KeratinocyteMucosalICD-10 C51
Vulval squamous cell carcinoma
Vulvar SCC; carcinoma of vulva; squamous cell carcinoma of vulva
Vulval squamous cell carcinoma accounts for approximately 90% of vulval cancers. Two distinct biological pathways are recognised — HPV-driven disease (usually arising from usual VIN / uVIN in younger women) and HPV-independent disease (typically arising from differentiated VIN / dVIN in the context of vulval lichen sclerosus in older women). Management is multidisciplinary at the gynae-oncology MDT and combines surgery, sentinel lymph node biopsy, inguinofemoral lymphadenectomy, radiotherapy and chemoradiation depending on stage. UK practice follows FIGO 2021 staging and BGCS / ESGO guidance.
CurrentLast reviewed 15 May 2026
Two pathways
- HPV-driven (~ 30% of vulval SCC) — arises from usual VIN (uVIN), typically in younger women (median 50–60); HPV-16 dominant; multifocal disease; better prognosis stage-for-stage. Smoking is an additional risk factor.
- HPV-independent (~ 70%) — arises from differentiated VIN (dVIN) in the context of long-standing vulval lichen sclerosus; older women (median 70–80); unifocal; worse prognosis stage-for-stage; TP53 mutations common.
- The HPV-independent pathway is responsible for most invasive disease in the UK; LS surveillance is therefore essential preventive care.
Clinical features
- Often presents late — vulval pruritus, soreness, ulcer, plaque, bleeding, lump or non-healing erosion.
- Common sites — labia majora (most common), labia minora, clitoris, perineum, posterior fourchette.
- Examine the entire anogenital tract — multifocal HPV disease often involves the cervix and anal canal.
- Palpate inguinal nodes carefully.
- Threshold for biopsy of any persistent vulval lesion in older women should be low.
Staging (FIGO 2021 / TNM)
- Stage I — tumour confined to the vulva; IA < 2 cm and stromal invasion ≤ 1 mm; IB other tumours confined to the vulva.
- Stage II — tumour involves lower 1/3 of urethra / vagina / anus.
- Stage III — extension to upper perineal structures and/or regional nodes: IIIA — upper 2/3 urethra, upper 2/3 vagina, bladder mucosa or rectal mucosa, OR regional node metastasis ≤ 5 mm; IIIB — regional node metastasis > 5 mm; IIIC — extracapsular (extranodal) extension.
- Stage IV — IVA: fixation to pelvic bone, OR fixed/ulcerated regional nodes; IVB: distant metastasis.
- Workup — examination under anaesthetic, biopsy mapping, CT NCAP, MRI pelvis, FDG-PET in selected cases; SLNB for stage I–II.
Management
- Early-stage (IA–IB) — radical local excision with 1 cm margin (or 8 mm pathological); SLNB for unifocal tumours < 4 cm with clinically negative groins (cN0) and > 1 mm depth (GROINSS-V); tumours ≥ 4 cm or multifocal require inguinofemoral lymphadenectomy. Inguinofemoral lymphadenectomy if SLNB positive.
- Stage II — wider radical local excision; SLNB; consider neoadjuvant chemoradiation for sphincter / urethra preservation.
- Stage III — inguinofemoral lymphadenectomy with chemoradiation; for SLN micrometastasis ≤ 2 mm, inguinofemoral radiotherapy is a safe alternative to lymphadenectomy (GROINSS-V-II); SLN metastasis > 2 mm requires inguinofemoral lymphadenectomy.
- Stage IV — chemoradiation (5-FU / mitomycin / cisplatin + RT); pembrolizumab and cemiplimab considered for PD-L1+ recurrent / metastatic disease in trial settings.
- Long-term surveillance — 3-monthly clinical examination for 2 years, 6-monthly to 5 years, then annual lifelong.
- Concurrent surveillance for cervical / anal HPV disease.
Prognosis
- Stage I — 5-year OS > 90%.
- Stage II — 5-year OS 75–85%.
- Stage III — 5-year OS 40–60%.
- Stage IV — 5-year OS 10–20%.
- HPV-driven disease has better stage-matched survival than HPV-independent dVIN-derived disease.
- Risk of recurrence at the primary site and in contralateral inguinal nodes — both require active surveillance.
References
- Olawaiye AB et al. The 2021 FIGO staging system for vulvar cancer. Int J Gynaecol Obstet; 2021.
- BGCS Vulval Cancer Guidelines 2020.
- ESGO / ESTRO / ESP guidelines for the management of patients with vulvar cancer 2023.
- Van der Zee AG et al. Sentinel node dissection is safe in the treatment of early-stage vulvar cancer (GROINSS-V). J Clin Oncol; 2008.
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