{"orpha":398058,"name":"Squamous cell carcinoma of the penis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Penile squamous cell carcinoma"],"definition":"A rare urogenital tumor characterized by origin from squamous epithelial cells of the penis, most commonly the glans or inner surface of the prepuce. Macroscopically, the tumors can appear either papillary or flat and ulcerating. Histological subtypes include usual squamous cell carcinoma as the most common type, as well as basaloid, warty, verrucous, papillary, and mixed carcinomas. Patients may initially be asymptomatic but present with itching, bleeding, discharge, foul odor, and pain, as the disease progresses. Regional lymph node involvement is common, while distant metastases occur only late in the disease. Risk factors include HPV infection, smoking, poor hygiene, and HIV infection. Neonatal circumcision is implicated as strongly protective.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-398058/","orphanet_url":"https://www.orpha.net/en/disease/detail/398058","names":{"fr":{"name":"Carcinome épidermoïde de la verge","synonyms":["Carcinome épidermoïde du pénis"],"definition":"Tumeur urogénitale rare prenant naissance dans les cellules épithéliales squameuses de la verge, la plupart du temps au niveau du gland ou de la surface interne du prépuce. Sur le plan macroscopique, les tumeurs prennent la forme de lésions papillaires ou de lésions plates et ulcérées. Les sous-types histologiques comprennent le carcinome épidermoïde habituel qui représente le type le plus courant de cancer de la verge, ainsi que les carcinomes basaloïdes, verruqueux, papillaires et mixtes. Dans un premier temps, les patients peuvent être asymptomatiques mais, à mesure que la maladie progresse, les signes suivants sont observés : démangeaisons, saignements, écoulements, odeur fétide et douleurs. Les ganglions lymphatiques régionaux sont généralement atteints, alors que les métastases à distance ne surviennent qu'à un stade tardif de la maladie. Les facteurs de risque sont une infection à HPV, le tabagisme, une mauvaise hygiène et une infection par le VIH. La circoncision néonatale est un important facteur protecteur."},"de":{"name":"Plattenepithelkarzinom des Penis","synonyms":["Squamöses Karzinom des Penis"],"definition":"Ein seltener urogenitaler Tumor, der von Plattenepithelzellen des Penis ausgeht, am häufigsten von der Eichel oder der Innenseite der Vorhaut. Makroskopisch können die Tumoren entweder papillär oder flach und ulzerierend erscheinen. Zu den histologischen Subtypen gehören das gewöhnliche Plattenepithelkarzinom als häufigster Typ sowie basaloide, warzige, verruköse, papilläre und gemischte Karzinome. Die Patienten können anfangs asymptomatisch sein, aber mit fortschreitender Erkrankung Juckreiz, Blutungen, Ausfluss, üblen Geruch und Schmerzen zeigen. Ein regionaler Lymphknotenbefall ist häufig, während Fernmetastasen erst im Spätstadium der Erkrankung auftreten. Zu den Risikofaktoren gehören eine HPV-Infektion, Rauchen, schlechte Hygiene und eine HIV-Infektion. Eine im Kleinkindalter durchgeführte Beschneidung führt zu einer effektiven Senkung des Tumorrisikos."},"es":{"name":"Carcinoma de células escamosas del pene","definition":"Es un tumor urogenital poco frecuente caracterizado por su origen en las células epiteliales escamosas del pene, principalmente del glande o de la superficie interna del prepucio. Macroscópicamente, los tumores pueden ser papilares o planos y ulcerados. Los subtipos histológicos incluyen el carcinoma de células escamosas habitual, considerado el tipo más frecuente, así como los carcinomas basaloides, condilomas, verrucosos, papilares y mixtos. Los pacientes pueden permanecer inicialmente asintomáticos, pudiendo presentar prurito, sangrado, secreción, mal olor y dolor a medida que la enfermedad progresa. La afectación de los ganglios linfáticos regionales es frecuente, mientras que la metástasis a distancia ocurre únicamente etapas tardías de la enfermedad. Los factores de riesgo incluyen la infección por VPH, el tabaquismo, la falta de higiene y la infección por VIH. La circuncisión neonatal es una medida preventiva altamente eficaz."},"it":{"name":"Carcinoma a cellule squamose del pene","synonyms":["Carcinoma penieno a cellule squamose"],"definition":"Si tratta di un tumore urogenitale raro, che origina dalle cellule epiteliali squamose del pene, in genere dal glande o dalla superficie interna del prepuzio. Dal punto di vista macroscopico, i tumori hanno un aspetto papillare oppure piatto e ulcerato. I sottotipi istologici comprendono il carcinoma a cellule squamose classico (il più comune), e i carcinomi basaloidi, verrucosi, papillari e misti. Inizialmente i pazienti possono essere asintomatici, ma con la progressione della malattia presentano prurito, emorragie, secrezioni, cattivo odore e dolore, man mano che la malattia progredisce. È comune l'interessamento dei linfonodi limitrofi, mentre le metastasi si sviluppano solo nelle fasi più avanzate della malattia. I fattori di rischio comprendono l'infezione da HPV, il fumo, la scarsa igiene e l'infezione da HIV. La circoncisione neonatale risulta molto protettiva."},"nl":{"name":"Plaveiselcelcarcinoom van penis","synonyms":["Peniel plaveiselcelcarcinoom"],"definition":"Een zeldzame urogenitale tumor, gekarakteriseerd door een oorsprong in squameuze epitheelcellen van de penis, doorgaans de eikel of de binnenzijde van de voorhuid. Macroscopisch kunnen de tumoren er papillair of plat en ulcererend uitzien. Histologische subtypes zijn onder meer gewoon plaveiselcelcarcinoom als meest gangbare type, alsook basaloïd, wrattig, verruceus, papillair, en gemengd carcinoom. Patiënten kunnen initieel asymptomatisch zijn, maar vertonen jeuk, bloeding, afscheiding, onaangename geur, en pijn naarmate de ziekte vordert. Betrokkenheid van regionale lymfeklieren is gangbaar, terwijl metastasen op afstand pas in latere stadia van de ziekte optreden. Risicofactoren zijn onder meer HPV-infectie, roken, ondermaatse hygiëne, en HIV-infectie. Neonatale besnijdenis biedt vermoedelijk sterke bescherming."},"pt":{"name":"Carcinoma de células escamosas do pénis"},"pl":{"name":"Rak płaskonabłonkowy penisa","synonyms":["Rak płaskonabłonkowy prącia"]},"cs":{"name":"Skvamocelulární karcinom penisu","synonyms":["Penilní skvamocelulární karcinom"]},"uk":{"name":"Плоскоклітинний рак статевого члена","synonyms":["Плоскоклітинний рак статевого члена"],"definition":"A rare urogenital tumor characterized by origin from squamous epithelial cells of the penis, most commonly the glans or inner surface of the prepuce. Macroscopically, the tumors can appear either papillary or flat and ulcerating. Histological subtypes include usual squamous cell carcinoma as the most common type, as well as basaloid, warty, verrucous, papillary, and mixed carcinomas. Patients may initially be asymptomatic but present with itching, bleeding, discharge, foul odor, and pain, as the disease progresses. Regional lymph node involvement is common, while distant metastases occur only late in the disease. Risk factors include HPV infection, smoking, poor hygiene, and HIV infection. Neonatal circumcision is implicated as strongly protective."},"zh":{"name":"阴茎鳞状细胞癌","synonyms":["阴茎鳞状细胞癌"]},"tr":{"name":"Penisin skuamöz hücreli karsinomu","synonyms":["Penil skuamöz hücreli karsinom"]}},"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":"2C81.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"186592209"},{"source":"MONDO","code":"0018352","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0238348","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10059631","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":"Value and class","class":"1-9 / 1 000 000","value":0.57,"area":"Europe","status":"Validated","source":"22119351[PMID]_RARECARE Surveillance of Rare Cancers in Europe 2003[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.612,"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":0.491,"area":"Austria","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":0.605,"area":"Belgium","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":0.516,"area":"Bulgaria","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":0.423,"area":"Croatia","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":0.684,"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":0.6,"area":"Estonia","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":0.426,"area":"Finland","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":0.638,"area":"Germany","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":0.682,"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":0.475,"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":0.606,"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":0.552,"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":0.517,"area":"Lithuania","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":0.628,"area":"Malta","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":0.744,"area":"Norway","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":0.396,"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":0.745,"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":0.499,"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":0.45,"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":0.791,"area":"Spain","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":0.597,"area":"Switzerland","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":0.691,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}