Valval cancer
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vulval vulvar cancer overview epidemiology diagnosis treatment 2025
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Here is a comprehensive overview of Vulval (Vulvar) Cancer:
Vulval Cancer
Epidemiology
Vulvar cancer is the 4th most common gynecologic malignancy, representing ~4–6% of female genital tract malignancies and 0.6% of all cancers in women. In the US, approximately 6,020 new cases and 1,150 deaths occur annually. The overall 5-year survival is 72%. It predominantly affects postmenopausal women (most common anogenital cancer in women >70 years), though the mean age at diagnosis (~65 years) has trended downward due to rising HPV-related cases in younger women.
- Age-adjusted incidence: 2.8/100,000 (white women), 1.7/100,000 (black women)
- Incidence of in situ disease is increasing, driven by younger women (75% of in situ cases)
Histological Types
| Type | Frequency |
|---|---|
| Squamous cell carcinoma (SCC) | >80–90% |
| Melanoma | ~5% |
| Basal cell carcinoma | ~2% |
| Adenocarcinoma / Bartholin gland carcinoma | Rare |
| Sarcoma | 1–2% |
| Others (Paget disease, verrucous carcinoma, Merkel cell) | Rare |
Pathogenesis: Two Distinct Pathways
1. HPV-Associated (~30–40%)
- Related to high-risk HPV (principally HPV-16)
- Precursor lesion: usual-type VIN (uVIN) / squamous intraepithelial lesion (SIL)
- Basaloid or warty histology
- Occurs in younger women (average age ~30)
- Risk factors: early sexual debut, multiple partners, cigarette smoking, immunosuppression
- Often multicentric; 10–30% also have vaginal/cervical HPV lesions
- Spontaneous regression of VIN possible, especially in younger women
2. HPV-Independent (~60–70%)
- Arises from lichen sclerosus or lichen simplex chronicus
- Precursor: differentiated VIN (dVIN)
- Keratinizing SCC histology
- Occurs in older women (average age ~75)
- Driven by TP53 somatic mutations (also PIK3CA, NOTCH1, HRAS in some subtypes)
- Higher risk of malignant progression, especially if age >45 or immunosuppressed
Clinical Features
Symptoms:
- Vulvar pruritus (most common)
- Vulvar mass, ulcer, or warty lesion
- Vulvar bleeding or discharge
- Enlarged inguinal lymph nodes (advanced disease)
- Dysuria
Key points:
- Physician delay in diagnosis is common (especially warty-appearing lesions mistaken for condylomata)
- Any persistent, atypical vulvar lesion must be biopsied
- Concurrent vaginal and cervical lesions must be excluded
Diagnosis
- Biopsy is mandatory — Keys punch biopsy or wedge biopsy under local anesthesia
- Must include sufficient underlying dermis to assess stromal invasion depth
- Colposcopy of the cervix and vagina to exclude synchronous lesions
- Imaging (CT/MRI) to assess nodal and distant spread in advanced disease
Routes of Spread
- Direct extension — to vagina, urethra, clitoris, anus
- Lymphatic embolization — to inguinal → femoral → pelvic (external iliac) nodes
- Hematogenous — lungs, liver, bone (rare; usually late)
Lymphatic spread pattern:
- Superficial inguinal nodes (between Camper's fascia and fascia lata) → deep femoral nodes (medial to femoral vessels) → Cloquet's/Rosenmüller's node → pelvic nodes
- Bilateral drainage from clitoris, anterior labia minora, and perineum
- Contralateral nodal metastasis without ipsilateral involvement is rare (0–0.4%) for lateral tumors ≤2 cm
- Overall inguinofemoral nodal metastasis rate: ~32%
- Pelvic nodal metastasis: ~12% of cases (rare without groin involvement)
FIGO Staging (2009)
| Stage | Description |
|---|---|
| IA | Tumor confined to vulva/perineum, ≤2 cm, stromal invasion ≤1 mm, negative nodes |
| IB | Tumor confined to vulva/perineum, >2 cm OR stromal invasion >1 mm, negative nodes |
| II | Tumor of any size, adjacent spread to lower 1/3 urethra, lower 1/3 vagina, or anus; negative nodes |
| IIIA | Positive inguinofemoral nodes: (i) 1 node ≥5 mm or (ii) 1–2 nodes <5 mm |
| IIIB | (i) ≥2 nodes ≥5 mm or (ii) ≥3 nodes <5 mm |
| IIIC | Positive nodes with extracapsular spread |
| IVA | Invades upper 2/3 urethra, upper 2/3 vagina, bladder, rectal mucosa, or fixed/ulcerated nodes |
| IVB | Distant metastases including pelvic nodes |
Treatment
Primary Tumor Management
Microinvasive (Stage IA — ≤2 cm, ≤1 mm invasion):
- Wide local excision only; lymphadenectomy may be safely omitted
Early disease (T1b–early T2, unifocal):
- Radical local excision (1–2 cm margins, down to deep perineal fascia) with ipsilateral or bilateral inguinofemoral lymphadenectomy
- Vulvar conservation is preferred over radical vulvectomy for unifocal tumors
Sentinel lymph node (SLN) biopsy:
- Suitable for unifocal tumors <4 cm with clinically negative nodes
- Eliminates full inguinofemoral lymphadenectomy in node-negative patients
- Must be performed in experienced centres
Advanced disease (large T2 / T3):
- Chemoradiation followed by limited surgical resection (avoids exenteration)
- Preoperative radiation to reduce tumor bulk and preserve sphincters/urethra
Groin Node Management
- Full inguinofemoral lymphadenectomy when groin dissection is indicated
- Separate groin incisions (rather than en bloc) improve wound healing
- Contralateral groin dissection may be omitted for strictly lateral T1 tumors with negative ipsilateral nodes
- Postoperative (chemo-)radiation to the groins for patients with multiple positive inguinofemoral nodes — decreases groin recurrence risk
Recurrent Disease
- Groin recurrence is almost universally fatal
- Local recurrence may be salvageable with re-excision ± radiation
- Systemic chemotherapy (cisplatin-based) and immunotherapy (pembrolizumab for PD-L1 positive/MSI-H) for metastatic disease
Prognosis
The single most important prognostic factor is lymph node status:
| Nodal Status | 5-Year Survival |
|---|---|
| Negative groin nodes | >90% |
| Positive groin nodes | ~50% |
| Groin recurrence | Nearly 0% |
Other adverse prognostic factors: depth of invasion, tumor size, extracapsular nodal extension, and positive surgical margins.
Key Takeaway Points
(from Berek & Novak's Gynecology)
- All vulvar lesions require biopsy to avoid diagnostic delay
- Management is multidisciplinary and individualized
- Primary lesion and groin node management are determined separately
- Most T1/early T2 lesions → radical local excision
- Large T2/T3 → chemoradiation, then limited resection
- SLN dissection suitable for unifocal <4 cm, clinically node-negative tumors
- If groin dissection needed → thorough inguinofemoral lymphadenectomy
- Postoperative (chemo-)radiation for multiple positive groin nodes
Sources: Berek & Novak's Gynecology; Schwartz's Principles of Surgery, 11th ed.; Robbins, Cotran & Kumar Pathologic Basis of Disease; Sabiston Textbook of Surgery. A 2025 IJGO update on vulvar cancer also reports emerging targeted therapies with ~20% overall response rates in recurrent/metastatic vaginal/vulval cancers.
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