• Clinical science

Vulvar and vaginal cancer

Abstract

Vulvar cancer is a malignancy of the outer female genitalia that predominantly occurs in postmenopausal women. Major risk factors are HPV infection, smoking, vulvar dystrophy, and vulvar or cervical intraepithelial neoplasia. Clinical manifestations of vulvar cancer include new lumps or lesions, itching, a burning sensation and, less frequently, vulvar bleeding. Suspicious lesions must be biopsied for histological analysis and to rule out other similar conditions, such as vulvar dermatoses or vulvar intraepithelial neoplasia, which both increase the risk of vulvar cancer. Vulvar cancer is staged based on the depth of the lesion and the involvement of neighboring structures. Surgical resection (radical vulvectomy) is the first-line treatment, but advanced stages may require radiotherapy and/or palliative chemotherapy. Vulvar cancer is usually associated with a poor prognosis.

Vaginal cancer is closely related to vulvar cancer in terms of etiology and histology, but it occurs inside the vagina (typically the posterior third of the vaginal wall), rather than the vulva.

Etiology

Risk factors

References:[1][2]

Classification

References:[2]

Clinical features

  • May initially be asymptomatic
  • Local pruritus, possibly with burning sensation and pain
  • Reddish, blackish, and/or whitish patches of discoloration
  • Lumps or growths of various shapes, often wart-like lesions or ulcers
  • Vulvar bleeding or discharge
  • Dysuria, dyspareunia
  • Lymphadenopathy in the groin area

References:[2][3]

Diagnostics

All suspicious lesions must be biopsied for histological analysis.

References:[2]

Differential diagnoses

Vulvar dermatoses

Vulvar dermatoses are not inherently precancerous, but they do increase the risk of squamous cell carcinoma.

Vulvar intraepithelial neoplasia (VIN)

  • Precancerous lesion caused by dysplasia of squamous cells
  • International Society for the Study of Vulvar Diseases (ISSVD) classification:
    • VIN, usual type (most common)
      • Associated with HPV
      • Commonly multifocal
    • VIN, differentiated type
      • Associated with lichen sclerosis and other dermatoses
      • Commonly unifocal
  • Diagnosis: tissue biopsy
  • Treatment: : depending on severity, excision or ablation may become necessary
  • May progress to vulvar carcinoma despite treatment (in < 10% of cases)

Vaginal cancer

  • Localization: The upper third of the posterior vaginal wall is the most common site of vaginal carcinoma.
  • Etiology: same as vulvar neoplasia (e.g., HPV 16 and 18)
  • Subtypes
    • Squamous cell carcinoma (most common)
    • Clear cell adenocarcinoma: seen in daughters of women who received diethylstilbestrol during pregnancy
    • Sarcoma botryoides
      • Rare, highly malignant rhabdomyosarcoma that arises most commonly, but not exclusively in the genitourinary system
      • Polypoid mass that resembles a bunch of grapes protruding through the vagina
      • Peak incidence before the age of 8
  • Symptoms
    • Vaginal bleeding
    • Leukoplakia, vaginal ulceration with contact bleeding
    • Malodorous discharge
    • Possibly urinary frequency
  • Diagnosis:
    • Pelvic exam
    • Colposcopy: if abnormal cytology results without a clearly visible lesion during pelvic exam
    • Biopsy of mass to determine histopathology: spindle-shaped cells, desmin positive
  • Therapy
    • Radiotherapy: Indicated in squamous cell carcinomas. Preserves external genitalia.
    • Surgical therapy
      • Involvement of the middle and upper thirds of the vagina: vaginectomy + hysterectomy ± inguinal lymphadenectomy
      • Involvement of the lower thirds of the vagina: vaginectomy + vulvectomy + inguinal lymphadenectomy
      • Infiltrating growth: removal of the affected organs

Senile vaginitis should also be considered in patients presenting with vaginal pruritus, burning, and pain.

References:[4][5][6][7][8]

The differential diagnoses listed here are not exhaustive.

Treatment

  • First-line treatment; : local excision and surgical resection (radical vulvectomy)
  • Radiotherapy and/or palliative chemotherapy: when disease metastasizes to peripheral lymph nodes or other organs

References:[9]

Prognosis

The average 5-year survival rates range from 30–50%. However, survival rates vary greatly depending on the stage of the disease (90% with Figo I; 20% with FIGO IV).

References:[10]