{"orpha":289682,"name":"Lymphoepithelial-like carcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Lymphoepithelial-like carcinoma is a rare, malignant epithelial tumor, composed of undifferentiated epithelial cells with dense lymphoid stroma, mimicking lymphoepithelioma. It often shows association with Epstein-Barr virus infection and can develop in various organs, such as the nasopharynx, stomach, skin, breast and lungs, among others. The presenting symptoms, as well as the radiologic features, are usually nonspecific and depend on the affected site and organ.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-289682/","orphanet_url":"https://www.orpha.net/en/disease/detail/289682","names":{"fr":{"name":"Carcinome lympho-épithélial-like","definition":"Une tumeur épithéliale maligne rare, composée de cellules épithéliales non différenciées possédant un stroma lymphoïde dense, et mimant un lympho-épithéliome. Elle est souvent associée à une infection par le virus d'Epstein-Barr, et peut se développer dans plusieurs organes, tels que, entre autres, le nasopharynx, l'estomac, la peau, la poitrine et les poumons. Habituellement, les signes de présentation et les signes radiologiques ne sont pas spécifiques et dépendent de l'organe affecté et de la région atteinte."},"de":{"name":"Karzinom, lymphoepitheliom-artiges","definition":"Das Lymphoepitheliom-artige Karzinom ist ein seltener, bösartiger Epitheltumor, der aus undifferenzierten Epithelzellen mit dichtem lymphoidem Stroma besteht und ein Lymphoepitheliom imitiert. Er zeigt häufig eine Assoziation mit einer Epstein-Barr-Virus-Infektion und kann sich in verschiedenen Organen entwickeln, wie z. B. Nasopharynx, Magen, Haut, Brust und Lunge, unter anderem. Die präsentierenden Symptome sowie die radiologischen Merkmale sind meist unspezifisch und hängen von der betroffenen Stelle und dem Organ ab."},"es":{"name":"Carcinoma similar al linfoepitelial","definition":"El carcinoma similar al linfoepitelial es un tumor epitelial maligno poco frecuente, compuesto por células epiteliales indiferenciadas con estroma linfoide denso, que simula un linfoepitelioma. A menudo se asocia con infección por el virus de Epstein-Barr y puede desarrollarse en varios órganos, tales como la nasofaringe, el estómago, la piel, los senos y los pulmones, entre otros. Los síntomas de presentación, así como las características radiológicas, suelen ser inespecíficos y dependen tanto de la localización como del órgano afectado."},"it":{"name":"Carcinoma simil-linfoepiteliale","definition":"Il carcinoma simil-linfoepiteliale è un tumore epiteliale maligno raro, composto da cellule epiteliali indifferenziate contenenti uno stroma linfoide denso, che ricorda il linfoepitelioma. Spesso si associa all'infezione da virus di Epstein-Barr e può localizzarsi in diversi organi, come il naso-faringe, lo stomaco, la cute, il seno, i polmoni. Di solito, sintomi d'esordio e le caratteristiche radiologiche sono aspecifiche e dipendono dalla localizzazione del carcinoma."},"nl":{"name":"Lymfo-epitheliaalachtig carcinoom","definition":"Lymfo-epitheliaalachtig carcinoom is een zeldzame, maligne epitheliale tumor die bestaat uit ongedifferentieerde epitheelcellen met dens lymfoïd stroma, en die lijkt op lymfo-epithelioom. Het toont vaak associatie met infectie met Epstein-Barr virus en kan ontwikkelen in verschillende organen, zoals onder andere de nasofarynx, maag, huid, borsten en longen. De aanwezige symptomen, alsook de radiologische kenmerken, zijn meestal niet specifiek en afhankelijk van de getroffen locatie en het getroffen orgaan."},"pt":{"name":"Carcinoma linfoepitelial-like"},"pl":{"name":"Rak przypominający lymphoepithelioma (lymphoepithelioma-like)"},"cs":{"name":"Karcinom podobný lymfoepiteliomu"},"uk":{"name":"Лімфоепітеліоподібна карцинома","definition":"Lymphoepithelial-like carcinoma is a rare, malignant epithelial tumor, composed of undifferentiated epithelial cells with dense lymphoid stroma, mimicking lymphoepithelioma. It often shows association with Epstein-Barr virus infection and can develop in various organs, such as the nasopharynx, stomach, skin, breast and lungs, among others. The presenting symptoms, as well as the radiologic features, are usually nonspecific and depend on the affected site and organ."},"zh":{"name":"淋巴上皮样癌","definition":"淋巴水肿样癌是一种罕见的恶性上皮性肿瘤，由未分化上皮细胞和密集的淋巴间质组成，类似淋巴上皮瘤样癌。常与EB病毒感染有关，可在各器官中发生，如鼻咽、胃、皮肤、乳房和肺等。目前临床症状及放射学特征常为非特异性，取决于受累部位和器官。"},"tr":{"name":"Lenfoepitelyal-benzeri karsinom"}},"xrefs":[{"source":"ICD-10","code":"C80.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017348","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334254","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":289651,"name":"Epstein-Barr Virus-associated carcinoma","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}