Vulval Cancer

1. Clinical Overview

Summary

Vulval cancer is a relatively uncommon gynaecological malignancy, representing approximately 4% of all gynaecological cancers and 0.6% of all female cancers. The vast majority (approximately 90%) are Squamous Cell Carcinomas (SCC). [1,2] There are two distinct aetiological pathways:

1. HPV-dependent pathway (30-40%): Affects younger women (40-60 years), associated with High-Risk HPV infection (types 16, 18), characterized by usual-type Vulval Intraepithelial Neoplasia (uVIN), and demonstrates warty/basaloid histology. [3]

2. HPV-independent pathway (60-70%): Affects elderly women (> 65 years), associated with chronic inflammatory dermatoses (particularly Lichen Sclerosus), characterized by differentiated VIN (dVIN), and demonstrates keratinizing SCC histology. [4]

Presentation typically involves a persistent vulval lump, ulcer, or chronic pruritus unresponsive to treatment in an elderly woman. The groin lymph node status is the single most important prognostic factor. [5] Treatment is primarily surgical, involving wide local excision with adequate margins and inguinal lymph node assessment. The introduction of Sentinel Lymph Node Biopsy (SLNB) has significantly reduced surgical morbidity, particularly lymphoedema, in appropriately selected patients. [6]

Clinical Pearls

Two Distinct Pathways: HPV-dependent (younger, multifocal, better prognosis) versus HPV-independent (elderly, lichen sclerosus, worse prognosis). These require different surveillance and prevention strategies.

Lichen Sclerosus = Lifelong Cancer Risk: Up to 4-5% of women with Lichen Sclerosus will develop vulval SCC over their lifetime. Annual surveillance is mandatory. [7]

"Biopsy Any Persistent Vulval Lesion": Any non-healing ulcer, indurated area, or treatment-resistant lesion in an older woman warrants punch biopsy. Delay in diagnosis is common.

Groin Node Status Trumps Tumour Size: A woman with a 1cm tumour and positive groin nodes has worse prognosis than one with a 5cm tumour and negative nodes. Nodal assessment is critical. [5]

SLNB Revolution: The GROINSS-V studies demonstrated that sentinel lymph node biopsy in unifocal tumours less than 4cm with clinically negative nodes is safe and dramatically reduces lymphoedema (from 25% to less than 5%). [6,8]

Psychosexual Impact: The impact on body image, sexual function, and quality of life is profound. Early involvement of clinical psychology and specialist vulval cancer nurses is essential.

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2. Epidemiology

Demographics

Risk Factors

Exam Detail: Molecular Pathogenesis - HPV-Dependent Pathway: High-risk HPV (particularly type 16) integrates into host genome. Viral oncoproteins E6 and E7 inactivate tumour suppressors p53 and pRb respectively, leading to cellular proliferation and genomic instability. Usual-type VIN (uVIN) develops, characterized by p16 overexpression (immunohistochemistry marker). Progressive accumulation of mutations leads to invasive SCC (warty/basaloid type). [3]

Molecular Pathogenesis - HPV-Independent Pathway: Chronic inflammatory dermatoses (lichen sclerosus, lichen planus) cause repeated cycles of epithelial damage and repair. TP53 mutations occur early (differentiated VIN, dVIN). Unlike HPV pathway, p16 is NOT expressed. Additional mutations in NOTCH1, CDKN2A, and PIK3CA drive progression to invasive keratinizing SCC. This pathway has worse prognosis due to intrinsic biological aggressiveness and older patient age. [4,14]

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3. Aetiology and Pathophysiology

Histological Classification

Two-Pathway Model of Vulval SCC

HPV-Dependent Pathway (30-40% of Cases)

Sequence: HPV infection → Usual-type VIN (uVIN/VIN usual type/HSIL) → Warty/Basaloid SCC

Characteristics:

• Age: Younger (35-60 years, mean ~50 years)
• HPV Status: Positive (predominantly HPV-16, also HPV-18, 33)
• Precursor Lesion: Usual-type VIN (uVIN) - high-grade squamous intraepithelial lesion (HSIL)
• Morphology: Warty (exophytic, papillary) or Basaloid (poorly differentiated, immature cells)
• Distribution: Often multifocal
• Associated Conditions: Smoking, immunosuppression, concurrent cervical/vaginal neoplasia
• Immunohistochemistry: p16 positive, p53 wild-type pattern
• Prognosis: Better 5-year survival (~80-85% Stage I) [3,15]

HPV-Independent Pathway (60-70% of Cases)

Sequence: Chronic inflammation (Lichen Sclerosus/Lichen Planus) → Differentiated VIN (dVIN) → Keratinizing SCC

Characteristics:

• Age: Elderly (> 65 years, mean ~75 years)
• HPV Status: Negative
• Precursor Lesion: Differentiated VIN (dVIN) - often subtle, adjacent to invasive cancer
• Morphology: Keratinizing SCC (well-differentiated, keratin pearl formation)
• Distribution: Usually unifocal
• Associated Conditions: Lichen sclerosus (60-80% of cases), lichen planus
• Immunohistochemistry: p16 negative, p53 mutation pattern (overexpression or null)
• Prognosis: Worse 5-year survival (~65-70% Stage I) due to aggressive biology and older age [4,14]

Exam Detail: Why Does HPV-Independent Have Worse Prognosis?

Several factors contribute:

1. Biological Aggressiveness: TP53 mutations drive genomic instability; keratinizing SCC demonstrates higher rates of lymphovascular space invasion (LVSI) and perineural invasion. [14]

2. Late Presentation: Lichen sclerosus causes chronic itching; new symptoms may be attributed to background dermatosis, delaying diagnosis.

3. Subtle Precursor: Differentiated VIN (dVIN) is difficult to detect clinically (appears as white or red areas indistinguishable from lichen sclerosus). Unlike uVIN, dVIN progresses rapidly (33% develop SCC, often within 12-36 months). [11]

4. Patient Age: Elderly patients have more comorbidities, poorer performance status, and may not tolerate aggressive treatment.

5. Field Change: Background lichen sclerosus represents widespread epithelial field change, increasing recurrence risk even after adequate excision.

Patterns of Spread

1. Local Invasion: Direct extension to vagina, urethra, anus, or perineal skin
2. Lymphatic Spread:
   • Primary drainage: Inguinofemoral lymph nodes (superficial inguinal → deep femoral → external iliac)
   • Midline/clitoral lesions: Bilateral groin drainage
   • Lateral lesions: Ipsilateral groin drainage initially; contralateral drainage if nodes involved
   • Skip metastases to pelvic nodes are rare (less than 1%) without inguinal involvement [16]
3. Haematogenous Spread: Rare at presentation. Late manifestation to lungs, liver, bone (less than 5% of cases)

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4. Clinical Presentation

Symptoms

RED FLAG SYMPTOMS:

• Any persistent vulval lesion > 4 weeks
• Pruritus unresponsive to standard treatment
• Non-menstrual vulval bleeding in postmenopausal women
• Rapid growth of existing lesion

Signs on Examination

Inspection

Palpation

Exam Detail: Examination Technique for OSCE/Clinical Exam:

1. Consent and Chaperone: Essential for intimate examination
2. Positioning: Dorsal lithotomy position, good lighting
3. Inspection: Systematically examine all vulval structures (mons pubis, labia majora, labia minora, clitoris, vestibule, perineum, perianal area)
4. Palpation: Gently palpate any lesion (size, consistency, fixation, tenderness)
5. Groin Examination: Palpate bilateral inguinal regions for lymphadenopathy (size, number, mobility, consistency)
6. Speculum Examination: Assess vaginal extension (particularly posterior fourchette and lower third of vagina)
7. Digital Examination: Assess vaginal and rectal involvement in advanced cases
8. Document: Size, site, appearance, background skin changes, nodal status

Common OSCE Pitfall: Failing to examine the groin nodes. Always include inguinal lymph node palpation in vulval lesion examination.

Examination Pearls

"If in Doubt, Biopsy": Any persistent vulval lesion unresponsive to treatment for 4 weeks requires punch biopsy. Delay is common due to patient embarrassment and clinician reluctance.

Palpable Nodes ≠ Metastases: Approximately 50% of clinically palpable inguinal nodes are reactive/inflammatory rather than metastatic. Imaging and/or SLNB/lymphadenectomy required for definitive assessment. [5]

Background Lichen Sclerosus: Look for classic features (white, atrophic skin, loss of labia minora, clitoral hood adhesions, "cigarette paper" skin). If present, lifelong surveillance needed even after cancer treatment.

Bartholin's Mass in > 40 Years: Any Bartholin's gland mass in a woman > 40 years requires biopsy to exclude Bartholin's gland carcinoma. Simple cyst unlikely.

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5. Differential Diagnosis

Vulval Lesions - Comparison Table

Exam Detail: Distinguishing VIN from Invasive SCC Clinically:

However: Clinical assessment cannot reliably distinguish VIN from early invasive SCC. Biopsy is mandatory for any suspicious lesion.

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6. Investigations

Diagnosis

Punch Biopsy (GOLD STANDARD for Diagnosis)

Staging Investigations (Once Diagnosis Confirmed)

Imaging

Examination Under Anaesthesia (EUA)

Indication:

• Large or locally advanced tumours
• Assessment of urethral, vaginal, or anal involvement
• Combined with biopsy if not previously performed
• Cystoscopy/proctoscopy if bladder/rectal involvement suspected (Stage IVA)

Sentinel Lymph Node Biopsy (SLNB)

Indications (GROINSS-V Criteria) [6,8]:

• Unifocal primary tumour
• Tumour size less than 4cm
• Clinically negative inguinal lymph nodes (no palpable nodes)
• Squamous cell histology
• Patient fit for surgery

Technique:

• Technetium-99m nanocolloid injection around tumour (day before or day of surgery)
• Lymphoscintigraphy to identify sentinel node(s)
• Blue dye injection intraoperatively (Patent Blue V)
• Gamma probe detection + visual identification
• Excision of "hot" and "blue" nodes
• Ultrastaging: serial sectioning and immunohistochemistry (cytokeratin staining) to detect micrometastases

Interpretation:

• Negative SLNB: No further groin surgery required (if less than 2mm metastasis). Close surveillance.
• Positive SLNB (macrometastasis > 2mm): Proceed to complete inguinofemoral lymphadenectomy.
• Isolated tumour cells or micrometastases (less than 2mm): Controversial; may proceed to lymphadenectomy or radiotherapy.

Benefits:

• Reduces lymphoedema from 25% (full lymphadenectomy) to less than 5% (SLNB alone) [6]
• Reduces wound complications, infection, operative time
• Maintains oncological safety (false-negative rate less than 5% in experienced hands)

Contraindications:

• Multifocal disease
• Tumour > 4cm
• Clinically suspicious/palpable groin nodes (proceed directly to lymphadenectomy)
• Previous inguinal surgery/radiotherapy (disrupted lymphatic drainage)

Baseline Investigations

• FBC: Anaemia (chronic disease, bleeding)
• U&E, LFT: Baseline renal/hepatic function (pre-operative assessment, chemotherapy planning)
• Tumour Markers: NOT routinely used for SCC (SCC antigen unreliable)
• ECG, CXR: Pre-operative assessment (elderly patients)
• HIV, HPV Testing: May be performed; HPV typing less useful than p16 immunohistochemistry

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7. Classification and Staging

FIGO Staging System (2009) for Vulval Cancer

FIGO staging for vulval cancer is surgico-pathological (based on operative findings and histology).

Key Points:

• Stage IA: Superficially invasive (≤1mm invasion) and ≤2cm. These tumours have less than 1% risk of nodal metastases; groin node dissection may be omitted in highly selected cases. [17]
• Nodal Status: Defines Stage III. Number, size, and extracapsular extension all impact prognosis.
• Contralateral Nodes: Even with unilateral disease, midline/clitoral lesions can metastasize to bilateral nodes.
• Pelvic Nodes: Stage IVB unless directly contiguous with inguinal disease.

Exam Detail: Why is Groin Node Status So Important?

1. Single Most Important Prognostic Factor: 5-year survival drops from 80-90% (node-negative) to 40-60% (node-positive). [5]

2. Number of Positive Nodes Matters:

   • 1 positive node: ~60% 5-year survival
   • 2 positive nodes: ~40% 5-year survival
   • ≥3 positive nodes: ~25% 5-year survival [10]

3. Extracapsular Extension: When tumour breaches lymph node capsule, 5-year survival drops to 25-35% even with adjuvant radiotherapy. [18]

4. Guides Adjuvant Therapy: Node-positive disease → adjuvant radiotherapy to reduce locoregional recurrence.

Recurrence Patterns:

• Node-negative disease: ~80% of recurrences are local (vulva)
• Node-positive disease: ~50% local, 50% groin/distant

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8. Management

Multidisciplinary Team (MDT) Approach

All cases of vulval cancer should be managed by a specialist Gynaecological Oncology MDT including:

• Gynaecological Oncologist (Surgeon)
• Clinical Oncologist (Radiotherapy/Chemotherapy)
• Histopathologist
• Radiologist
• Clinical Nurse Specialist (Vulval Cancer)
• Clinical Psychologist
• Lymphoedema Specialist
• Specialist Vulval Dermatologist

Management Algorithm

        SUSPECTED VULVAL CANCER
        (Persistent Lump/Ulcer/Non-Healing Lesion)
                     ↓
              PUNCH BIOPSY
                     ↓
           HISTOLOGY: SCC Confirmed
                     ↓
    STAGING: MRI Pelvis, CT Chest/Abdomen/Pelvis
              EUA if locally advanced
                     ↓
              MDT DISCUSSION
                     ↓
         CLINICAL STAGE ASSESSMENT
    ┌──────────────┴──────────────┐
EARLY STAGE                  ADVANCED STAGE
(IA, IB, II)                (III, IVA, IVB)
    ↓                              ↓
SURGICAL                    PRIMARY TREATMENT
MANAGEMENT                       OPTIONS
    ↓                         ┌────┴────┐
Wide Local Excision         CHEMORADIOTHERAPY  PALLIATIVE CARE
(1cm margin minimum)        (Cisplatin-based)   (Stage IVB)
or                                  ↓
Radical Vulvectomy         Response Assessment
(larger tumours)                    ↓
    ↓                         Surgery if resectable
GROIN NODE ASSESSMENT              +/- Adjuvant RT
    ├─ SLNB (if eligible:          
    │  less than 4cm, unifocal, cN0)        
    └─ Inguinofemoral              
       Lymphadenectomy             
       (if SLNB+, > 4cm,            
       multifocal, cN+)            
    ↓                              
POST-OP HISTOLOGY                  
    ├─ Close/Positive Margins → Re-excision or Adjuvant RT
    ├─ Node Positive → Adjuvant RT to groin ± pelvis
    └─ Node Negative, Clear Margins → Surveillance
                     ↓
         SURVEILLANCE PROGRAMME
         - Clinical exam every 3 months (Year 1-2)
         - Every 4-6 months (Year 3-5)
         - Annually thereafter
         - Lifelong if lichen sclerosus background

Surgical Treatment

Primary Tumour Excision

Surgical Principles:

1. Adequate Margins: ≥1cm gross margin; pathological margin ≥8mm associated with reduced local recurrence (from 50% to less than 10%). [19]
2. Separate Incisions: Modern "triple incision technique" (vulva + bilateral groins) reduces wound complications vs en-bloc "butterfly" incision.
3. Tissue Preservation: Preserve uninvolved tissue wherever oncologically safe (clitoris, labia) to optimize functional/sexual outcomes.
4. Reconstructive Surgery: Primary closure often possible for less than 4cm excisions; larger defects may require:
   • V-Y advancement flaps
   • Rhomboid flaps
   • Gracilis myocutaneous flap
   • Rectus abdominis myocutaneous flap (extensive defects)

Inguinofemoral Lymph Node Assessment

Laterality:

• Midline/Clitoral Tumours: Bilateral groin assessment (risk of bilateral nodal disease)
• Lateral Tumours (> 2cm from midline): Ipsilateral groin assessment initially; contralateral only if ipsilateral nodes positive

Stage IA Exception: Tumours ≤2cm with ≤1mm stromal invasion have less than 1% risk of nodal metastases. Groin node dissection may be omitted (individualized decision). [17]

Non-Surgical Treatment

Radiotherapy

Evidence for Adjuvant RT: GOG-37 trial: Adjuvant groin radiotherapy reduced groin recurrence (5% vs 24%) in node-positive disease but did not improve overall survival (OS) due to increased distant metastases. Current practice: RT for locoregional control. [20]

Chemotherapy

Management of Vulval Intraepithelial Neoplasia (VIN)

VIN III / High-Grade Squamous Intraepithelial Lesion (HSIL) is the precursor to invasive SCC.

Surveillance After VIN Treatment:

• Every 6 months for 2 years, then annually
• Lifelong if background lichen sclerosus

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9. Complications and Prognosis

Complications of Disease

Complications of Treatment

Surgical Complications

Radiotherapy Complications

Recurrence

Risk Factors for Recurrence:

• Close surgical margins (less than 8mm)
• Node-positive disease (especially ≥2 nodes or extracapsular spread)
• HPV-independent/keratinizing SCC subtype
• Lymphovascular space invasion (LVSI)
• Tumour grade (poorly differentiated)

Prognosis

Overall Survival by FIGO Stage

Prognostic Factors

Exam Detail: Viva Question: "A 68-year-old woman has a 3cm vulval SCC excised with clear 1cm margins. Two out of eight inguinal nodes are positive for metastases, each 6mm diameter, without extracapsular spread. What is her FIGO stage and what adjuvant treatment would you recommend?"

Model Answer:

• FIGO Stage IIIB: T2 (tumour > 2cm, confined to vulva) N2b (2 nodes each ≥5mm) M0
• Prognosis: 35-45% 5-year survival (Stage IIIB)
• Adjuvant Treatment: Adjuvant radiotherapy to bilateral groins recommended based on GOG-37 trial showing reduced locoregional recurrence in node-positive disease [20]
• RT Dose: 45-50 Gy in 25 fractions to bilateral inguinal regions
• Pelvic RT: Not indicated (no extracapsular spread, less than 3 nodes positive) - controversial, individualized decision
• Chemotherapy: No role for adjuvant chemotherapy in localized disease (no survival benefit demonstrated)
• Surveillance: 3-monthly clinical examination for 2 years, then 6-monthly to 5 years, then annual
• Lymphoedema Risk: Discuss with patient (40-60% risk with surgery + RT); early referral to lymphoedema service

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10. Prevention and Screening

Primary Prevention

Secondary Prevention (Early Detection)

NO Screening Programme: Unlike cervical cancer, there is no population-based screening for vulval cancer (too rare; no validated screening test).

Tertiary Prevention (Prevent Recurrence)

• Adequate Surgical Margins: ≥8mm pathological margin [19]
• Adjuvant Radiotherapy: Node-positive disease (reduces locoregional recurrence) [20]
• Smoking Cessation: Improves wound healing, reduces recurrence
• Long-term Surveillance: Detect recurrence early when potentially curable
• Management of Background Dermatosis: Continue topical steroids for lichen sclerosus even after cancer treatment

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11. Evidence and Guidelines

Key Guidelines

Landmark Evidence

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12. Examination Focus

High-Yield Exam Topics

Written Exams (MRCOG Part 2, FRANZCOG)

1. Two-Pathway Model: HPV-dependent vs HPV-independent (histology, age, precursors, prognosis)
2. FIGO Staging: Nodal substaging (IIIA/IIIB/IIIC based on number, size, extracapsular spread)
3. SLNB Criteria: less than 4cm, unifocal, cN0 (GROINSS-V)
4. Groin Node Assessment: Midline lesions → bilateral; lateral lesions → ipsilateral (initially)
5. Adjuvant Radiotherapy Indications: Close margins, node-positive disease
6. VIN Management: Excision vs ablation vs imiquimod; surveillance protocols
7. Lichen Sclerosus Cancer Risk: 4-5% lifetime risk; annual surveillance
8. Surgical Margins: ≥1cm gross, ≥8mm pathological
9. Complications: Lymphoedema (25-40% inguinofemoral), psychosexual dysfunction
10. Prognosis: Node status most important factor

Clinical Exams (OSCE, Long Case)

Scenario: "This 72-year-old woman presents with a 6-month history of vulval itching and a lump. Please examine and discuss your findings."

Examination Findings (Simulated):

• 3cm indurated, ulcerated lesion on right labium majus
• Keratinized surface, irregular margins
• Background white, atrophic skin (lichen sclerosus)
• 2cm mobile right inguinal lymph node

Expected Discussion:

1. Differential Diagnosis: Vulval SCC most likely (keratinizing type, given age and lichen sclerosus background); consider VIN, melanoma, Paget's disease
2. Investigations: Punch biopsy (diagnostic), MRI pelvis (local staging), CT CAP (metastases), baseline bloods
3. Staging: Clinical Stage IB-II (if nodes reactive) or IIIA/IIIB (if nodes metastatic)
4. Management: MDT discussion; surgery (WLE with 1cm margin + groin node assessment - likely full inguinofemoral lymphadenectomy given palpable node and 3cm tumour); adjuvant RT if nodes positive
5. Complications: Lymphoedema, wound breakdown, psychosexual impact
6. Prognosis: Depends on nodal status (80-90% if node-negative vs 40-60% if node-positive)

Viva Voce (MRCOG Part 3, FRANZCOG Oral)

Question 1: "What are the two pathways to vulval squamous cell carcinoma, and how do they differ?"

Model Answer:

Clinical Relevance: Different prevention strategies (HPV vaccination for HPV-dependent; lichen sclerosus surveillance for HPV-independent). Prognosis differs. Guides counselling.

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Question 2: "What are the indications and benefits of sentinel lymph node biopsy in vulval cancer?"

Model Answer:

Indications (GROINSS-V Criteria) [6,8]:

1. Squamous cell carcinoma (SCC)
2. Unifocal primary tumour
3. Tumour size less than 4cm
4. Clinically negative inguinal lymph nodes (cN0)
5. Patient fit for surgery

Contraindications:

• Multifocal disease (unpredictable drainage)
• Tumour > 4cm (higher risk of nodal disease)
• Clinically suspicious/palpable nodes (proceed directly to lymphadenectomy)
• Previous groin surgery/radiotherapy (altered lymphatics)

Benefits:

1. Reduced Lymphoedema: less than 5% with SLNB alone vs 25-40% with full inguinofemoral lymphadenectomy [6]
2. Reduced Wound Complications: Wound breakdown 10% (SLNB) vs 30-50% (full)
3. Shorter Operative Time: ~30-45 minutes (SLNB) vs 2-3 hours (bilateral full)
4. Shorter Hospital Stay: Often day-case or 1-2 days vs 5-7 days
5. Better Quality of Life: Preserved mobility, reduced chronic pain
6. Oncological Safety: False-negative rate less than 5% in experienced hands; 3-year survival 97% [6]

Technique: Technetium-99m + blue dye, lymphoscintigraphy, gamma probe detection, ultrastaging of nodes (serial sectioning, cytokeratin IHC)

If SLNB Positive: Proceed to completion inguinofemoral lymphadenectomy (if macrometastasis > 2mm) OR adjuvant radiotherapy (controversial; emerging evidence for RT alone without completion)

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Question 3: "A 65-year-old woman has a 2.5cm vulval SCC excised with 7mm pathological margin. Nodes are negative. What would you do next?"

Model Answer:

Clinical Scenario: Close margin (less than 8mm) but negative nodes.

Options:

1. Re-excision (preferred if anatomically feasible without functional compromise)
2. Adjuvant Radiotherapy (if re-excision not possible or patient declines)
3. Close Surveillance (if patient unfit/declines treatment)

Discussion:

Re-excision:

• Margins less than 8mm associated with 50% local recurrence vs less than 10% with ≥8mm [19]
• If vulval tissue available (i.e., not involving urethra/anus), re-excision to achieve ≥1cm gross margin
• Send for histology to confirm clear margins

Adjuvant Radiotherapy:

• If re-excision not anatomically possible (e.g., clitoral tumour, urethral involvement)
• Dose: 50-60 Gy to tumour bed
• Toxicity: Skin reactions, lymphoedema (lower than nodal RT), vaginal stenosis

Recommendation: "I would recommend re-excision if anatomically feasible to achieve adequate margins, as this reduces local recurrence risk from 50% to less than 10%. If the patient declines or re-excision would compromise urinary/sexual function significantly, I would offer adjuvant radiotherapy to the tumour bed. I would discuss both options with the patient and MDT."

Surveillance: 3-monthly for 2 years, then 6-monthly to 5 years, then annual (lifelong if lichen sclerosus background).

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Question 4: "What is differentiated VIN (dVIN) and why is it important?"

Model Answer:

Definition: Differentiated VIN (dVIN) is a precursor lesion to HPV-independent vulval squamous cell carcinoma, characterized by TP53 mutations, basal atypia on histology, and association with lichen sclerosus or lichen planus. [11]

Comparison with Usual-type VIN (uVIN):

Clinical Importance:

1. High Malignant Potential: One-third progress to invasive SCC, often rapidly
2. Difficult to Detect: Subtle clinical appearance; easily missed
3. Requires Biopsy: Cannot distinguish from lichen sclerosus clinically
4. Treatment: Excision preferred (vs ablation for uVIN) because of high progression risk and need to exclude invasion
5. Surveillance: Close follow-up (every 3-6 months) essential

Practice Point: "Any woman with lichen sclerosus who develops a new area of induration, ulceration, or treatment-resistant symptoms should have a biopsy to exclude dVIN or invasive SCC."

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Common Exam Pitfalls

❌ "HPV vaccine prevents all vulval cancers": No - only prevents 30-40% (HPV-dependent pathway); majority are HPV-independent (lichen sclerosus pathway).

❌ "All vulval cancers need bilateral groin dissection": No - lateral lesions (> 2cm from midline) with negative ipsilateral nodes do not require contralateral dissection.

❌ "SLNB can be used for all vulval cancers": No - only for unifocal less than 4cm with cN0 (GROINSS-V criteria).

❌ "Palpable groin nodes = metastases": No - 50% are reactive; imaging/histology required.

❌ "Adjuvant chemotherapy improves survival in node-positive vulval cancer": No evidence - radiotherapy is adjuvant treatment of choice.

❌ "Stage IA lesions need groin node dissection": No - less than 1% nodal risk; can omit in selected cases (individualized).

❌ "Lichen sclerosus should be treated with observation": No - potent topical steroids (clobetasol propionate 0.05%) reduce symptoms and may reduce cancer risk; annual surveillance essential.

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13. Patient and Layperson Explanation

What is Vulval Cancer?

Vulval cancer is a cancer that develops in the vulva - the area of skin around the outside of the vagina. It usually affects older women (over 65 years). The most common type is called squamous cell carcinoma, which starts in the skin cells.

There are two main ways vulval cancer can develop:

1. Related to a virus (HPV): In younger women (40-60 years), vulval cancer can be caused by the human papillomavirus (HPV) - the same virus that causes cervical cancer. This type can be prevented by the HPV vaccine.

2. Related to a long-term skin condition: In older women (over 65), vulval cancer is often linked to a skin condition called lichen sclerosus, which causes itchy, white patches on the vulva. About 1 in 20 women with lichen sclerosus will develop cancer over their lifetime, so regular check-ups are important.

What are the Symptoms?

The most common symptoms are:

• A lump, growth, or thickened area on the vulva
• A sore or ulcer that doesn't heal
• Persistent itching that doesn't get better with creams
• Bleeding (not related to periods)
• Pain or soreness
• A swelling in the groin (lymph nodes)

Important: Many of these symptoms can be caused by non-cancerous conditions, but you should always see your doctor if you have any persistent vulval symptoms, especially if you're over 60.

How is it Diagnosed?

Your doctor will:

1. Examine the vulva: Look at the affected area carefully
2. Take a biopsy: Remove a small piece of tissue (under local anaesthetic) to check for cancer cells under a microscope
3. Arrange scans: If cancer is confirmed, you'll have MRI and CT scans to see if the cancer has spread

How is it Treated?

The main treatment is surgery to remove the cancer. This might involve:

• Wide local excision: Removing the cancer and a margin of healthy tissue around it (for small cancers)
• Larger surgery: Removing more of the vulva (for bigger cancers)
• Lymph node removal: Checking or removing lymph nodes in the groin to see if the cancer has spread

Newer technique - Sentinel lymph node biopsy: For small cancers, doctors can now remove just 1-2 "sentinel" lymph nodes instead of all the groin nodes. This greatly reduces side effects like leg swelling (lymphoedema).

Other treatments:

• Radiotherapy: High-energy rays to kill cancer cells - used after surgery if the cancer was close to the edge of the removed tissue, or if lymph nodes were affected
• Chemotherapy: Cancer-killing drugs - usually combined with radiotherapy for advanced cancers

What are the Side Effects?

Treatment can cause:

• Lymphoedema: Leg swelling if groin lymph nodes are removed (25-40% with full removal, but less than 5% with sentinel node biopsy)
• Wound healing problems: The surgical area may take several weeks to heal
• Sexual problems: Changes to the vulva can affect sexual function and body image
• Scarring and tightness: Especially if radiotherapy is used

Support available: Specialist nurses, physiotherapists (for lymphoedema), psychologists, and sexual health counsellors can all help.

What is the Outlook?

The outlook depends mainly on whether the cancer has spread to the lymph nodes:

• Cancer confined to the vulva (no lymph node spread): 80-90% of women are alive 5 years later
• Cancer spread to lymph nodes: 40-60% are alive at 5 years (depends on how many nodes are affected)

If the cancer is caught early (when it's small and hasn't spread), the chances of cure are very good.

Can it be Prevented?

• HPV vaccination: The HPV vaccine (given to girls aged 12-13 in the UK) prevents the types of HPV that cause about one-third of vulval cancers
• Lichen sclerosus treatment: If you have lichen sclerosus, use prescribed steroid creams and attend annual check-ups - this can help prevent cancer developing
• Don't smoke: Smoking increases the risk of vulval cancer
• Be aware: Check your vulva regularly and report any changes to your doctor

Living After Treatment

• Regular check-ups: You'll need examinations every 3 months for the first 2 years, then less frequently
• Self-examination: You'll be taught to examine yourself and watch for any signs of the cancer coming back
• Support groups: Many women find support groups helpful for coping with physical and emotional challenges

Questions to Ask Your Doctor

1. What stage is my cancer?
2. What are my treatment options?
3. Can I have sentinel lymph node biopsy instead of full lymph node removal?
4. What are the risks and benefits of each treatment?
5. How will treatment affect my sex life?
6. What support is available for me and my family?
7. What is my prognosis?
8. What follow-up will I need?

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14. References

Primary Sources

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