{"orpha":314022,"name":"Gastric adenocarcinoma and proximal polyposis of the stomach","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Familial fundic gland polyposis with gastric cancer","GAPPS"],"definition":"Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is a rare hereditary gastric cancer characterized by proximal gastric polyposis and increased risk of early-onset, intestinal-type adenocarcinoma of the gastric body, with no duodenal or colorectal polyposis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-314022/","orphanet_url":"https://www.orpha.net/en/disease/detail/314022","names":{"fr":{"name":"Adénocarcinome gastrique et polypose proximale de l'estomac","synonyms":["GAPPS","Polypose familiale des glandes fundiques avec cancer gastrique"],"definition":"Cancer gastrique héréditaire rare caractérisé par une polypose gastrique proximale et un risque accru d'adénocarcinome du corps gastrique de type intestinal à début précoce, en l'absence de polypose duodénale ou colorectale."},"de":{"name":"Gastrisches Adenokarzinom und proximale Polyposis des Magens","synonyms":["Familiäre Drüsenkörperzysten mit Magenkarzinom","GAPPS-Syndrom"]},"es":{"name":"Adenocarcinoma gástrico y poliposis proximal del estómago","synonyms":["GAPPS","Poliposis familiar de glándulas fúndicas con cáncer gástrico"],"definition":"Es un cáncer gástrico, hereditario y poco frecuente, caracterizado por poliposis gástrica proximal y un mayor riesgo de adenocarcinoma del cuerpo gástrico, de tipo intestinal e inicio temprano, sin poliposis duodenal ni colorrectal."},"it":{"name":"Adenocarcinoma gastrico con poliposi prossimale dello stomaco","synonyms":["GAPPS","Poliposi familiare delle ghiandole fundiche con cancro gastrico"],"definition":"L'adenocarcinoma gastrico con poliposi prossimale dello stomaco (GAPPS) è una neoplasia gastrica ereditaria rara, caratterizzata da poliposi gastrica prossimale e rischio di adenocarcinoma di tipo intestinale ad esordio precoce del corpo dello stomaco, in assenza di poliposi duodenale o colorettale."},"nl":{"name":"Gastrisch adenocarcinoom en proximale polyposis van maag","synonyms":["Familiale polyposis van fundusklieren met maagkanker","GAPPS"],"definition":"Gastrisch adenocarcinoom en proximale polypose van de maag (GAPPS) is een zeldzame, erfelijke maagkanker die gekarakteriseerd wordt door proximale gastrische polypose en een verhoogd risico op vroeg aanvangende, intestinaal-type adenocarcinoom van het maaglichaam, zonder duodenale of colorectale polypose."},"pt":{"name":"Adenocarcinoma gástrico e polipose proximal do estômago","synonyms":["GAPPS","Polipose familiar da glândula fúndica com cancro gástrico"]},"pl":{"name":"Gruczolakorak żołądka i proksymalna polipowatość żołądka","synonyms":["GAPPS"],"definition":"*Gruczolakorak żołądka i proksymalna polipowatość żołądka (ang. Gastric Adenocarcinoma and Proximal Polyposis of the Stomach, GAPPS) to rzadki dziedziczny rak żołądka, który charakteryzuje się polipowatością proksymalnego odcinka żołądka i zwiększonym ryzykiem wczesnego rozwoju gruczolakoraka żołądka o typie jelitowym, bez polipowatości dwunastnicy lub jelita grubego."},"cs":{"name":"Adenokarcinom a proximální polypóza žaludku","synonyms":["Familiární polypóza žlázek fundu s karcinomem žaludku","GAPPS"]},"uk":{"name":"Аденокарцинома шлунка та проксимальний поліпоз шлунка","synonyms":["Сімейний поліпоз фундальної залози з раком шлунка","GAPPS"],"definition":"Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is a rare hereditary gastric cancer characterized by proximal gastric polyposis and increased risk of early-onset, intestinal-type adenocarcinoma of the gastric body, with no duodenal or colorectal polyposis."},"zh":{"name":"胃腺癌和胃近端息肉","synonyms":["家族性基底腺息肉伴胃癌"],"definition":"胃腺癌和胃近端息肉病（GAPPS）是一种罕见的遗传性胃癌，以胃近端息肉病为特征，并增加胃体肠型腺癌的早期发病风险，不伴有十二指肠或结肠息肉病。"},"tr":{"name":"Mide adenokarsinomu ve midenin proksimal polipozu","synonyms":["GAPPS","Mide kanseri ile seyreden ailesel fundik bezi polipozu"]}},"xrefs":[{"source":"OMIM","code":"619182","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C16.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B72.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017790","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1868001","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":423776,"name":"Hereditary gastric cancer","classifications":[152,202]},{"orpha":140162,"name":"Inherited cancer-predisposing syndrome","classifications":[156,202]}],"children":[],"genes":[{"symbol":"APC","name":"APC regulator of Wnt signaling pathway","gene_type":"gene with protein product","association":"Disease-causing germline mutation(s) in","status":"Assessed","source":"27087319[PMID]","hgnc":"583","ensembl":"ENSG00000134982","omim":"611731","loci":["5q22.2"]}],"genes_via_subtypes":[],"inheritance":["Autosomal dominant"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":28.0,"area":"Worldwide","status":"Validated","source":"21813476[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}