{"orpha":494418,"name":"Vulvar carcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Carcinoma of vulva"],"definition":"A group of rare tumors of the vulva comprising HPV-associated and HPV-independent squamous cell carcinomas as the most frequent malignant vulvar tumors, basal cell carcinomas, adenocarcinomas, and Bartholin gland carcinomas. Depending on the type of tumor and disease stage, patients may present with a painless vulvar mass or ulcer, or with pruritus, a burning sensation, pain, or bleeding.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-494418/","orphanet_url":"https://www.orpha.net/en/disease/detail/494418","names":{"fr":{"name":"Carcinome de la vulve","synonyms":["Carcinome vulvaire"],"definition":"Groupe de tumeurs rares de la vulve comprenant des carcinomes épidermoïdes induits ou pas par l'l'HPV, c'est-à-dire, les tumeurs malignes les plus fréquentes de la vulve, des carcinomes basocellulaires, des adénocarcinomes et des carcinomes de la glande de Bartholin. Selon le type de tumeur et le stade de la maladie, les patientes peuvent présenter une masse vulvaire indolore ou un ulcère, un prurit, une sensation de brûlure, une douleur ou des saignements."},"de":{"name":"Vulvakarzinom","synonyms":["Karzinom der Vulva"],"definition":"Eine Gruppe seltener Tumoren der Vulva, die HPV-assoziierte und HPV-unabhängige Plattenepithelkarzinome als häufigste maligne Vulvakarzinome, Basalzellkarzinome, Adenokarzinome und Bartholin-Drüsenkarzinome umfasst. Je nach Tumorart und Krankheitsstadium können sich die Patientinnen mit einer schmerzlosen Gewebeverdichtung oder einem Geschwür an der Vulva oder mit Juckreiz, einem brennenden Gefühl, Schmerzen oder Blutungen vorstellen."},"es":{"name":"Carcinoma vulvar","synonyms":["Carcinoma de la vulva"],"definition":"Es un grupo poco frecuente de tumores de la vulva que comprende carcinomas de células escamosas asociados e independientes del VPH, como los tumores vulvares malignos más frecuentes, carcinomas de células basales, adenocarcinomas y carcinomas de la glándula de Bartolino. Según el tipo de tumor y el estadío de la enfermedad, las pacientes pueden presentar una masa o úlcera vulvar indolora, prurito, sensación de ardor, dolor o sangrado."},"it":{"name":"Carcinoma vulvare","definition":"Comprende un gruppo di tumori rari della vulva, che include i carcinomi a cellule squamose associati e indipendenti dall'HPV (i tumori vulvari maligni più frequenti), i carcinomi basocellulari, gli adenocarcinomi e i carcinomi delle ghiandole di Bartolini. A seconda del tipo di tumore e dello stadio della malattia, le pazienti possono presentare una massa o un'ulcera vulvare indolore, oppure prurito, sensazione di bruciore, dolore o emorragie."},"nl":{"name":"Vulvacarcinoom","synonyms":["Carcinoom van vulva"],"definition":"Een groep van zeldzame tumoren van vulva bestaande uit HPV-geassocieerde en HPV-onafhankelijke plaveiselcelcarcinomen als de meest frequente maligne tumoren van vulva, basaalcelcarcinomen, adenocarcinomen, en carcinomen van klier van Bartholin. Afhankelijk va het type van de tumor en het stadium van de ziekte, kunnen patiënten zich presenteren met een pijnloze massa of ulcus van vulva, of met pruritus, een brandend gevoel, pijn, of bloeding."},"pt":{"name":"Carcinoma vulvar"},"pl":{"name":"Rak sromu","synonyms":["Rak warg sromowych i łechtaczki"]},"cs":{"name":"Karcinom vulvy","synonyms":["Carcinoma of vulva"]},"uk":{"name":"Карцинома вульви","synonyms":["Карцинома вульви"],"definition":"A group of rare tumors of the vulva comprising HPV-associated and HPV-independent squamous cell carcinomas as the most frequent malignant vulvar tumors, basal cell carcinomas, adenocarcinomas, and Bartholin gland carcinomas. Depending on the type of tumor and disease stage, patients may present with a painless vulvar mass or ulcer, or with pruritus, a burning sensation, pain, or bleeding."},"zh":{"name":"外阴癌","synonyms":["Carcinoma of vulva"]},"tr":{"name":"Vulvar karsinom","synonyms":["Vulva karsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C51.8","qualifier":"E","icd_relation":"specific","validated":false},{"source":"ICD-11","code":"2C70","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1530443809"},{"source":"MONDO","code":"0005215","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0677055","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9349","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":180312,"name":"Rare vulvovaginal tumor","classifications":[202,205]}],"children":[{"orpha":494454,"name":"Vulvar adenocarcinoma","type":"Histopathological subtype"},{"orpha":494451,"name":"Vulvar basal cell carcinoma","type":"Histopathological subtype"},{"orpha":494448,"name":"Vulvar squamous cell carcinoma","type":"Histopathological subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.86,"area":"Worldwide","status":"Validated","source":"37531169[PMID]_ORPHANET"}],"phenotypes":null}