{"orpha":398053,"name":"Adenocarcinoma of the penis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Penile adenocarcinoma"],"definition":"An extremely rare penile epithelial neoplasm, histologically composed of nests of epithelilal cells floating in lakes of extracellular, PAS-positive mucin, clinically characterized by a nonhealing ulcer or soft mass in the preputium or glans area, with itching and burning often preceding appearance of the lesion. Lymphadenopathy may indicate dissemination. Mucinous metaplasia of the penis may be a risk factor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-398053/","orphanet_url":"https://www.orpha.net/en/disease/detail/398053","names":{"fr":{"name":"Adénocarcinome de la verge","synonyms":["Adénocarcinome du pénis"],"definition":"Un néoplasme épithélial pénien extrêmement rare. Sur le plan histologique, il se compose de nids de cellules épithéliales flottant dans des étendues de mucine extracellulaire, positive pour la coloration PAS. Sur le plan clinique, il se caractérise par un ulcère non cicatrisant ou une masse molle, situés au niveau du prépuce ou du gland. Des démangeaisons et une sensation de brûlure précèdent souvent l'apparition de la lésion. Une lymphadénopathie peut indiquer une dissémination. La métaplasie mucineuse pénienne peut être un facteur de risque."},"de":{"name":"Adenokarzinom des Penis","definition":"Ein extrem seltene epitheliale Neoplasie des Penis, das histologisch aus Nestern epithelialer Zellen besteht, die in Seen aus extrazellulärem PAS-positivem Muzin schwimmen. Klinisch ist es durch ein nicht heilendes Geschwür oder eine weiche Masse im Bereich des Präputiums oder der Glans gekennzeichnet, wobei Juckreiz und Brennen oft dem Auftreten der Läsion vorausgehen. Lymphadenopathie kann auf eine Dissemination hinweisen. Eine muzinöse Metaplasie des Penis kann ein Risikofaktor sein."},"es":{"name":"Adenocarcinoma del pene","definition":"Es una neoplasia epitelial del pene muy poco frecuente compuesta histológicamente por nidos de células epiteliales que flotan en lagunas de mucina extracelular PAS-positiva. Clínicamente está caracterizado por una úlcera que no cicatriza o una masa blanda en el área prepucial o glande, con prurito y ardor que a menudo precede a la aparición de la lesión. La presencia de linfadenopatías puede indicar diseminación. La metaplasia mucinosa del pene puede constituir un factor de riesgo."},"it":{"name":"Adenocarcinoma del pene","synonyms":["Adenocarcinoma penieno"],"definition":"L'adenocarcinoma del pene è una neoplasia molto rara dell'epitelio del pene. A livello istologico, è composta da nidi di cellule epiteliali incluse in raccolte di mucina extracellulare PAS-positiva. Dal punto di vista clinico, il tumore è caratterizzato da una lesione ulcerosa che non guarisce o da una massa molle a livello del prepuzio o del glande; spesso la comparsa della lesione è preceduta da prurito e bruciore. La linfoadenopatia è indicativa della diffusione del tumore. La metaplasia mucinosa del pene può costituire un fattore di rischio."},"nl":{"name":"Adenocarcinoom van penis","synonyms":["Peniel adenocarcinoom"],"definition":"Een uiterst zeldzaam peniel epitheliaal neoplasma, dat histologisch bestaat uit nesten van epitheelcellen die ronddrijven in poelen van extracellulair, PAS-positief mucine, en dat klinisch gekarakteriseerd wordt door een niet-helende zweer of zachte massa in preputium (voorhuid) of eikel, waarvan de verschijning vaak vooraf wordt gegaan door jeuk en een brandend gevoel. Lymfadenopathie kan wijzen op disseminatie. Mucineuze metaplasie van de penis kan een risicofactor zijn."},"pt":{"name":"Adenocarcinoma do penis","synonyms":["Adenocarcinoma peniano"]},"pl":{"name":"Gruczolakorak penisa","synonyms":["Gruczolakorak prącia"]},"cs":{"name":"Adenokarcinom penisu","synonyms":["Penilní adenokarcinom"]},"uk":{"name":"Аденокарцинома статевого члена","synonyms":["Аденокарцинома статевого члена"],"definition":"An extremely rare penile epithelial neoplasm, histologically composed of nests of epithelilal cells floating in lakes of extracellular, PAS-positive mucin, clinically characterized by a nonhealing ulcer or soft mass in the preputium or glans area, with itching and burning often preceding appearance of the lesion. Lymphadenopathy may indicate dissemination. Mucinous metaplasia of the penis may be a risk factor."},"zh":{"name":"阴茎腺癌","synonyms":["阴茎腺癌"],"definition":"本病是一种极其罕见的阴茎上皮性肿瘤，组织学上由漂浮在细胞外、PAS阳性的粘液湖中的上皮细胞巢组成，临床特征是先出现出现瘙痒和灼热,后出现包皮或龟头区域出现无法愈合的溃疡或软块。淋巴结肿大可能提示播散。阴茎粘液化生可能是本病的一个危险因素。"},"tr":{"name":"Penis adenokarsinomu","synonyms":["Penil adenokarsinom"]}},"xrefs":[{"source":"ICD-10","code":"C60.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C60.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C60.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C60.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C60.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C81.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"UMLS","code":"C2212153","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":398043,"name":"Malignant tumor of penis","classifications":[185,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"22119351[PMID]_RARECARE Surveillance of Rare Cancers in Europe 2003[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.006,"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":0.006,"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":0.003,"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":0.003,"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":0.004,"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":0.002,"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":0.005,"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":0.003,"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":0.004,"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":0.005,"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":0.012,"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":0.005,"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":0.003,"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":0.025,"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":"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":0.006,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}