{"orpha":494451,"name":"Vulvar basal cell carcinoma","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Basal cell carcinoma of vulva"],"definition":"A rare vulvar carcinoma characterized by a slowly growing ulcer or nodule which is histologically composed of demarcated nests of palisaded basal cells originating at the epidermal-dermal junction. Occasionally, the tumor may be extensively pigmented. Patients most commonly present with pruritus. The lesion is usually treated by local excision, although groin metastases have been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-494451/","orphanet_url":"https://www.orpha.net/en/disease/detail/494451","names":{"fr":{"name":"Carcinome basocellulaire de la vulve","synonyms":["Carcinome basocellulaire vulvaire"],"definition":"Carcinome de la vulve rare caractérisé par une ulcération ou un nodule à croissance lente qui, à l'examen histologique, apparaît sous la forme de foyers délimités de cellules basales formant une palissade en bordure de la jonction épidermique-dermique. La tumeur peut dans certains cas être fortement pigmentée. Les patients présentent le plus souvent un prurit. La lésion est généralement traitée par excision locale, bien que des métastases à l'aine aient été rapportées."},"de":{"name":"Basalzellkarzinom der Vulva","definition":"Ein seltenes Vulvakarzinom, das durch ein langsam wachsendes Geschwür oder Knötchen gekennzeichnet ist, das histologisch aus abgegrenzten Nestern von Basalzellen besteht, die von der epidermalen-dermalen Grenzfläche ausgehen. Gelegentlich kann der Tumor stark pigmentiert sein. In den meisten Fällen leiden die Patienten unter Juckreiz. Die Läsion wird in der Regel durch lokale Exzision behandelt, obwohl auch über inguinale Metastasen berichtet wurde."},"es":{"name":"Carcinoma de células basales de la vulva","definition":"Es un carcinoma poco frecuente de la vulva caracterizado por una úlcera o nódulo de crecimiento lento compuesto histológicamente por nidos bien delimitados de células basales en empalizada que se originan en la unión dermo-epidérmica. En ocasiones, el tumor puede estar excesivamente pigmentado. Los pacientes se presentan con mayor frecuencia con prurito. La lesión suele tratarse mediante escisión local, aunque se ha descrito la metástasis inguinal."},"it":{"name":"Carcinoma della vulva a cellule basali","definition":"Si tratta di un carcinoma raro della vulva, caratterizzato da un'ulcera o un nodulo a crescita lenta, composto, dal punto di vista istologico, da nidi delimitati di cellule basali disposte a palizzata, che originano dalla giunzione dermo-epidermica. Occasionalmente, il tumore può essere diffusamente pigmentato. Di solito le pazienti presentano prurito. In genere la lesione viene trattata con una escissione locale, sebbene possano essere presenti metastasi all'inguine."},"nl":{"name":"Basaalcelcarcinoom van vulva","definition":"Een zeldzaam carcinoom van vulva, gekarakteriseerd door een traag groeiende ulcus of nodulus die histologisch bestaat uit begrensde nesten van gepalissadeerde basale cellen afkomstig van de dermo-epidermale overgang. Occasioneel is de tumor uitvoerig gepigmenteerd. Patiënten presenteren zich doorgaans met pruritus. De laesie wordt meestal behandeld door lokale excisie, maar metastase in liesstreek werd ook reeds gerapporteerd."},"pt":{"name":"Carcinoma basocelular vulvar"},"pl":{"name":"Rak podstawnokomórkowy sromu","synonyms":["Rak podstawnokomórkowy warg sromowych i łechtaczki"]},"cs":{"name":"Bazocelulární karcinom vulvy","synonyms":["Basal cell carcinoma of vulva"]},"uk":{"name":"Базальноклітинна карцинома вульви","synonyms":["Базальноклітинна карцинома вульви"],"definition":"A rare vulvar carcinoma characterized by a slowly growing ulcer or nodule which is histologically composed of demarcated nests of palisaded basal cells originating at the epidermal-dermal junction. Occasionally, the tumor may be extensively pigmented. Patients most commonly present with pruritus. The lesion is usually treated by local excision, although groin metastases have been reported."},"zh":{"name":"外阴基底细胞癌","synonyms":["Basal cell carcinoma of vulva"]},"tr":{"name":"Vulvar bazal hücreli karsinom","synonyms":["Vulvanın bazal hücreli karsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C51.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C70.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"247568702"},{"source":"MONDO","code":"0002955","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1336977","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10082639","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}