{"orpha":494448,"name":"Vulvar squamous cell carcinoma","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Squamous cell carcinoma of the vulva"],"definition":"A rare vulvar carcinoma characterized by an ulcer, nodule, macule, or pedunculated mass which is histologically composed of infiltrating islands of malignant squamous cells. Histological subtypes include keratinizing, non-keratinizing, basaloid, warty, and verrucous carcinomas. Some tumors are associated with human papilloma virus, smoking, high grade squamous intraepithelial lesion, chronic vulvar inflammatory disorders, or differentiated vulvar intraepithelial neoplasia. Patients may present with discharge, bleeding, or pain. Most important prognostic features are tumor depth and lymph node status.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-494448/","orphanet_url":"https://www.orpha.net/en/disease/detail/494448","names":{"fr":{"name":"Carcinome épidermoïde de la vulve","synonyms":["Carcinome épidermoïde vulvaire"],"definition":"Carcinome rare de la vulve caractérisé par un ulcère, un nodule, une macule ou une masse pédonculée apparaissant à l'examen histologique sous la forme d'infiltration par un amas spinocellulaire malin. Sur le plan histologie, on distingue les sous-types suivants : carcinomes kératinisants, non kératinisants, basaloïdes et verruqueux. Certaines tumeurs sont associées au papillomavirus humain, au tabagisme, à un carcinome épidermoïde sur lésion de haut grade, à des maladies inflammatoires vulvaires chroniques ou à une néoplasie intraépithéliale différenciée de la vulve. Les patients peuvent présenter des écoulements muqueux, des saignements ou des douleurs. Les principaux signes cliniques prédictifs sont l'infiltration tumorale et l'état des ganglions lymphatiques."},"de":{"name":"Plattenepithelkarzinom der Vulva","synonyms":["Squamöses Zellkarzinom der Vulva","Vulvakarzinom, squamöses"],"definition":"Ein seltenes Vulvakarzinom, das durch ein Geschwür, ein Knötchen, ein Makula oder eine gestielte Gewebeverdichtung gekennzeichnet ist, die histologisch aus infiltrierenden Inseln bösartiger Plattenepithelzellen besteht. Zu den histologischen Subtypen gehören keratinisierende, nicht-keratinisierende, basaloide, warzige und verruköse Karzinome. Einige Tumore stehen im Zusammenhang mit dem humanen Papillomavirus, Rauchen, hochgradigen Plattenepithelkarzinomen, chronischen Entzündungen der Vulva oder differenzierten vulvären intraepithelialen Neoplasien. Die Patientinnen können sich mit Ausfluss, Blutungen oder Schmerzen vorstellen. Die wichtigsten prädiktiven Merkmale sind die Tumortiefe und der Lymphknotenstatus."},"es":{"name":"Carcinoma de células escamosas de vulva","definition":"Es un carcinoma vulvar poco frecuente caracterizado por una úlcera, nódulo, mácula o masa pediculada compuesta histológicamente por islotes infiltrantes de células escamosas malignas. Los subtipos histológicos incluyen los carcinomas queratinizantes, no queratinizantes, basalioides, condilomas y verrucosos. Algunos tumores están asociados con infección por el virus del papiloma humano, tabaquismo, lesión intraepitelial escamosa de alto grado, trastornos inflamatorios vulvares crónicos o neoplasia intraepitelial vulvar diferenciada. Las pacientes pueden presentar secreción, sangrado o dolor. Las características pronósticas más importantes son la profundidad del tumor y el grado de afectación de los nódulos linfáticos."},"it":{"name":"Carcinoma vulvare a cellule squamose","definition":"Si tratta di un carcinoma raro della vulva, caratterizzato da un'ulcera, un nodulo, una macchia o una massa peduncolata composta, dal punto di cista istologico, da isole infiltranti di cellule squamose maligne. I sottotipi istologici comprendono i carcinomi cheratinizzanti, non cheratinizzanti, basaloidi e verrucosi. Alcuni tumori si associano al virus del papilloma umano, al fumo, alle lesioni intraepiteliali squamose di alto grado, alle infiammazioni croniche della vulva o alle neoplasie intraepiteliali vulvari differenziate. Le pazienti possono presentare secrezioni, emorragia o dolore. I fattori predittivi più importanti sono la profondità del tumore e l'eventuale interessamento dei linfonodi."},"nl":{"name":"Plaveiselcelcarcinoom van vulva","synonyms":["Vulvair plaveiselcelcarcinoom"],"definition":"Een zeldzaam carcinoom van vulva, gekarakteriseerd door een ulcus, nodulus, macula, of gesteelde massa die histologisch bestaat uit infiltrerende eilandjes van maligne plaveiselcellen. Histologische subtypes zijn keratiniserend, niet-keratiniserend, basaloïd, wrattig, en verruceus carcinoom. Sommige tumoren zijn geassocieerd met humaan papillomavirus, roken, hooggradige squameuze intra-epitheliale laesie, aandoeningen van chronische inflammatie van vulva, of gedifferentieerde vulvaire intra-epitheliale neoplasie. Patiënten vertonen mogelijk afscheiding, bloeding, of pijn. De belangrijkste voorspellende kenmerken zijn diepte van de tumor en status van lymfeklieren."},"pt":{"name":"Carcinoma de células escamosas vulvares","synonyms":["Carcinoma espinocelular vulvar"]},"pl":{"name":"Rak płaskonabłonkowy sromu","synonyms":["Rak płaskonabłonkowy warg sromowych i łechtaczki"]},"cs":{"name":"Dlaždicobuněčný (spinocelulární) karcinom vulvy","synonyms":["Squamous cell carcinoma of the vulva"]},"uk":{"name":"Плоскоклітинний рак вульви","synonyms":["Плоскоклітинний рак вульви"],"definition":"A rare vulvar carcinoma characterized by an ulcer, nodule, macule, or pedunculated mass which is histologically composed of infiltrating islands of malignant squamous cells. Histological subtypes include keratinizing, non-keratinizing, basaloid, warty, and verrucous carcinomas. Some tumors are associated with human papilloma virus, smoking, high grade squamous intraepithelial lesion, chronic vulvar inflammatory disorders, or differentiated vulvar intraepithelial neoplasia. Patients may present with discharge, bleeding, or pain. Most important prognostic features are tumor depth and lymph node status."},"zh":{"name":"外阴鳞状细胞癌","synonyms":["Squamous cell carcinoma of the vulva"]},"tr":{"name":"Vulvar skuamöz hücreli karsinom","synonyms":["Vulvanın skuamöz hücreli karsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C51.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C70.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"146824338"},{"source":"MONDO","code":"0024609","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0280856","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10041875","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":494418,"name":"Vulvar carcinoma","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[],"phenotypes":null}