Vulval Cancer
Summary
Vulval Cancer is a relatively rare gynaecological malignancy, accounting for ~4% of gynaecological cancers. The vast majority (~90%) are Squamous Cell Carcinomas (SCC). There are two main pathways to vulval SCC: HPV-related (younger women, associated with Vulval Intraepithelial Neoplasia - VIN) and HPV-independent (older women, associated with chronic vulval dermatoses, especially Lichen Sclerosus). Presentation is typically a persistent vulval lump, ulcer, or pruritus in an elderly woman. Diagnosis is by biopsy. Treatment is primarily surgical (wide local excision or radical vulvectomy with inguinal lymph node assessment), with significant associated morbidity (lymphoedema, wound breakdown, psychosexual impact). [1,2]
Clinical Pearls
Two Pathways to SCC: 1) HPV-related (younger, VIN history). 2) HPV-independent (older, Lichen Sclerosus).
Lichen Sclerosus Risk: Up to 5% of women with Lichen Sclerosus will develop vulval SCC. Lifelong surveillance is needed for these patients.
"Any Persistent Vulval Lesion in an Older Woman": Have a low threshold to biopsy any non-healing ulcer, lump, or area resistant to treatment.
Groin Node Status = Key Prognostic Factor: Inguinal lymph node involvement dramatically worsens prognosis.
Demographics
- Incidence: ~1,400 cases/year in the UK. ~6,000 in the USA.
- Age: Peak >65 years (HPV-independent). Younger peak 35-45 years (HPV-related).
- Increasing: Incidence rising, partly due to increased HPV-related disease in younger women.
Risk Factors
| Factor | Notes |
|---|---|
| Lichen Sclerosus | Major risk for HPV-independent SCC. Causes chronic inflammation. ~5% lifetime risk of SCC. |
| HPV Infection (16, 18) | Causes Vulval Intraepithelial Neoplasia (VIN), which can progress to SCC. |
| Vulval Intraepithelial Neoplasia (VIN) | Precursor lesion. High-Grade VIN (VIN 2/3 / HSIL) carries risk of progression. |
| Smoking | Increases VIN and SCC risk. |
| Immunosuppression | HIV, transplant patients. Increased HPV-related disease. |
| Age >65 | Peak incidence. |
| Other Vulval Dermatoses | Lichen Planus (less common than Lichen Sclerosus). |
Histological Types
| Type | Percentage | Notes |
|---|---|---|
| Squamous Cell Carcinoma (SCC) | ~90% | Most common. Two subtypes: HPV-related (Warty/Basaloid) and HPV-independent (Keratinizing). |
| Melanoma | ~5% | Second most common. Poor prognosis. |
| Bartholin Gland Carcinoma | less than 5% | Rare. Adenocarcinoma or SCC. |
| Paget's Disease of Vulva | less than 5% | Intraepithelial adenocarcinoma in situ. Red, eczematoid appearance. May indicate underlying adenocarcinoma elsewhere. |
| Other (Sarcoma, BCC) | Rare |
Two Pathways to SCC
- HPV-Related (Warty/Basaloid SCC):
- Younger patients.
- Associated with High-Grade VIN (Usual Type / HSIL).
- Often Multifocal.
- Better prognosis.
- HPV-Independent (Keratinizing SCC):
- Older patients (>65).
- Associated with chronic Lichen Sclerosus or Lichen Planus.
- Often Unifocal.
- Worse prognosis.
| Condition | Key Features |
|---|---|
| Vulval Cancer (SCC) | Persistent lump/ulcer. Elderly. Non-healing. Inguinal nodes. Biopsy confirms. |
| Lichen Sclerosus | White, atrophic skin ("parchment"). Loss of architecture. Itchy. Chronic. Can co-exist with cancer. |
| Vulval Intraepithelial Neoplasia (VIN) | Precursor. White/Red plaques. May be multifocal. Biopsy shows dysplasia. |
| Genital Warts (Condylomata) | HPV. Warty papules. Usually younger. |
| Vulval Melanoma | Pigmented lesion. Asymmetry. May be nodular. Biopsy. |
| Paget's Disease | Eczematoid red plaque. Pruritus. Biopsy shows Paget cells. Screen for underlying adenocarcinoma. |
| Bartholin's Cyst/Abscess | Swelling at 5 or 7 o'clock. If solid or >40yrs -> exclude cancer. |
| Contact Dermatitis / Eczema | Itchy. Responds to steroids. Bilateral. |
| Herpes Simplex | Painful vesicles/ulcers. Recurrent. |
Symptoms
Signs
Biopsy (Essential)
- Punch or Excision Biopsy: Of any suspicious lesion. Confirms diagnosis and histological type.
Staging Investigations (Once Diagnosis Confirmed)
| Investigation | Purpose |
|---|---|
| MRI Pelvis | Local staging. Tumour size. Depth of invasion. Lymph node assessment. |
| CT Chest/Abdomen/Pelvis | Distant metastases (Stage IV). |
| Examination Under Anaesthesia (EUA) | Assess extent, especially if involving urethra/anus. |
Sentinel Lymph Node Biopsy (SLNB)
- Increasingly used for unifocal tumours less than 4cm with clinically negative groin nodes.
- Reduces morbidity of full lymphadenectomy.
Management Algorithm
SUSPECTED VULVAL CANCER
(Persistent Lump / Ulcer / Non-Healing Lesion)
↓
PUNCH BIOPSY
↓
HISTOLOGY CONFIRMS SCC
↓
STAGING (MRI Pelvis, CT C/A/P)
↓
MDT DISCUSSION
(Gynaecological Oncology)
↓
CLINICAL STAGE?
┌───────────────┴───────────────┐
EARLY STAGE ADVANCED
(IA, IB, II) (III, IV)
↓ ↓
SURGERY CHEMORADIOTHERAPY
- Wide Local Excision (WLE) (Primary or Neo-adjuvant)
(1cm margin minimum) +/- Surgery
- Radical Vulvectomy (larger)
↓
GROIN NODE ASSESSMENT
- Sentinel Lymph Node Biopsy (SLNB)
(for tumours less than 4cm, uninodal)
- Inguino-femoral Lymphadenectomy
(if SLNB positive or high-risk)
↓
POST-OPERATIVE
- Adjuvant Radiotherapy (if margins close, node +ve)
↓
LONG-TERM SURVEILLANCE
(Regular vulval/groin examination)
Surgical Treatment
| Procedure | Indication |
|---|---|
| Wide Local Excision (WLE) | Small tumours. Aim for 1cm clear margin. |
| Radical Vulvectomy | Larger tumours. En-bloc or separate incisions. High morbidity. |
| Inguino-Femoral Lymphadenectomy | Assess nodal spread. Unilateral if tumour is lateral. Bilateral if midline. |
| Sentinel Lymph Node Biopsy (SLNB) | Alternative for select cases. Reduces lymphoedema risk. |
| Pelvic Exenteration | Rarely. For advanced/recurrent disease involving bladder/rectum. |
Non-Surgical Treatment
- Radiotherapy: Adjuvant (post-operative if close margins or node +ve). Primary (if unfit for surgery or advanced disease).
- Chemoradiotherapy (Platinum-based): For locally advanced (Stage III/IV) disease. Cisplatin/5-FU.
- Palliative Care: For metastatic/incurable disease. Symptom control.
Of Disease
- Local Spread: Invasion to Urethra, Vagina, Anus.
- Lymph Node Metastases: Inguinal and Pelvic nodes.
- Distant Metastases: Rare at presentation (Lung, Liver, Bone).
Of Treatment
- Lymphoedema: Common after lymphadenectomy. Chronic leg swelling. Major impact on QoL.
- Wound Breakdown / Infection: Common. Delayed healing.
- Urinary/Faecal Incontinence: If extensive surgery.
- Psychosexual Dysfunction: Body image, sexual function. Important to address.
- Vaginal Stenosis: If radiotherapy used.
- Recurrence: Local recurrence is common (30-40%).
| Stage | Description | 5-Year Survival |
|---|---|---|
| IA | Tumour ≤2cm, confined to vulva, stromal invasion ≤1mm, nodes negative. | >95% |
| IB | Tumour >2cm OR stromal invasion >1mm, confined to vulva/perineum, nodes negative. | ~80% |
| II | Tumour of any size with extension to lower 1/3 urethra/vagina/anus, nodes negative. | ~70% |
| IIIA/B/C | Inguino-femoral lymph node metastases (Number and size of nodes determines sub-stage). | 40-60% |
| IVA | Tumour invades upper urethra, bladder, rectum, or pelvic bone, OR fixed/ulcerated inguinal nodes. | ~15% |
| IVB | Distant metastases (including pelvic lymph nodes). | less than 10% |
Key Guidelines
| Guideline | Organisation | Key Recommendations |
|---|---|---|
| Vulval Cancer Management | BGCS / RCOG / NICE | MDT management. Surgery primary. SLNB for select cases. Adjuvant RT if high risk. |
| VIN Management | BSSVD / ISSVD | Surveillance, Excision, or Ablation for VIN. |
Landmark Evidence
- GROINSS-V Studies: Established safety and efficacy of Sentinel Lymph Node Biopsy in vulval cancer.
What is Vulval Cancer?
It is a rare cancer affecting the skin and tissue around the opening of the vagina (the vulva). Most vulval cancers are a type called squamous cell carcinoma. It is most common in older women.
What are the symptoms?
A lump, sore, or ulcer on the vulva that doesn't heal. Persistent itching. Bleeding. Any new or changing skin lesion.
How is it treated?
Surgery to remove the cancer is the main treatment. This may involve removing part or all of the vulva (vulvectomy) and often the lymph nodes in the groin. Radiotherapy or chemotherapy may be used if the cancer has spread or cannot be fully removed by surgery.
What is the outlook?
If caught early (when the cancer is small and has not spread to the lymph nodes), the outlook is very good. The prognosis depends mainly on whether the cancer has spread to the groin lymph nodes.
Primary Sources
- Royal College of Obstetricians and Gynaecologists. Guidelines for the Diagnosis and Management of Vulval Carcinoma. 2014.
- Hacker NF, et al. Surgery for vulvar cancer. Best Pract Res Clin Obstet Gynaecol. 2012;26:271-277.
Common Exam Questions
- Risk Factor: "Chronic vulval condition associated with SCC?"
- Answer: Lichen Sclerosus.
- Histology: "Most common type of vulval cancer?"
- Answer: Squamous Cell Carcinoma (90%).
- Prognosis Factor: "Most important prognostic factor?"
- Answer: Inguinal Lymph Node Status.
- Surgery Complication: "Most common complication after groin lymphadenectomy?"
- Answer: Lymphoedema.
Viva Points
- Two Pathways to SCC: HPV-related (younger, VIN) vs HPV-independent (older, Lichen Sclerosus).
- SLNB: Know the indications and benefits (reduced lymphoedema) vs full lymphadenectomy.
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