopapiller karsinom","Pankreasın katı psödopapiller neoplazmı"]}},"xrefs":[{"source":"ICD-10","code":"C25.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C25.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C25.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C25.7","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C25.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C10.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"410394500"},{"source":"MONDO","code":"0018525","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1336029","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":217074,"name":"Rare carcinoma of pancreas","classifications":[152,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.003,"area":"Austria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.008,"area":"Belgium","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.003,"area":"Croatia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.011,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.005,"area":"France","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.004,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.005,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.003,"area":"Norway","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.009,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.002,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.001,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.003,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}