Cancer - vulva

Alternative names
Cancer - perineum

Definition
Cancer of the vulva involves tumors that originate at the external structures of the reproductive tract (the labia, the vaginal opening, the urethral opening, and the clitoris).

Causes, incidence, and risk factors

Approximately 90% of vulvar cancers are squamous cell carcinoma. About 5% are melanoma, 4% adenocarcinoma, 2% sarcoma, and 1% basal cell carcinoma.

The cause has not been identified, but a sexually-transmitted disease such as human papilloma virus (HPV, or genital warts) may play a role. Vulvar cancer is relatively rare, accounting for about 5% of all gynecological cancers and less than 1% of all new cancers in women.

Cancer of the vulva usually occurs in postmenopausal women with peak incidence between the ages of 65 and 75. However, 15% of the cases occur in women 40 or younger. Risk factors include previous HPV infection, previous cervical cancer or vaginal cancer, syphilis infection, diabetes, obesity, and hypertension.

Early lesions of the vulva that may change into vulvar cancer are called vulvar intraepithelial neoplasia.

Symptoms

     
  • Ulcer, thickening or lump       o Usually on the labia majora       o May be anywhere on the vulva  
  • Local itching, pain, burning, bleeding  
  • Pain with urination  
  • Pain with intercourse  
  • Unusual odor

Nearly 20% of women with vulvar cancer have no symptoms.

Signs and tests
A routine pelvic examination may be the best means of noting early changes. Skin changes include any color changes and the presence of lesions that may vary in size and shape. Excision and biopsy of the lesion is critical for accurate diagnosis.

Treatment
Surgical removal is the standard treatment. If the tumor is large (more than 2 cm) or has grown deeply into the underlying skin, excision of the lymph nodes in the groin may be necessary as well. Radiation, with or without chemotherapy, may be used to treat advanced tumors or tumor recurrences.

Support Groups
The stress of illness may be eased by joining a support group whose members share common experiences and problems. See cancer - support group.

Expectations (prognosis)
Five-year survival rates in women who are diagnosed and treated in the early stages of vulvar cancer can be excellent (more than 90%). The outcome depends on the size of the lesion, the type of cancer, and whether or not spreading (metastasis) to the lymph nodes of the region has occurred. Recurrence at or near the original site is fairly common.

Complications

Complications may include metastasis, as well as side effects of radiation, surgery, or chemotherapy.

Calling your health care provider
Call your health care provider if any vulvar lesion, skin color change, or local irritation persists longer than 2 weeks.

Prevention
Safer sex behaviors and reducing or controlling the risk factors may decrease the risk in some women.

Johns Hopkins patient information

Last revised: December 3, 2012
by Martin A. Harms, M.D.

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