{"orpha":418945,"name":"Carcinoma of esophagus, salivary gland type","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Esophageal carcinoma, salivary gland type"],"definition":"A rare gastroesophageal tumor characterized by a typically submucosal tumor occurring usually in the middle to distal esophagus and histologically characterized as either mucoepidermoid (intimate mixture of mucus, intermediate, and epidermoid cells) or as adenoid cystic carcinoma (biphasic admixture of duct&#8208;lining epithelial and myoepithelial cells with tubular, cribriform, solid, or basaloid growth patterns). Patients may be asymptomatic or may present with progressive dysphagia, heartburn, retrosternal pain and/or weight loss.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-418945/","orphanet_url":"https://www.orpha.net/en/disease/detail/418945","names":{"fr":{"name":"Carcinome de l'oesophage type glande salivaire","definition":"Tumeur gastro-oesophagienne rare caractérisée par une tumeur généralement sous-muqueuse, affectant habituellement la partie située entre le tiers moyen et distal de l'oesophage, dont les caractéristiques histologiques sont soit celles d'un carcinome mucoépidermoïde (mélange de cellules productrices de mucus, de cellules épidermoïdes et de cellules intermédiaires) soit celles d'un carcinome adénoïde kystique (mélange biphasique de cellules myoépithéliales et de cellules épithéliales canalaires au schéma de croissance tubulaire, cribriforme, solide ou basaloïde). Les patients peuvent être asymptomatiques ou présenter une dysphagie progressive, des brûlures d'estomac, une douleur rétrosternale et/ou une perte de poids."},"de":{"name":"Ösophaguskarzinom vom Speicheldrüsentyp","synonyms":["Speiseröhrenkrebs vom Speicheldrüsentyp"]},"es":{"name":"Carcinoma de esófago tipo glándulas salivales","synonyms":["Carcinoma esofágico tipo glándulas salivales"],"definition":"Es un tumor gastroesofágico poco frecuente caracterizado por una masa submucosa típica que, por lo general, se localiza entre el esófago medio y distal y que se caracteriza histológicamente bien como un carcinoma mucoepidermoide (mezcla íntima de contenido mucoso, intermedio y de células epidermoides) o como un carcinoma adenoide quístico (mezcla bifásica de células epiteliales de revestimiento de los conductos y mioepiteliales con un patrón de crecimiento tubular, cribriforme, sólido o basaloide). Los pacientes pueden estar asintomáticos o presentar disfagia progresiva, pirosis, dolor retroesternal y/o pérdida de peso."},"it":{"name":"Carcinoma dell'esofago, tipo ghiandola salivare","synonyms":["Carcinoma esofageo, tipo ghiandola salivare"],"definition":"È un tumore gastroesofageo raro, consistente in una di neoplasia sottomucosale, di solito localizzata nell'esofago medio-distale. Dal punto di vista istologico, si tratta di un carcinoma mucoepidermoide (che contiene cellule mucipare, intermedie ed epidermoidi) oppure di un carcinoma adenoido-cistico (commistione bifasica di cellule epiteliali e mioepiteliali di rivestimento del dotto con crescita tubulare, cribriforme, solida o basaloide). I pazienti possono essere asintomatici oppure presentare disfagia progressiva, bruciore allo stomaco, dolore retrosternale e/o perdita di peso."},"nl":{"name":"Carcinoom van oesofagus, speekselklier-type","synonyms":["Carcinoom van slokdarm, speekselklier-type","Oesofageaal carcinoom, speekselklier-type"],"definition":"Een zeldzame gastro-oesofageale tumor gekarakteriseerd door een doorgaans submucosale tumor die meestal voorkomt in het middelste tot distale deel van de slokdarm en die histologisch gekarakteriseerd wordt als muco-epidermoïd (innig mengsel van muceuze, intermediaire en epidermoïde cellen) of als adenoïd cystisch carcinoom (bifasisch mengsel van epitheel- en myo-epitheelcellen die het kanaal bekleden met tubulaire, cribriforme, solide, of basaloïde groeipatronen). Patiënten kunnen asymptomatisch zijn of kunnen progressieve dysfagie, maagzuur, retrosternale pijn en/of gewichtsverlies vertonen."},"pt":{"name":"Carcinoma do esófago, tipo glândulas salivares"},"pl":{"name":"Rak przełyku, typ gruczołu ślinowego","synonyms":["Esophageal carcinoma, salivary gland type"]},"cs":{"name":"Karcinom jícnu, typ slinné žlázy","synonyms":["Ezofageální karcinom, typ slinné žlázy"]},"uk":{"name":"Карцинома стравоходу, тип слинної залози","synonyms":["Карцинома стравоходу за типом слинної залози"],"definition":"A rare gastroesophageal tumor characterized by a typically submucosal tumor occurring usually in the middle to distal esophagus and histologically characterized as either mucoepidermoid (intimate mixture of mucus, intermediate, and epidermoid cells) or as adenoid cystic carcinoma (biphasic admixture of duct&#8208;lining epithelial and myoepithelial cells with tubular, cribriform, solid, or basaloid growth patterns). Patients may be asymptomatic or may present with progressive dysphagia, heartburn, retrosternal pain and/or weight loss."},"zh":{"name":"唾液腺型食管癌","synonyms":["唾液腺型食管癌"],"definition":"罕见的胃食管肿瘤，特征为典型的粘膜下肿瘤，通常发生在食管中部至远端，组织学上表现为粘液性表皮样瘤（粘液、间质、表皮样细胞紧密混合物）或腺样囊性癌（双相混合的导管，表皮和导管肌上皮细胞，网状、实心或基底细胞样的生长模式）。患者可能无症状或可能出现进行性吞咽困难，烧心，胸骨后疼痛和/或体重减轻。"},"tr":{"name":"Yemek borusu karsinomu, tükrük bezi tipi","synonyms":["Özofageal karsinomu, tükrük bezi tipi"]}},"xrefs":[{"source":"ICD-10","code":"C15.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C15.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C15.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C15.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C15.4","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C15.5","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C15.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B70.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018480","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5190854","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":423781,"name":"OBSOLETE: Carcinoma of stomach, salivary gland type","status":"inactive"}],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":70482,"name":"Carcinoma of esophagus","classifications":[152,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.004,"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":"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":0.017,"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":0.002,"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":0.007,"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":0.002,"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":0.004,"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":0.009,"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":0.001,"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":"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":0.013,"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":0.006,"area":"Slovenia","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":"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":0.005,"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":0.008,"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":0.004,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}