Vulvar cancer

Rare and aggressive gynecological malignancy of external female genital tract.

• #4 M/C gynecologic cancer and contains 5% of all malignancies of the female genital tract (after cancer of the uterine corpus, ovary, and cervix)

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Etiology

Risk factors:

The risk of developing vulvar carcinoma is related to different behavioral, reproductive, hormonal, and genetic aspects.

• Other genital cancers
• Chronic inflammatory diseases of the vulva
• Smoking
• History of genital warts
• Vulvar intraepithelial neoplasia

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Anatomy

Vulva

The vulva is comprised of the female external genitalia, which include the labia majora and minora, clitoris, vestibule, vaginal introitus, and urethral meatus. The vulva serves to direct urine flow, prevent foreign bodies from entering the urogenital tract, as well as being a sensory organ for sexual arousal. The internal pudendal artery and, to a lesser extent, the external pudendal artery are responsible for the blood supply. The ilioinguinal and genitofemoral nerve innervates the anterior part of the vulva, whereas the posterior part is innervated by the perineal branch of the posterior cutaneous nerve.

• Labia majora (50%)
• Labia minora
• Clitoris
• Bartholin gland

Lymph drainage:

The majority of the vulva is drained by lymphatics that pass laterally to the superficial inguinal lymph nodes. The clitoris and anterior labia minora may also drain directly to the deep inguinal or internal iliac lymph nodes

• Tumors along the midline in the clitoral or urethral areas may spread to either groin. From the inguinal-femoral nodes, lymphatic spread continues to the deep pelvic iliac and obturator nodes.

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Pathophysiology

Histological subtypes:

Squamous cell carcinoma (95% cases):

2 distinct histological patterns of vulvar carcinomas, with 2 different risk factor profiles:

1. HPV‐dependent usual type VIN (uVIN) | Basaloid/warty lesions (common in young women):
   • Associated with HPV simulating risk factor profile of cervical cancer.
2. HPV‐independent differentiated VIN (dVIN) | Keratinizing vulvar carcinomas (common variety, >60% and seen in older women):
   • Associated with chronic dermatoses: Lichen sclerosus et atrophicans and lichen planus

Other less common histological subtypes:

• Melanoma, basal cell
• Bartholin gland adenocarcinoma
• Sarcoma
• Paget’s disease of the vulva (rare)

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Diagnosis

Physical examination: Suspicious vulvar lesion

Pruritic, keratinized or pigmented, possibly bleeding vulvar mass in postmenopausal women.

• 4 P’s :
  • Papule (raised lesion ± bleeding)
  • Pruritic
  • Patriotic (Red, white, blue in colour)
  • Perakeratosis

Tissue biopsy:

• Punch biopsy
  • Indications:
    • Pigmented lesion or genital wart in immunocompromised or postmenopausal woman
    • Persistent genital warts despite topical therapy
    • Evolving lesion
  • Obtained via:
    • Vulvoscopy

International Federation of Gynecology and Obstetrics (FIGO)

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Management

Surgical management:

gold standard for even a small invasive carcinoma of the vulva was historically radical vulvectomy with removal of the tumor with a wide margin followed by an en bloc resection of the inguinal and often the pelvic lymph nodes.

Adjuvant chemo/radiotherapy:

Also used as a primary treatment option for patients who were not eligible for surgery or who refused surgery and also a treatment option for patients with recurrence after surgery.

• External beam radiation therapy (EBRT) with/without concurrent chemotherapy
• Completion of inguinofemoral node dissection followed by EBRT with or without concurrent chemotherapy (especially if ≥ 2 positive nodes or 1 positive node with > 2 mm metastasis) is recommended

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Summary