OnCo
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Vulvar cancer

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.

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.

State of the art today

  • 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.
Who it affects

About 45,000 cases per year worldwide (GLOBOCAN); two peaks: younger women with HPV-related disease and older women with lichen sclerosus-associated disease.

Where the cases are

Cases by country · GLOBOCAN 2022
All countries →

No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.

Standard of care

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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.

NCCN · Category 2A
Sentinel lymph node biopsyIMRT / IGRT (modern external beam)
Node-positive after surgery

Adjuvant radiotherapy to groins and pelvis (± concurrent cisplatin) for ≥2 nodes or extracapsular spread (AGO-CaRE-1 supports chemoradiation).

NCCN · Category 2A
CisplatinIMRT / IGRT (modern external beam)
Locally advanced (T3 / fixed nodes)

Definitive or neoadjuvant chemoradiation with weekly cisplatin (GOG 279: ~70% complete response), reserving exenterative surgery for residual disease.

NCCN · Category 2A
CisplatinIMRT / IGRT (modern external beam)
Metastatic or recurrent

Carboplatin-paclitaxel ± bevacizumab (by cervical analogy); pembrolizumab for PD-L1 CPS ≥1, TMB-H or MSI-H; clinical trials.

NCCN · Category 2A
CarboplatinPaclitaxel / nab-paclitaxelBevacizumabPembrolizumab

Subtypes & biomarkers

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Subtypes
Biomarkers clinicians test

Target prevalence in this cancer

History

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  1. 1912Basset describes radical vulvectomy with en bloc lymphadenectomy

    Cure at the cost of severe morbidity; standard for 70 years.

  2. 1990Separate groin incisions replace en bloc dissection
  3. 2008GROINSS-V I: sentinel node biopsy safe in early vulvar cancer
    Sentinel lymph node biopsy
  4. 2016HPV-independent vs HPV-associated pathways defined (WHO 2020 adopts)
  5. 2021GROINSS-V II: radiotherapy for sentinel micrometastases
    IMRT / IGRT (modern external beam)
  6. 2021GOG 279: cisplatin-gemcitabine chemoradiation for locally advanced disease
    Cisplatin

Pipeline

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ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)
ApprovedMonoclonal antibody (anti-PD-1)
Cemiplimab · Libtayo

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.

Trials

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Recruiting now (live from ClinicalTrials.gov)

Recruiting trials near you · live from ClinicalTrials.gov
Vulvar cancer
condition: Vulvar cancer
Open on ClinicalTrials.gov →

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Expert centres

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Where the expertise is

Centres linked to this cancer in OnCo

Seeking a second opinion: ask your oncologist for a referral to a high-volume centre; most accept records and pathology by mail or telehealth. In the US, use the NCI's Find a Cancer Center tool or the nonprofit Cancer Commons, which navigates options for advanced cancers at no cost.

Questions to ask

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Bring to your appointment

Questions to ask your oncologist about Vulvar cancer

Generated from this cancer's standard of care, biomarkers, and pipeline · 16 questions

Newly diagnosed

  1. What is my exact diagnosis, stage, and grade, and which tests established them?
    Why: Everything else follows from an accurate stage and subtype.
  2. Which biomarkers have been tested on my tumour (for example p16and p53 IHC, Sentinel node status and metastasis size, Depth of invasion, PD-L1 CPS / TMB / MSI, Margin status), and what were the results?
    Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
  3. Which subtype is my cancer, and does that change the recommended treatment?
    Why: Recognised subtypes for this cancer include HPV-associated SCC, HPV-independent SCC, Vulvar melanoma.
  4. Is germline (inherited) genetic testing recommended for me or my family?
    Why: Inherited variants can change treatment and matter for relatives.

Early (T1, <4 cm, unifocal)

  1. For my situation (early (t1, <4 cm, unifocal)), which of the standard options do you recommend and why?
    Why: Guideline options include: Radical local excision with 1 cm margin and sentinel lymph node biopsy (GROINSS-V); radiotherapy for sentinel micrometastases ≤2 mm, lymphadenectomy for macrometastases.

Node-positive after surgery

  1. For my situation (node-positive after surgery), which of the standard options do you recommend and why?
    Why: Guideline options include: Adjuvant radiotherapy to groins and pelvis (± concurrent cisplatin) for ≥2 nodes or extracapsular spread (AGO-CaRE-1 supports chemoradiation).
  2. Am I a candidate for Cisplatin, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Locally advanced (T3 / fixed nodes)

  1. For my situation (locally advanced (t3 / fixed nodes)), which of the standard options do you recommend and why?
    Why: Guideline options include: Definitive or neoadjuvant chemoradiation with weekly cisplatin (GOG 279: ~70% complete response), reserving exenterative surgery for residual disease.
  2. Am I a candidate for Cisplatin, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Metastatic or recurrent

  1. For my situation (metastatic or recurrent), which of the standard options do you recommend and why?
    Why: Guideline options include: Carboplatin-paclitaxel ± bevacizumab (by cervical analogy); pembrolizumab for PD-L1 CPS ≥1, TMB-H or MSI-H; clinical trials.
  2. Am I a candidate for Carboplatin, Paclitaxel / nab-paclitaxel, Bevacizumab or related drugs, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Any stage

  1. Are there clinical trials I could join, for example of Pembrolizumab, Cemiplimab?
    Why: Trials are how the next standard of care is set; asking early keeps options open.
  2. Would a second opinion at a high-volume centre change anything, and can you help arrange it?
    Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
  3. What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
    Why: Supportive care improves quality of life and helps patients complete treatment.
  4. I read that “HPV-independent p53-mutant disease recurs often and has no targeted therapy”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.
  5. I read that “Lichen sclerosus surveillance and prevention of malignant transformation”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.

Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.

Everything relevant

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Direct links plus the targets, companies, and technologies of this cancer's products.

technologies

8
Anti-angiogenic therapyCytotoxic chemotherapyHPV & HBV vaccinationImmune checkpoint inhibitorsIMRT / IGRT (modern external beam)Monoclonal antibodiesPlatinum agentsSentinel lymph node biopsy

targets

3
PD-1TP53VEGF / VEGFR

drugs

7
ApprovedMonoclonal antibody (anti-VEGF)
Bevacizumab · Avastin (and biosimilars)
ApprovedCytotoxic chemotherapy (platinum)
Carboplatin
ApprovedMonoclonal antibody (anti-PD-1)
Cemiplimab · Libtayo
ApprovedCytotoxic chemotherapy (platinum)
Cisplatin · Platinol (generic)
ApprovedProphylactic vaccine (virus-like particles)
Nonavalent HPV vaccine · Gardasil 9
ApprovedCytotoxic chemotherapy (taxane)
Paclitaxel / nab-paclitaxel · Taxol / Abraxane
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)

companies

3
Merck & Co. (MSD)RegeneronRoche / Genentech

terms

2
Combined positive score (CPS)HPV-positive (p16) head and neck cancer

Latest papers

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Latest papers · live from Europe PMC
Open in Europe PMC

Query for this cancer: (TITLE:"Vulvar cancer" OR ABSTRACT:"Vulvar cancer") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Vulvar cancer, not a curated reading list.

Connected

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Pages like this

not linked directly; found by shared links

technologies

5
HPV & HBV vaccinationImmune checkpoint inhibitorsIMRT / IGRT (modern external beam)Platinum agentsSentinel lymph node biopsy

targets

2
PD-1TP53

drugs

7
ApprovedMonoclonal antibody (anti-VEGF)
Bevacizumab · Avastin (and biosimilars)
ApprovedCytotoxic chemotherapy (platinum)
Carboplatin
ApprovedMonoclonal antibody (anti-PD-1)
Cemiplimab · Libtayo
ApprovedCytotoxic chemotherapy (platinum)
Cisplatin · Platinol (generic)
ApprovedProphylactic vaccine (virus-like particles)
Nonavalent HPV vaccine · Gardasil 9
ApprovedCytotoxic chemotherapy (taxane)
Paclitaxel / nab-paclitaxel · Taxol / Abraxane
ApprovedMonoclonal antibody (anti-PD-1)
Pembrolizumab · Keytruda / Keytruda Qlex (SC)

companies

2
Merck & Co. (MSD)Regeneron

terms

2
Combined positive score (CPS)HPV-positive (p16) head and neck cancer