# Vulvar cancer

Source: https://onco.cc/cancers/vulvar/  
OnCo record `vulvar` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

Early 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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-08
- Tags: gap-fill; gynaecologic; hpv
- 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

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/vulvar/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/vulvar/#overview [4 subtypes, 4 state-of-the-art points]
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/vulvar/#what-it-is [10 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/vulvar/#finding-it [5 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/vulvar/#treating-it [4 settings, 1 regimen, 4 decisions with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/vulvar/#evidence [7 trials, 6 milestones]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/vulvar/#science [9 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/vulvar/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/vulvar/#living-with-it [16 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/vulvar/coming/ [9 medicines, 7 trials, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/vulvar/data/ [53 connected records]

## Standard of care

- Early (T1, <4 cm, unifocal): Radical local excision with 1 cm margin and sentinel lymph node biopsy (GROINSS-V); radiotherapy for sentinel micrometastases ≤2 mm, lymphadenectomy for macrometastases. ([Sentinel lymph node biopsy](https://onco.cc/technologies/sentinel-node/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Node-positive after surgery: Adjuvant radiotherapy to groins and pelvis (± concurrent cisplatin) for ≥2 nodes or extracapsular spread (AGO-CaRE-1 supports chemoradiation). ([Cisplatin](https://onco.cc/drugs/cisplatin/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Locally advanced (T3 / fixed nodes): Definitive or neoadjuvant chemoradiation with weekly cisplatin (GOG 279: ~70% complete response), reserving exenterative surgery for residual disease. ([Cisplatin](https://onco.cc/drugs/cisplatin/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Metastatic or recurrent: Carboplatin-paclitaxel ± bevacizumab (by cervical analogy); pembrolizumab for PD-L1 CPS ≥1, TMB-H or MSI-H; clinical trials. ([Carboplatin](https://onco.cc/drugs/carboplatin/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Bevacizumab](https://onco.cc/drugs/bevacizumab/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/))

## State of the art

- 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Vulvar_cancer
- NCCN Guidelines: Vulvar Cancer: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1476
- GROINSS-V II (JCO 2021): https://doi.org/10.1200/JCO.21.00006
- NCI PDQ: vulvar cancer: https://www.cancer.gov/types/vulvar/patient/vulvar-treatment-pdq

## Connected records

- cancers: [Bartholin gland carcinoma](https://onco.cc/cancers/bartholin-gland-carcinoma/), [HPV-associated vulvar squamous cell carcinoma](https://onco.cc/cancers/hpv-associated-vulvar-cancer/), [HPV-independent vulvar squamous cell carcinoma (p53-mutant)](https://onco.cc/cancers/hpv-independent-vulvar-cancer/), [Mucosal melanoma](https://onco.cc/cancers/mucosal-melanoma/), [Penile cancer](https://onco.cc/cancers/penile/), [Squamous cell carcinoma of the urethra](https://onco.cc/cancers/urethral-squamous-cell-carcinoma/), [Urethral cancer](https://onco.cc/cancers/urethral/), [Vaginal cancer](https://onco.cc/cancers/vaginal/), [Vulvar melanoma](https://onco.cc/cancers/vulvar-melanoma/)
- technologies: [Colposcopes and digital cervical screening devices (DYSIS, EVA System, AVE)](https://onco.cc/technologies/colposcopes-digital-cervical-screening/), [Compression, decongestive therapy and exercise for lymphoedema](https://onco.cc/technologies/lymphoedema-decongestive-therapy/), [Gamma probes, handheld gamma cameras and dose calibrators](https://onco.cc/technologies/gamma-probes-dose-calibrators/), [HPV & HBV vaccination](https://onco.cc/technologies/hpv-vaccine/), [Immune checkpoint inhibitors](https://onco.cc/technologies/checkpoint-inhibitor/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Platinum agents](https://onco.cc/technologies/platinum/), [Sentinel lymph node biopsy](https://onco.cc/technologies/sentinel-node/)
- targets: [BCL11B](https://onco.cc/targets/bcl11b/), [FAT2](https://onco.cc/targets/fat2/), [KMT2B](https://onco.cc/targets/kmt2b/), [MAML2](https://onco.cc/targets/maml2/), [PD-1](https://onco.cc/targets/pd1/), [TP53](https://onco.cc/targets/tp53/), [ZFHX3](https://onco.cc/targets/zfhx3/)
- drugs: [Bevacizumab](https://onco.cc/drugs/bevacizumab/), [Carboplatin](https://onco.cc/drugs/carboplatin/), [Cemiplimab](https://onco.cc/drugs/cemiplimab/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [HPV quadrivalent vaccine (types 6, 11, 16 and 18)](https://onco.cc/drugs/hpv-quadrivalent-vaccine/), [Lorigerlimab](https://onco.cc/drugs/lorigerlimab/), [Nonavalent HPV vaccine](https://onco.cc/drugs/gardasil-9/), [Paclitaxel / nab-paclitaxel](https://onco.cc/drugs/paclitaxel/), [Pembrolizumab](https://onco.cc/drugs/pembrolizumab/)
- companies: [AGO Study Group](https://onco.cc/companies/ago-study-group/), [Frantz Viral Therapeutics](https://onco.cc/companies/frantz-viral-therapeutics/), [Merck & Co. (MSD)](https://onco.cc/companies/merck/), [Regeneron](https://onco.cc/companies/regeneron/), [Shanghai Bovax Biotechnology](https://onco.cc/companies/shanghai-bovax-biotechnology/)
- terms: [Combined positive score (CPS)](https://onco.cc/terms/cps/), [HPV-positive (p16) head and neck cancer](https://onco.cc/terms/hpv-p16/)
- trials: [AK104 for Recurrent or Metastatic Vulvar Cancer](https://onco.cc/trials/nct05932212/), [Electrochemotherapy for Recurrent Vulvar Cancer](https://onco.cc/trials/nct07443475/), [Electrochemotherapy With Carboplatinum Plus Bleomycin Versus Bleomycin Alone in Vulvar Cancer](https://onco.cc/trials/nct05395962/), [Evaluate the Efficacy, Immunogenicity and Safety of 9-valent HPV Recombinant Vaccine in Chinese Healthy Females](https://onco.cc/trials/nct04422366/), [Pembrolizumab Combination With Lenvatinib in Pts With Recurrent,Persistent,Metastatic or Locally Advanced Vulvar Cancer Not Amenable to Curative Surge](https://onco.cc/trials/nct05903833/), [Phase II Study of Artesunate Ointment for the Treatment of Vulvar High Grade Squamous Intraepithelial Lesions (Vulvar HSIL, VIN2/3)](https://onco.cc/trials/nct06075264/), [Study of Pembrolizumab (MK-3475) in Participants With Advanced Solid Tumors (MK-3475-158/KEYNOTE-158)](https://onco.cc/trials/nct02628067/)
- journals: [Gynecologic Oncology](https://onco.cc/journals/gynecologic-oncology/), [International journal of gynecological cancer](https://onco.cc/journals/international-journal-of-gynecological-cancer/), [Journal of gynecologic oncology](https://onco.cc/journals/journal-of-gynecologic-oncology/)

---
JSON: https://onco.cc/api/v1/entities/vulvar.json