{"orpha":329984,"name":"Goblet cell carcinoma","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["GCC","Goblet cell adenocarcinoid","Goblet cell carcinoid","Goblet cell tumor"],"definition":"Goblet cell carcinoma (GCC) is an aggressive type of endocrine tumor of the appendix presenting equally in males and females in the fifth decade of life and manifesting with a palpable mass and abdominal pain or acute appendicitis. Metastasis to the ovaries, peritoneum or right colon has usually already occurred in half of patients at the time of diagnosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-329984/","orphanet_url":"https://www.orpha.net/en/disease/detail/329984","names":{"fr":{"name":"Carcinome à cellules caliciformes","synonyms":["Tumeur carcinoïde à cellules caliciformes","GCC","Tumeur adénocarcinoïde à cellules caliciformes"],"definition":"Le carcinome à cellules caliciformes est un type agressif de tumeur endocrine de l'appendice. Il se manifeste au cours de la cinquième décennie et affecte les hommes et les femmes de la même manière. Il se caractérise par une masse palpable et une douleur abdominale ou une appendicite aigüe. Au moment du diagnostic, des métastases se sont généralement déjà développées au niveau des ovaires, du péritoine ou du colon droit chez la moitié des patients."},"de":{"name":"Becherzellkarzinoid","synonyms":["Becherzellkarzinom","GCC","Goblet-Zell-Adenokarzinoid","Goblet-Zell-Karzinoid"]},"es":{"name":"Carcinoma de células caliciformes","synonyms":["Célula caliciforme adenocarcinoide","Célula caliciforme carcinoide","GCC","Tumor de células caliciformes"],"definition":"El tumor carcinoide de células caliciformes (CCG, por sus siglas en inglés) es un tipo agresivo de tumor endocrino del apéndice que se presenta por igual en hombres y mujeres en la quinta década de la vida y que se manifiesta con una masa palpable y dolor abdominal o apendicitis aguda. Generalmente en la mitad de los pacientes ya se han producido metástasis en los ovarios, el peritoneo o el colon derecho en el momento del diagnóstico."},"it":{"name":"Carcinoma a cellule calciformi","synonyms":["Adenocarcinoide a cellule calciformi","Carcinoide a cellule calciformi","GCC","Tumore a cellule calciformi","Tumore adenocarcinoide a cellule calciformi","Tumore carcinoide a cellule calciformi"]},"nl":{"name":"Slijmbekercelcarcinoom","synonyms":["GCC","Gobletcelcarcinoom","Slijmbekerceladenocarcinoïd","Slijmbekercelcarcinoïd","Slijmbekerceltumor"],"definition":"Slijmbekercelcarcinoom (GCC) is een agressief type endocriene tumor van de appendix (zie deze term) die evenveel voorkomt in mannen en vrouwen tijdens het vijfde levensdecennium en zich manifesteert met een voelbare knobbel en pijn in de buik of appendicitis. Op het moment van de diagnose is er bij de helft van de patiënten meestal reeds uitzaaiing opgetreden naar de eierstokken, peritoneum, of rechterdeel van de darm."},"pt":{"name":"Carcinoma de células caliciformes","synonyms":["Adenocarcinoide de células caliciformes","Carcinoide de células caliciformes","GCC","Tumor de células caliciformes"]},"pl":{"name":"Rak z komórek kubkowych","synonyms":["GCC","Gruczolakorakowiak z komórek kubkowych","Guz z komórek kubkowych","Rakowiak z komórek kubkowych"]},"cs":{"name":"Karcinom s pohárkovými buňkami","synonyms":["GCC","Adenokarcinoid s pohárkovými buňkami","Karcinoid s pohárkovými buňkami","Nádor s pohárkovými buňkami"]},"uk":{"name":"Келихоподібна карцинома","synonyms":["GCC","Келихоподібний аденокарциноїд","Келихоподібний карциноїд","Келихоподібна пухлина"],"definition":"Goblet cell carcinoma (GCC) is an aggressive type of endocrine tumor of the appendix (see this term) presenting equally in males and females in the fifth decade of life and manifesting with a palpable mass and abdominal pain or acute appendicitis. Metastasis to the ovaries, peritoneum or right colon has usually already occurred in half of patients at the time of diagnosis."},"zh":{"name":"杯状细胞癌","synonyms":["杯状细胞腺类癌"],"definition":"杯状细胞癌（GCC）是一种侵袭性的阑尾内分泌肿瘤（见该术语），男性和女性都有同样的表现，发病于50岁左右，表现为明显肿块、腹痛或急性阑尾炎。通常在该病诊断时已经发生卵巢、腹膜或右半结肠的转移。"},"tr":{"name":"Goblet hücreli karsinom","synonyms":["GCC","Goblet hücre tümörü","Goblet hücre karsinoidi","Goblet hücre adenokarsinoidi"]}},"xrefs":[{"source":"ICD-10","code":"C18.1","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"MONDO","code":"0018017","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0205695","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10414","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":100079,"name":"Neuroendocrine neoplasm of appendix","classifications":[152,193,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.025,"area":"Worldwide","status":"Not yet validated","source":"20524865[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}