{"orpha":418959,"name":"Squamous cell carcinoma of the stomach","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Gastric squamous cell carcinoma"],"definition":"A rare epithelial tumour of stomach, defined histopathologically as keratinizing cell masses with pearl formation, mosaic pattern of cell arrangement, intercellular bridges, and high concentrations of sulphydryl or disulphide bonds, arising directly from gastric mucosa, without esophageal involvement. It is characterized by preferential location in the upper third of the stomach, high probability of lymphovascular and serosal invasion and late onset of clinical symptoms associated with poor prognosis including nonspecific symptoms of abdominal pain, dysphagia, vomiting, melena or hematochezia, haematemesis and weight loss.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-418959/","orphanet_url":"https://www.orpha.net/en/disease/detail/418959","names":{"fr":{"name":"Carcinome épidermoïde de l'estomac","synonyms":["Carcinome épidermoïde gastrique"],"definition":"Une tumeur épithéliale rare de l'estomac, définie sur le plan histopathologique par des masses de cellules kératinisées en forme de perle, un arrangement cellulaire en forme de mosaïque, des ponts intercellulaires et de fortes concentrations de ponts disulfures ou sulphydryles, affectant directement la muqueuse gastrique sans atteinte oesophagienne. La tumeur se trouve préférentiellement au niveau du tiers supérieur de l'estomac. Elle est associée à un fort risque d'invasion séreuse et lymphovasculaire. Les signes cliniques tardifs associés à un mauvais pronostic incluent des symptômes non spécifiques de douleur abdominale, une dysphagie, des vomissements, un méléna ou une hématochézie, une hématémèse et une perte de poids."},"de":{"name":"Plattenepithelkarzinom des Magens","synonyms":["Squamöses Karzinom des Magens"],"definition":"Ein seltener epithelialer Magentumor, der histopathologisch definiert ist als keratinisierende Zellmasse mit Perlenbildung, mosaikartige Zellanordnung, interzellulären Brücken und hohen Konzentrationen von Sulfhydryl- oder Disulfidbindungen, die direkt von der Magenschleimhaut ausgehen, ohne Beteiligung des Ösophagus. Es tritt bevorzugt im oberen Drittel des Magens auf und zeigt eine hohe Wahrscheinlichkeit einer lymphovaskulären und serosalen Invasion. Die klinischen Symptome treten typischerweise spät auf und sind mit einer schlechten Prognose verbunden. Unspezifische Symptome sind Bauchschmerzen, Dysphagie, Erbrechen, Meläna oder Hämatochezie, Hämatemesis und Gewichtsverlust."},"es":{"name":"Carcinoma de células escamosas del estómago","synonyms":["Carcinoma gástrico de células escamosas"],"definition":"Es un tumor epitelial del estómago poco frecuente definido histopatológicamente por la presencia de masas celulares queratinizantes con formación de gránulos, patrón en mosaico de la disposición celular, puentes intercelulares y elevadas concentraciones de enlaces sulfhidrilo o disulfuro, que surgen directamente de la mucosa gástrica, sin afectación esofágica. Se caracteriza por una ubicación preferencial en el tercio superior del estómago, elevada probabilidad de invasión linfovascular y serosa, y aparición tardía de síntomas clínicos asociados con un pronóstico desfavorable que incluye hallazgos inespecíficos tales como dolor abdominal, disfagia, vómitos, melena o hematoquezia, hematemesis y pérdida de peso."},"it":{"name":"Carcinoma a cellule squamose dello stomaco","synonyms":["Carcinoma gastrico a cellule squamose"],"definition":"Il carcinoma a cellule squamose dello stomaco è una neoplasia epiteliale rara, caratterizzata istologicamente da gruppi di cellule cheratinizzanti che formano perle, da una organizzazione cellulare a mosaico, da ponti intercellulari e concentrazioni elevate di legami di sulfidrile e disolfuro, che originano direttamente dalla mucosa gastrica, senza interessare l'esofago. Di solito è localizzato nel terzo superiore dello stomaco con elevata probabilità di invasione linfovascolare e serosale. L'insorgenza tardiva dei segni clinici, alcuni dei quali aspecifici (dolore addominale, disfagia, vomito, melena o ematochezia, ematemesi e perdita di peso) comporta una prognosi sfavorevole."},"nl":{"name":"Plaveiselcelcarcinoom van maag","synonyms":["Gastrisch plaveiselcelcarcinoom"],"definition":"Een zeldzame epitheliale tumor van maag, histopathologisch gedefinieerd als keratiniserende celmassa's met parelvorming, schikking van cellen volgens een mozaïekpatroon, intercellulaire bruggen, en hoge concentraties van sulfhydryl- of disulfidebindingen, rechtstreeks ontstaan uit maagslijmvlies, zonder betrokkenheid van oesofagus. Het wordt gekenmerkt door een preferentiële locatie in het bovenste derde deel van maag, hoge kans op lymfovasculaire en serosale invasie, en late aanvang van klinische symptomen geassocieerd met ongunstige prognose, waaronder aspecifieke symptomen als abdominale pijn, dysfagie, braken, melena of hematochezie, hematemese en gewichtsverlies."},"pt":{"name":"Carcinoma de células escamosas do estômago","synonyms":["Carcinoma gástrico de células escamosas"]},"pl":{"name":"Płaskonabłonkowy rak żołądka","synonyms":["Gastric squamous cell carcinoma"]},"cs":{"name":"Dlaždicobuněčný karcinom žaludku","synonyms":["Gastrický dlaždicobuněčný karcinom"]},"uk":{"name":"Плоскоклітинний рак шлунка","synonyms":["Плоскоклітинний рак шлунка"],"definition":"A rare epithelial tumour of stomach, defined histopathologically as keratinizing cell masses with pearl formation, mosaic pattern of cell arrangement, intercellular bridges, and high concentrations of sulphydryl or disulphide bonds, arising directly from gastric mucosa, without esophageal involvement. It is characterized by preferential location in the upper third of the stomach, high probability of lymphovascular and serosal invasion and late onset of clinical symptoms associated with poor prognosis including nonspecific symptoms of abdominal pain, dysphagia, vomiting, melena or hematochezia, haematemesis and weight loss."},"zh":{"name":"胃鳞状细胞癌","synonyms":["胃鳞状细胞癌"],"definition":"胃鳞状细胞癌是一种罕见的胃上皮肿瘤，在组织病理学上被定义为角质化的细胞团合并角化珠形成，细胞镶嵌排列，有细胞间桥，高度巯基或二硫键结合，直接起源于胃粘膜，无食道受累。其特点是：胃的上三分之一的位置居多，淋巴管和浆膜浸润的可能性较高，以及与不良预后相关的临床症状出现较晚，包括腹痛，吞咽困难，呕吐，黑便或便血，呕血和体重减轻等非特异性症状。"},"tr":{"name":"Midenin skuamöz hücreli karsinomu","synonyms":["Mide skuamöz hücreli karsinom"]}},"xrefs":[{"source":"ICD-10","code":"C16.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C16.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C16.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C16.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C16.4","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C16.5","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C16.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B72.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0006230","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333789","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":423771,"name":"Rare carcinoma of stomach","classifications":[152,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"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":"1-9 / 1 000 000","value":0.115,"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":0.079,"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":0.043,"area":"Iceland","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":0.207,"area":"Ireland","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":0.1,"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":0.401,"area":"Latvia","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":0.137,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.031,"area":"Malta","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.046,"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":0.04,"area":"Poland","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":0.115,"area":"Portugal","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":0.111,"area":"Slovakia","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":0.1,"area":"Slovenia","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":0.128,"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":0.051,"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":0.053,"area":"Netherlands","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":0.18,"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":0.034,"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":0.092,"area":"Czech Republic","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":0.176,"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":0.065,"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":0.08,"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":0.063,"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":0.025,"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":0.08,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}