{"entity":{"id":"vulvar","kind":"cancer","name":"Vulvar cancer","aka":[],"tldr":"An uncommon cancer of the external genitalia with two distinct causes: HPV infection in younger women and chronic skin inflammation in older women. Surgery is the mainstay, and sentinel-node biopsy has made it far less mutilating.","summary":"Vulvar squamous cell carcinoma has two pathways: HPV-associated (usual-type VIN, p16-positive, younger patients, better prognosis) and HPV-independent (differentiated VIN arising in lichen sclerosus, p53-mutant, older patients, higher recurrence). Rarer histologies include melanoma, Bartholin gland adenocarcinoma, Paget disease and basal cell carcinoma. Nodal status is the dominant prognostic factor.\n\nEarly disease is treated with radical local excision and sentinel lymph node biopsy (GROINSS-V I established safety for tumours <4 cm with unifocal disease, replacing inguinofemoral lymphadenectomy and its lymphoedema in most); GROINSS-V II showed radiotherapy can replace lymphadenectomy for micrometastases ≤2 mm. Locally advanced disease receives chemoradiation (cisplatin-based, GOG 205/279) to avoid exenteration. Metastatic or recurrent disease has limited options: platinum-based chemotherapy, pembrolizumab for PD-L1-positive or TMB-high disease (KEYNOTE-158), cemiplimab in trials, and, for HPV-independent p53-mutant disease, no targeted therapy.","asOf":"2026-09-08","wikipedia":"https://en.wikipedia.org/wiki/Vulvar_cancer","links":[{"label":"NCCN Guidelines: Vulvar Cancer","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1476"},{"label":"GROINSS-V II (JCO 2021)","url":"https://doi.org/10.1200/JCO.21.00006"},{"label":"NCI PDQ: vulvar cancer","url":"https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq"}],"tags":["gap-fill","gynaecologic","hpv"],"related":["vaginal"],"cancers":[],"sections":[],"technologies":["sentinel-node","imrt-igrt","checkpoint-inhibitor","hpv-vaccine","platinum"],"targets":["pd1","tp53"],"drugs":["cisplatin","carboplatin","paclitaxel","bevacizumab","pembrolizumab","cemiplimab","gardasil-9"],"companies":["merck","regeneron"],"institutions":[],"pathways":[],"terms":["hpv-p16","cps"],"trials":["nct04422366"],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"gynaecologic","burden":"About 45,000 cases per year worldwide (GLOBOCAN); two peaks: younger women with HPV-related disease and older women with lichen sclerosus-associated disease.","subtypes":["HPV-associated SCC (usual VIN precursor, p16+)","HPV-independent SCC (differentiated VIN, lichen sclerosus, p53-mutant)","Vulvar melanoma","Extramammary Paget disease","Bartholin gland carcinoma","Basal cell carcinoma of the vulva"],"biomarkers":["p16 (HPV) and p53 IHC (molecular subtype)","Sentinel node status and metastasis size (≤2 mm vs >2 mm)","Depth of invasion (>1 mm for nodal assessment)","PD-L1 CPS / TMB / MSI (pembrolizumab)","Margin status"],"standardOfCare":[{"setting":"Early (T1, <4 cm, unifocal)","approach":"Radical local excision with 1 cm margin and sentinel lymph node biopsy (GROINSS-V); radiotherapy for sentinel micrometastases ≤2 mm, lymphadenectomy for macrometastases.","refs":["sentinel-node","imrt-igrt"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Vulvar Cancer","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1476"}},{"setting":"Node-positive after surgery","approach":"Adjuvant radiotherapy to groins and pelvis (± concurrent cisplatin) for ≥2 nodes or extracapsular spread (AGO-CaRE-1 supports chemoradiation).","refs":["cisplatin","imrt-igrt"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Vulvar Cancer"}},{"setting":"Locally advanced (T3 / fixed nodes)","approach":"Definitive or neoadjuvant chemoradiation with weekly cisplatin (GOG 279: ~70% complete response), reserving exenterative surgery for residual disease.","refs":["cisplatin","imrt-igrt"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Vulvar Cancer"}},{"setting":"Metastatic or recurrent","approach":"Carboplatin-paclitaxel ± bevacizumab (by cervical analogy); pembrolizumab for PD-L1 CPS ≥1, TMB-H or MSI-H; clinical trials.","refs":["carboplatin","paclitaxel","bevacizumab","pembrolizumab"],"guideline":{"nccn":"Category 2A","version":"NCCN Guidelines: Vulvar Cancer"}}],"stateOfArt":["Sentinel-node biopsy and radiotherapy for micrometastases (GROINSS-V I/II) have removed lymphadenectomy morbidity for most early patients.","Molecular classification (HPV/p16 vs p53) is entering staging and predicts recurrence better than stage alone.","Chemoradiation avoids exenteration in most locally advanced disease.","Systemic therapy remains borrowed from cervical cancer; dedicated trials are few."],"history":[{"year":1912,"title":"Basset describes radical vulvectomy with en bloc lymphadenectomy","note":"Cure at the cost of severe morbidity; standard for 70 years.","refs":[]},{"year":1990,"title":"Separate groin incisions replace en bloc dissection","refs":[]},{"year":2008,"title":"GROINSS-V I: sentinel node biopsy safe in early vulvar cancer","refs":["sentinel-node"]},{"year":2016,"title":"HPV-independent vs HPV-associated pathways defined (WHO 2020 adopts)","refs":[]},{"year":2021,"title":"GROINSS-V II: radiotherapy for sentinel micrometastases","refs":["imrt-igrt"]},{"year":2021,"title":"GOG 279: cisplatin-gemcitabine chemoradiation for locally advanced disease","refs":["cisplatin"]}],"pipeline":["pembrolizumab","cemiplimab","lorigerlimab"],"openProblems":["HPV-independent p53-mutant disease recurs often and has no targeted therapy.","Lichen sclerosus surveillance and prevention of malignant transformation.","Few dedicated trials; therapy extrapolated from cervical cancer.","Psychosexual morbidity after treatment."]},"route":"/cancers/vulvar/","neighbours":{"cancer":[{"id":"bartholin-gland-carcinoma","kind":"cancer","name":"Bartholin gland carcinoma","route":"/cancers/bartholin-gland-carcinoma/"},{"id":"hpv-associated-vulvar-cancer","kind":"cancer","name":"HPV-associated vulvar squamous cell carcinoma","route":"/cancers/hpv-associated-vulvar-cancer/"},{"id":"hpv-independent-vulvar-cancer","kind":"cancer","name":"HPV-independent vulvar squamous cell carcinoma (p53-mutant)","route":"/cancers/hpv-independent-vulvar-cancer/"},{"id":"mucosal-melanoma","kind":"cancer","name":"Mucosal melanoma","route":"/cancers/mucosal-melanoma/"},{"id":"penile","kind":"cancer","name":"Penile cancer","route":"/cancers/penile/"},{"id":"urethral-squamous-cell-carcinoma","kind":"cancer","name":"Squamous cell carcinoma of the urethra","route":"/cancers/urethral-squamous-cell-carcinoma/"},{"id":"urethral","kind":"cancer","name":"Urethral cancer","route":"/cancers/urethral/"},{"id":"vaginal","kind":"cancer","name":"Vaginal cancer","route":"/cancers/vaginal/"},{"id":"vulvar-melanoma","kind":"cancer","name":"Vulvar melanoma","route":"/cancers/vulvar-melanoma/"}],"technology":[{"id":"colposcopes-digital-cervical-screening","kind":"technology","name":"Colposcopes and digital cervical screening devices (DYSIS, EVA System, AVE)","route":"/technologies/colposcopes-digital-cervical-screening/"},{"id":"lymphoedema-decongestive-therapy","kind":"technology","name":"Compression, decongestive therapy and exercise for lymphoedema","route":"/technologies/lymphoedema-decongestive-therapy/"},{"id":"gamma-probes-dose-calibrators","kind":"technology","name":"Gamma probes, handheld gamma cameras and dose calibrators","route":"/technologies/gamma-probes-dose-calibrators/"},{"id":"hpv-vaccine","kind":"technology","name":"HPV & HBV vaccination","route":"/technologies/hpv-vaccine/"},{"id":"checkpoint-inhibitor","kind":"technology","name":"Immune checkpoint inhibitors","route":"/technologies/checkpoint-inhibitor/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"},{"id":"platinum","kind":"technology","name":"Platinum agents","route":"/technologies/platinum/"},{"id":"sentinel-node","kind":"technology","name":"Sentinel lymph node biopsy","route":"/technologies/sentinel-node/"}],"target":[{"id":"bcl11b","kind":"target","name":"BCL11B","route":"/targets/bcl11b/"},{"id":"fat2","kind":"target","name":"FAT2","route":"/targets/fat2/"},{"id":"kmt2b","kind":"target","name":"KMT2B","route":"/targets/kmt2b/"},{"id":"maml2","kind":"target","name":"MAML2","route":"/targets/maml2/"},{"id":"pd1","kind":"target","name":"PD-1","route":"/targets/pd1/"},{"id":"tp53","kind":"target","name":"TP53","route":"/targets/tp53/"},{"id":"zfhx3","kind":"target","name":"ZFHX3","route":"/targets/zfhx3/"}],"drug":[{"id":"bevacizumab","kind":"drug","name":"Bevacizumab","route":"/drugs/bevacizumab/"},{"id":"carboplatin","kind":"drug","name":"Carboplatin","route":"/drugs/carboplatin/"},{"id":"cemiplimab","kind":"drug","name":"Cemiplimab","route":"/drugs/cemiplimab/"},{"id":"cisplatin","kind":"drug","name":"Cisplatin","route":"/drugs/cisplatin/"},{"id":"hpv-quadrivalent-vaccine","kind":"drug","name":"HPV quadrivalent vaccine (types 6, 11, 16 and 18)","route":"/drugs/hpv-quadrivalent-vaccine/"},{"id":"lorigerlimab","kind":"drug","name":"Lorigerlimab","route":"/drugs/lorigerlimab/"},{"id":"gardasil-9","kind":"drug","name":"Nonavalent HPV vaccine","route":"/drugs/gardasil-9/"},{"id":"paclitaxel","kind":"drug","name":"Paclitaxel / nab-paclitaxel","route":"/drugs/paclitaxel/"},{"id":"pembrolizumab","kind":"drug","name":"Pembrolizumab","route":"/drugs/pembrolizumab/"}],"company":[{"id":"ago-study-group","kind":"company","name":"AGO Study Group","route":"/companies/ago-study-group/"},{"id":"frantz-viral-therapeutics","kind":"company","name":"Frantz Viral Therapeutics","route":"/companies/frantz-viral-therapeutics/"},{"id":"merck","kind":"company","name":"Merck & Co. (MSD)","route":"/companies/merck/"},{"id":"regeneron","kind":"company","name":"Regeneron","route":"/companies/regeneron/"},{"id":"shanghai-bovax-biotechnology","kind":"company","name":"Shanghai Bovax Biotechnology","route":"/companies/shanghai-bovax-biotechnology/"}],"term":[{"id":"cps","kind":"term","name":"Combined positive score (CPS)","route":"/terms/cps/"},{"id":"hpv-p16","kind":"term","name":"HPV-positive (p16) head and neck cancer","route":"/terms/hpv-p16/"}],"trial":[{"id":"nct05932212","kind":"trial","name":"AK104 for Recurrent or Metastatic Vulvar Cancer","route":"/trials/nct05932212/"},{"id":"nct07443475","kind":"trial","name":"Electrochemotherapy for Recurrent Vulvar Cancer","route":"/trials/nct07443475/"},{"id":"nct05395962","kind":"trial","name":"Electrochemotherapy With Carboplatinum Plus Bleomycin Versus Bleomycin Alone in Vulvar Cancer","route":"/trials/nct05395962/"},{"id":"nct04422366","kind":"trial","name":"Evaluate the Efficacy, Immunogenicity and Safety of 9-valent HPV Recombinant Vaccine in Chinese Healthy Females","route":"/trials/nct04422366/"},{"id":"keynote-158","kind":"trial","name":"KEYNOTE-158","route":"/trials/keynote-158/"},{"id":"nct05903833","kind":"trial","name":"Pembrolizumab Combination With Lenvatinib in Pts With Recurrent,Persistent,Metastatic or Locally Advanced Vulvar Cancer Not Amenable to Curative Surge","route":"/trials/nct05903833/"},{"id":"nct06075264","kind":"trial","name":"Phase II Study of Artesunate Ointment for the Treatment of Vulvar High Grade Squamous Intraepithelial Lesions (Vulvar HSIL, VIN2/3)","route":"/trials/nct06075264/"}],"journal":[{"id":"gynecologic-oncology","kind":"journal","name":"Gynecologic Oncology","route":"/journals/gynecologic-oncology/"},{"id":"international-journal-of-gynecological-cancer","kind":"journal","name":"International journal of gynecological cancer","route":"/journals/international-journal-of-gynecological-cancer/"},{"id":"journal-of-gynecologic-oncology","kind":"journal","name":"Journal of gynecologic oncology","route":"/journals/journal-of-gynecologic-oncology/"}]}}