Vulvar Squamous Cell Carcinoma
~Introduction
Vulvar cancer is a relatively rare malignancy of the female genital tract, accounting for approximately 3–5% of all gynecologic cancers. Among the different histological types, Vulvar Squamous Cell Carcinoma (VSCC) is the most common, constituting nearly 90% of vulvar malignancies. It primarily affects postmenopausal women, although an increasing incidence has been observed in younger women due to human papillomavirus (HPV) infection.
Vulvar squamous cell carcinoma arises from the stratified squamous epithelium of the vulva, most commonly involving the labia majora, labia minora, and clitoris. Early detection is crucial, as early-stage disease has a favorable prognosis, whereas advanced disease carries significant morbidity and mortality.
~Anatomy of the Vulva
The vulva includes the following structures:
Labia majora
Labia minora
Clitoris
Vestibule
Bartholin glands
Perineum
The vulvar epithelium is primarily keratinized squamous epithelium, which explains why squamous cell carcinoma is the dominant histologic type.
~Epidemiology
VSCC most commonly occurs in women aged 60–80 years
Increasing incidence in younger women due to HPV-related disease
More common in developed countries
Risk increases with aging, immunosuppression, smoking, and chronic vulvar dermatoses
~Etiology and Pathogenesis
There are two distinct pathogenetic pathways for vulvar squamous cell carcinoma:
1. HPV-Associated Pathway
Seen in younger women
Associated with high-risk HPV types, especially HPV 16 and 18
Precursor lesion: Vulvar Intraepithelial Neoplasia (VIN), usual type
Often multifocal
Poorly differentiated or basaloid histology
2. HPV-Independent Pathway
Seen in older women
Not associated with HPV
Associated with chronic inflammatory vulvar conditions, especially:
Lichen sclerosus
Squamous hyperplasia
Precursor lesion: Differentiated VIN (dVIN)
Usually unifocal
Well-differentiated keratinizing carcinoma
~Risk Factors
Major risk factors include:
Persistent high-risk HPV infection
Smoking
Immunosuppression (HIV, transplant patients)
Chronic vulvar irritation or inflammation
Lichen sclerosus
Older age
Multiple sexual partners
Early onset of sexual activity
Poor genital hygiene
~Precursor Lesions
Vulvar Intraepithelial Neoplasia (VIN)
VIN is a premalignant lesion characterized by dysplastic changes in the vulvar epithelium.
Types of VIN
Usual-type VIN (uVIN)
HPV-related
Seen in younger women
Basaloid or warty morphology
Differentiated VIN (dVIN)
HPV-independent
Seen in older women
Associated with lichen sclerosus
Higher risk of progression to invasive carcinoma
~Clinical Features
Symptoms
Vulvar itching (pruritus)
Vulvar pain or burning sensation
Vulvar lump or mass
Non-healing ulcer
Bleeding or discharge
Dyspareunia (pain during intercourse)
Signs
Vulvar ulcer or exophytic mass
White, red, or pigmented lesions
Indurated plaque
Lesions commonly involve labia majora or minora
Enlarged inguinal lymph nodes in advanced disease
~Gross Pathology
Exophytic (fungating) growth
Ulcerative lesion with raised margins
Indurated mass
Size varies from small lesions to large destructive tumors
~Histopathology
Microscopic Features
Invasive nests and cords of malignant squamous cells
Keratin pearl formation (well-differentiated tumors)
Nuclear pleomorphism
Increased mitotic activity
Stromal invasion
Histological Subtypes
Keratinizing squamous cell carcinoma (most common)
Basaloid carcinoma
Warty carcinoma
Verrucous carcinoma (low-grade variant)
~Grading
Based on degree of differentiation:
Well-differentiated
Moderately differentiated
Poorly differentiated
Poor differentiation is associated with aggressive behavior and poor prognosis.
~Staging
Vulvar squamous cell carcinoma is staged according to the FIGO (International Federation of Gynecology and Obstetrics) staging system.
FIGO Staging
Stage I: Tumor confined to vulva/perineum
Stage II: Tumor of any size with adjacent spread (lower urethra, vagina, anus)
Stage III: Tumor with regional lymph node metastasis
Stage IV: Tumor invading upper urethra, bladder, rectum, or distant metastasis
Lymph node involvement is the most important prognostic factor.
~Patterns of Spread
Local extension to adjacent structures
Lymphatic spread to inguinal and femoral lymph nodes
Hematogenous spread (rare) to lungs, liver, bones
~Diagnosis
Clinical Examination
Thorough vulvar inspection
Palpation of inguinal lymph nodes
Biopsy
Punch biopsy or excisional biopsy
Mandatory for confirmation
Imaging
Ultrasound, CT, MRI for staging
PET scan for lymph node involvement
~Differential Diagnosis
Bartholin gland carcinoma
Vulvar melanoma
Basal cell carcinoma
Paget disease of vulva
Chronic inflammatory lesions
~Management
Surgical Treatment
Primary modality of treatment
Wide local excision or radical vulvectomy
Sentinel lymph node biopsy
Inguinofemoral lymphadenectomy if nodes are involved
Radiotherapy
Used in:
Advanced disease
Positive surgical margins
Lymph node metastasis
Can be used as primary or adjuvant therapy
Chemotherapy
Usually combined with radiotherapy
Common agents: Cisplatin, 5-fluorouracil
~Prognosis
Prognosis depends on:
Stage of disease
Tumor size
Depth of invasion
Lymph node status
Histological grade
Survival Rates
Stage I: 85–90%
Stage II: 70–80%
Stage III: 40–60%
Stage IV: <20%
~Complications
Local recurrence
Lymphedema of lower limbs
Sexual dysfunction
Psychological distress
Chronic pain
~Prevention
HPV vaccination
Smoking cessation
Early treatment of VIN
Regular gynecological examinations
Management of lichen sclerosus
~Follow-Up
Regular clinical follow-up every 3–6 months
Lifelong surveillance due to risk of recurrence
Examination of vulva and lymph nodes
~Conclusion
Vulvar squamous cell carcinoma is a significant gynecologic malignancy with distinct etiological pathways and well-defined precursor lesions. Early diagnosis and appropriate management lead to favorable outcomes, while advanced disease remains challenging. Increased awareness, HPV vaccination, and regular gynecological screening are essential in reducing disease burden. Understanding the pathology, clinical presentation, and treatment strategies is crucial for healthcare professionals to ensure optimal patient care.
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