- Clinical science
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.
- 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
- Pelvic exam and colposcopy
All suspicious lesions must be biopsied for histological analysis.
Vulvar dermatoses are not inherently precancerous, but they do increase the risk of squamous cell carcinoma.
- Etiology: unclear (hormonal, immunological, and/or infectious factors are believed to play a role)
- Epidemiology: postmenopausal women and, less commonly, prepubescent girls
- Parchment-like, thin, shiny vulvar skin
- Narrow, atrophic vaginal introitus resulting in dyspareunia
- Burning pain, pruritus, bleeding vulvar ulcers
- Lichen simplex chronicus is characterized by chronic itching, which provokes persistent scratching of the vulva and so causes lichenification of the skin.
- Diagnosis: Colposcopy and biopsy of suspicious lesions are required to rule out malignancy.
- Without atypical cellular morphology: local therapy with glucocorticoid-containing creams
- In the event of malignancy: surgical resection of the lesion
- Precancerous lesion caused by dysplasia of squamous cells
- International Society for the Study of Vulvar Diseases (ISSVD) classification:
- Diagnosis: tissue biopsy
- Treatment: : depending on severity, excision or ablation may become necessary
- May progress to vulvar carcinoma despite treatment (in < 10% of cases)
- 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)
- Squamous cell carcinoma (most common)
- Clear cell adenocarcinoma: seen in daughters of women who received diethylstilbestrol during pregnancy
- 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
- Vaginal bleeding
- Leukoplakia, vaginal ulceration with contact bleeding
- Malodorous discharge
- Possibly urinary frequency
- Radiotherapy: Indicated in squamous cell carcinomas. Preserves external genitalia.
- 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
The differential diagnoses listed here are not exhaustive.
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).