Vulvar Cancer

Symptoms

• Persistent itching in the vulvar area
• A lump, sore, or growth on the vulva
• Pain or tenderness in the vulvar area
• Skin changes, such as a thickened patch (plaque), a raised patch, or an ulcer that does not heal
• Changes in skin color, appearing red, white, or darker than the surrounding skin
• Abnormal bleeding or discharge not related to menstruation
• Pain during urination (dysuria)
• Pain during sexual intercourse (dyspareunia)
• Enlarged lymph nodes in the groin
• A new lump or mole on the vulva, especially one that changes shape or color
• An ulcer or sore on the vulva that persists for more than one month
• Any bleeding after menopause or unexplained bleeding from the vulva

Causes & Risk Factors

• Vulvar squamous cell carcinoma typically develops through two main pathways:
• HPV-associated pathway: This pathway is more common in younger, premenopausal women and is caused by persistent infection with high-risk types of Human Papillomavirus (HPV), particularly types 16 and 18. The precancerous lesion for this pathway is called high-grade squamous intraepithelial lesion (HSIL).
• HPV-independent pathway: This pathway is more common in older, postmenopausal women and is not linked to HPV infection. It often develops in women with chronic inflammatory skin conditions of the vulva, such as lichen sclerosus or other vulvar dermatoses. The precancerous lesion for this pathway is called differentiated VIN (dVIN) and is often associated with changes in the TP53 tumor suppressor gene.
• Age: The risk of vulvar cancer increases with age, with most cases occurring in women over 65.
• Human Papillomavirus (HPV) Infection: High-risk HPV types, especially 16 and 18, are a major cause, particularly in younger women.
• Vulvar Intraepithelial Neoplasia (VIN): This is a precancerous condition where abnormal cells are found on the surface of the vulvar skin. High-grade squamous intraepithelial lesion (HSIL) and differentiated VIN (dVIN) are precursors to invasive vulvar cancer.
• History of other HPV-related cancers: Having had cancers like cervical or vaginal cancer can increase the risk.
• Lichen Sclerosus: A chronic inflammatory skin condition of the vulva that is associated with HPV-independent vulvar cancer.
• Immunosuppression: Conditions or medications that weaken the immune system, such as HIV infection or organ transplant, increase the risk.
• Smoking: Smoking increases the risk, especially in women who also have an HPV infection.
• Lichen Planus: Another chronic inflammatory skin condition that may be a possible risk factor.
• Personal or family history of melanoma or atypical moles: This may increase the risk of vulvar melanoma, a less common type of vulvar cancer.

Diagnosis

• Medical History: Your doctor will ask about your gynecological history, including menstrual and sexual history, any past HPV infections or abnormal Pap tests, vulvar symptoms (itching, pain, lesions), vulvar skin conditions (like lichen sclerosus), smoking history, and personal or family history of cancer.
• Physical Examination: This includes a thorough visual inspection of your entire vulva, the area between the vagina and anus (perineum), and the anal region. Your doctor will also feel your vulva and the lymph nodes in your groin for any lumps or changes. A pelvic exam, including a speculum examination of the vagina and cervix, and a bimanual exam, will also be performed.
• Biopsy: This is the definitive test to diagnose vulvar cancer. A small sample of any abnormal tissue is removed and examined under a microscope by a pathologist. This can be a punch biopsy, shave biopsy, or excisional biopsy.
• Vulvoscopy/Colposcopy: A magnifying instrument is used to closely examine the vulva. Sometimes, special solutions like acetic acid or toluidine blue are applied to highlight abnormal areas and help guide the biopsy.
• Imaging Tests (for staging, not initial diagnosis): If cancer is diagnosed, imaging tests may be used to determine if it has spread (staging): Chest X-ray: To check for spread to the lungs. Computed Tomography (CT) scan: Of the abdomen and pelvis to assess lymph nodes and distant spread. Magnetic Resonance Imaging (MRI): Of the pelvis to evaluate the size of the primary tumor and how deeply it has invaded local tissues. Positron Emission Tomography (PET) scan / PET-CT: May be used to detect metastatic disease, especially in advanced cases or to evaluate lymph nodes.
• Diagnostic Confirmation: The diagnosis is confirmed when a biopsy specimen shows invasive carcinoma under microscopic examination. The cancer is then staged using systems like FIGO (International Federation of Gynecology and Obstetrics) and AJCC (American Joint Committee on Cancer) TNM systems to describe its extent.
• Differential Diagnosis: Your doctor will consider other conditions that can cause similar symptoms, such as precancerous conditions (Vulvar Intraepithelial Neoplasia), inflammatory skin conditions (lichen sclerosus, lichen planus, psoriasis, eczema), genital warts, benign tumors, or infectious ulcers (like herpes or syphilis).

Treatment Options

• Treatment for vulvar cancer is highly personalized, based on the type, size, and location of the tumor, its stage, and your overall health. A team of specialists, including a gynecologic oncologist, radiation oncologist, and medical oncologist, will work together to plan your care.
• Surgery: Wide Local Excision: Removal of the cancer along with a margin of healthy surrounding tissue, typically for small, early-stage cancers. Radical Local Excision: Removal of a larger area of tissue. Radical Vulvectomy: Removal of all or part of the vulva. This is less common now, with a preference for surgeries that preserve more function. Pelvic Exenteration: A more extensive surgery for recurrent or advanced cancer, which may involve removing the vulva, pelvic lymph nodes, and potentially parts of the bladder, rectum, or colon. Lymph Node Dissection: Sentinel Lymph Node (SLN) Biopsy: For early-stage cancers where lymph nodes do not appear suspicious. A special dye or tracer is used to identify the first lymph node(s) the cancer would likely spread to, which are then removed and checked for cancer cells. Inguinofemoral Lymphadenectomy: Removal of lymph nodes in the groin if the sentinel lymph node biopsy is positive for cancer, or if the lymph nodes are clinically suspicious.
• Medical Therapies: Chemotherapy: Often used in combination with radiation therapy (chemoradiation) for locally advanced disease, or as a primary treatment if surgery is not possible. It is also used for cancer that has spread (metastatic) or returned (recurrent). Common medications include Cisplatin, 5-fluorouracil (5-FU), Paclitaxel, and Carboplatin. Targeted Therapy: This is an area of ongoing research and is not yet widely established for vulvar cancer. Immunotherapy: Medications called checkpoint inhibitors (e.g., Pembrolizumab) may be an option for advanced vulvar cancer that has progressed after chemotherapy and has specific biomarkers (e.g., PD-L1 positive). Topical Therapy: Creams like Imiquimod or 5-FU are used for precancerous conditions (VIN), not for invasive cancer.
• Radiation Therapy: External Beam Radiation Therapy (EBRT): Uses high-energy rays to kill cancer cells. It may be used after surgery (adjuvant) if surgical margins are close or positive, or if lymph nodes are involved. It can also be a primary treatment, often with chemotherapy, for locally advanced disease to shrink tumors before surgery or for patients who cannot undergo surgery. Radiation therapy is also used for palliative care to relieve symptoms.
• Follow-up Care: After treatment, regular follow-up appointments are crucial. This typically involves a physical exam every 3-6 months for the first 2 years, then every 6-12 months for the next 3 years, and annually thereafter. Routine imaging is not usually recommended for surveillance unless there are specific clinical concerns. You will also be educated on how to perform self-examinations of the vulva.
• Referral: Any patient diagnosed with vulvar cancer should be referred to a gynecologic oncologist, and their care should be managed by a multidisciplinary team of specialists.

Frequently Asked Questions