{"orpha":423994,"name":"Squamous cell carcinoma of the colon","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare epithelial tumour of the colon arising from squamous cells of the colorectal epithelium without the presence of squamous-lined fistulous tracts or a proximal extension of an anal squamous cell carcinoma. It usually presents with nonspecific symptoms, such as anorexia, weight loss, abdominal pain, changes of bowel habits, hematochesia or melena. Cases of severe, symptomatic hypercalcemia have been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-423994/","orphanet_url":"https://www.orpha.net/en/disease/detail/423994","names":{"fr":{"name":"Carcinome épidermoïde du côlon","definition":"Une tumeur épithéliale rare du côlon provenant des cellules squameuses de l'épithélium colorectal, en l'absence de trajet fistuleux bordé de cellules squameuses, ou de l'extension proximale d'un carcinome épidermoïde anal. Elle se manifeste habituellement par des signes non spécifiques, tels qu'une anorexie, une perte de poids, une douleur abdominale, des troubles digestifs, une hématochézie ou un méléna. Des cas d'hypercalcémie symptomatique sévère ont été rapportés."},"de":{"name":"Plattenepithelkarzinom des Dickdarms","synonyms":["Squamöses Karzinom des Kolons"],"definition":"Ein seltener epithelialer Tumor des Dickdarms, der aus Plattenepithelzellen des kolorektalen Epithels entsteht, ohne dass es zu Plattenepithel-ausgekleideten Fistelgängen oder einer proximalen Ausdehnung eines analen Plattenepithelkarzinoms kommt. Er äußert sich in der Regel durch unspezifische Symptome wie Anorexie, Gewichtsverlust, Bauchschmerzen, Veränderungen der Stuhlgewohnheiten, Hämatochezie oder Melaena. Es wurden Fälle von schwerer symptomatischer Hyperkalzämie gemeldet."},"es":{"name":"Carcinoma de células escamosas de colon","definition":"Es un tumor epitelial poco frecuente del colon que se origina en las células escamosas del epitelio colorrectal sin la presencia de tractos fistulosos revestidos de escamas, o una extensión proximal de un SCC anal. Por lo general, se presenta con síntomas inespecíficos, tales como anorexia, pérdida de peso, dolor abdominal, cambios en los hábitos intestinales, hematoquecia o melena. Se han descrito casos de hipercalcemia sintomática grave."},"it":{"name":"Carcinoma a cellule squamose del colon","definition":"Il carcinoma a cellule squamose del colon (SCC) è una neoplasia epiteliale rara che origina dalle cellule squamose dell'epitelio del colon-retto, in assenza di tratti fistolosi rivestiti di cellule squamose o di una estensione prossimale di un SCC di origine anale. Spesso i sintomi sono aspecifici e comprendono l'anoressia, la perdita di peso, il dolore addominale, i cambiamenti delle abitudini intestinali, l'ematochezia o la melena. Sono stati descritti pazienti con ipercalcemia sintomatica grave."},"nl":{"name":"Plaveiselcelcarcinoom van colon","definition":"Een zeldzame epitheliale tumor van colon, die ontstaat uit plaveiselcellen van colorectaal epitheel, zonder aanwezigheid van fistuleuze, met plaveiselcellen beklede kanalen of een proximale uitbreiding van een anaal plaveiselcelcarcinoom (PCC). Het presenteert zich meestal met aspecifieke symptomen, zoals anorexie, gewichtsverlies, abdominale pijn, veranderingen van stoelgang, hematochezie, of melena. Gevallen van ernstige, symptomatische hypercalciëmie werden ook reeds gerapporteerd."},"pt":{"name":"Carcinoma de células escamosas do cólon"},"pl":{"name":"Rak płaskonabłonkowy jelita grubego","definition":"*Rak płaskonabłonkowy jelita grubego jest rzadkim nowotworem nabłonkowym jelita grubego, wywodzącym się z płaskich komórek nabłonka odbytnicy. Nie tworzy przetok wyścielonych komórkami nabłonka płaskiego i nie szerzy się proksymalnie w przypadku lokalizacji w okolicy odbytu. Zwykle daje niespecyficzne objawy, w tym jadłowstręt, chudnięcie, zmianę rytmu wypróżnień, obecność krwi w stolcu lub stolce smoliste. Opisywano przypadki ciężkiej, objawowej hiperkalcemii.inf brsup inf brsup"},"cs":{"name":"Dlaždicobuněčný karcinom tlustého střeva"},"uk":{"name":"Плоскоклітинний рак товстої кишки","definition":"A rare epithelial tumour of the colon arising from squamous cells of the colorectal epithelium without the presence of squamous-lined fistulous tracts or a proximal extension of an anal squamous cell carcinoma. It usually presents with nonspecific symptoms, such as anorexia, weight loss, abdominal pain, changes of bowel habits, hematochesia or melena. Cases of severe, symptomatic hypercalcemia have been reported."},"zh":{"name":"结肠鳞状细胞癌","definition":"结肠鳞状细胞癌（SCC）是一种罕见的结肠上皮肿瘤，起源于结肠直肠上皮鳞状细胞不合并鳞状内瘘管或肛门SCC的近端延伸。它通常表现出非特异性症状，例如厌食，体重减轻，腹痛，排便习惯改变，咯血或黑便。据报道有严重的症状性高钙血症。"},"tr":{"name":"Kolonun skuamöz hücreli karsinomu"}},"xrefs":[{"source":"ICD-10","code":"C18.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C18.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C18.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C18.4","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C18.5","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C18.6","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C18.7","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C18.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C18.9","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B90.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018513","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333100","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":423991,"name":"Rare epithelial tumor of colon","classifications":[152,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.026,"area":"Europe","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":0.01,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.035,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.027,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.024,"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":0.017,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.01,"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":0.021,"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":0.072,"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":0.014,"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":0.035,"area":"Bulgaria","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":"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":0.022,"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":0.009,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.014,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.026,"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":0.028,"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":0.025,"area":"Ireland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.041,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.13,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.037,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}