Vulvar Cancer

Vulvar Cancer: Symptoms, Causes, Types, Diagnosis, and Treatments

Vulvar cancer is a rare but serious form of cancer that affects the external female genitalia. Though it accounts for only about 5% of gynecologic cancers, understanding its symptoms, causes, types, diagnostic methods, and treatment options is crucial for early detection and effective management. This comprehensive guide offers a deep dive into all aspects of vulvar-cancer to empower women and healthcare providers with vital knowledge.

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What is Vulvar Cancer?

The vulva is the outer part of the female genitalia, comprising the labia majora and minora, clitoris, vaginal opening, and surrounding skin. Vulvar cancer occurs when malignant cells form in these tissues, leading to uncontrolled growth that can invade surrounding areas and spread to other parts of the body.

Though it predominantly affects postmenopausal women, vulvar-cancer can occur at any age. Early detection significantly improves prognosis, making awareness of symptoms and risk factors key.

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Symptoms of Vulvar Cancer

Vulvar cancer symptoms can often be subtle and easily mistaken for other less serious conditions such as infections or dermatitis. Early recognition can prompt timely medical evaluation. Common symptoms include:

1. Persistent Itching or Burning

Unexplained and ongoing vulvar itching or burning that doesn’t improve with topical treatments may signal cancerous changes.

2. Pain or Tenderness

Pain during walking, sitting, or sexual intercourse could be an early symptom.

3. Visible Changes in Vulvar Skin

• Development of a lump, ulcer, or sore that doesn’t heal within a few weeks
• Thickened, raised, or wart-like growths on the vulva
• Discoloration such as red, white, or darker patches on the skin

4. Bleeding or Discharge

Unusual vaginal bleeding, spotting between periods, or a foul-smelling discharge can occur.

5. Swelling or Mass

A noticeable swelling or lump on the vulva may be felt or seen.

6. Enlarged Lymph Nodes

In advanced cases, swollen lymph nodes in the groin may develop due to cancer spread.

Important: Many of these symptoms overlap with benign conditions like infections, eczema, or psoriasis, so persistent or worsening symptoms warrant medical consultation.

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Causes and Risk Factors

The exact cause of vulvar cancer is not fully understood, but several factors increase risk:

1. Human Papillomavirus (HPV) Infection

Persistent infection with high-risk HPV strains (especially HPV 16) is strongly linked to vulvar-cancer. HPV is a sexually transmitted virus responsible for many cervical and other genital cancers.

2. Age

Most cases occur in women over 60, though younger women can also be affected, especially those with HPV infections.

3. Vulvar Intraepithelial Neoplasia (VIN)

VIN refers to precancerous changes in vulvar skin cells that can progress to invasive cancer if untreated.

4. Smoking

Tobacco use weakens immune defenses and promotes carcinogenesis, increasing vulvar-cancer risk.

5. Immunosuppression

Conditions or medications that weaken the immune system (such as HIV/AIDS or post-transplant drugs) elevate cancer risk.

6. Chronic Skin Conditions

Long-standing inflammatory diseases like lichen sclerosus or lichen planus can predispose to vulvar-cancer.

7. History of Cervical or Vaginal Cancer

Women who had cervical or vaginal cancers are at higher risk due to similar etiological factors.

8. Multiple Sexual Partners and Early Sexual Activity

Increased exposure to HPV due to these behaviors may increase risk.

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Types of Vulvar Cancer

The majority of vulvar cancers are squamous cell carcinomas, arising from the squamous epithelial cells that cover the vulva. Other, less common types include:

1. Squamous Cell Carcinoma (SCC)

• Represents approximately 90% of cases.
• Divided into two main types:
  • HPV-associated SCC: Often occurs in younger women, linked to HPV infection.
  • Non-HPV-associated SCC: More common in older women, often related to chronic inflammatory conditions.

2. Melanoma

• A rare but aggressive cancer of pigment-producing cells.
• Typically presents as a darkly pigmented lesion on the vulva.

3. Adenocarcinoma

• Originates from glandular cells, such as those in Bartholin glands.
• Rare and often more aggressive.

4. Basal Cell Carcinoma

• Very rare on the vulva.
• Usually slow growing and less aggressive.

5. Sarcoma

• A rare cancer arising from connective tissues.
• Includes types like leiomyosarcoma.

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Diagnosis of Vulvar Cancer

Diagnosing vulvar cancer involves a combination of clinical evaluation, imaging, and laboratory tests.

1. Medical History and Physical Exam

• The doctor will inquire about symptoms, sexual history, HPV vaccination status, and risk factors.
• Careful inspection and palpation of the vulva, groin, and lymph nodes.

2. Biopsy

• Definitive diagnosis requires taking a tissue sample from the suspicious lesion.
• Types of biopsy:
  • Punch biopsy
  • Excisional biopsy
• The tissue is examined microscopically to identify cancer cells.

3. Imaging Studies

• To evaluate the extent of disease and lymph node involvement:
  • Ultrasound of groin lymph nodes
  • MRI or CT scans of the pelvis
  • PET scan for detecting distant metastasis

4. Colposcopy and Cytology

• Sometimes used to assess adjacent areas and exclude other genital tract cancers.

5. Blood Tests

• Generally used to assess overall health and readiness for treatment.

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Staging of Vulvar Cancer

Once diagnosed, the cancer is staged based on size, lymph node involvement, and spread:

• Stage I: Cancer confined to the vulva, ≤2 cm (IA) or >2 cm (IB), no lymph node spread.
• Stage II: Cancer has spread to adjacent perineal structures.
• Stage III: Involvement of lymph nodes in the groin.
• Stage IV: Spread to distant organs or upper urethra, bladder, or rectum.

Staging guides treatment and prognosis.

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Treatment of Vulvar Cancer

Treatment depends on the cancer type, stage, patient’s overall health, and preferences.

1. Surgery

Surgery is the primary treatment for most vulvar-cancers and aims to remove the tumor with clear margins.

• Wide Local Excision: Removal of tumor plus some healthy tissue.
• Vulvectomy: Partial or total removal of the vulva in extensive cases.
• Lymphadenectomy: Removal of groin lymph nodes if involved or high risk.

Minimally invasive approaches are increasingly preferred to reduce complications.

2. Radiation Therapy

Radiation may be used:

• Before surgery to shrink tumors.
• After surgery if margins are unclear or lymph nodes are positive.
• As a primary treatment in patients who cannot undergo surgery.
• To treat metastatic or recurrent disease.

External beam radiation and/or brachytherapy (internal radiation) may be utilized.

3. Chemotherapy

Chemotherapy is less common but can be combined with radiation (chemoradiation) for advanced or recurrent disease. Drugs like cisplatin or 5-fluorouracil are typically used.

4. Targeted Therapy and Immunotherapy

Emerging treatments include:

• Targeted drugs aimed at specific molecular markers.
• Immunotherapy to boost the body’s immune response against cancer cells.

These are mostly under clinical trial and may be options for advanced cases.

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Prognosis and Follow-Up

• Early-stage vulvar cancer has a good prognosis with 5-year survival rates around 70-90%.
• Prognosis worsens with lymph node involvement and advanced stages.
• Regular follow-up exams are essential to detect recurrence or new lesions.
• Survivors are encouraged to maintain healthy lifestyle habits, including smoking cessation and HPV vaccination for partners or younger women.

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Prevention of Vulvar Cancer

While not all cases are preventable, risk can be reduced by:

• HPV Vaccination: Protects against high-risk HPV strains.
• Safe Sexual Practices: Limiting partners and using condoms.
• Smoking Cessation: Reduces risk of many cancers.
• Regular Gynecologic Exams: Early detection of precancerous changes.
• Prompt Treatment of Vulvar Conditions: Managing lichen sclerosus or VIN.

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Living with Vulvar Cancer: Emotional and Physical Support

A diagnosis of vulvar cancer can be physically and emotionally challenging. Support includes:

• Counseling and psychological support
• Physical therapy for post-surgical recovery
• Support groups and patient education
• Sexual health counseling due to possible changes in sensation or function

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Conclusion

Vulvar cancer, although uncommon, requires vigilance for early symptoms and prompt medical attention. Advances in diagnosis and treatment have improved outcomes significantly. Awareness about HPV’s role and preventive measures like vaccination can help reduce incidence. If you notice persistent itching, pain, skin changes, or sores in the vulvar area, do not delay consulting a healthcare provider. Early diagnosis and personalized treatment remain the keys to successful management and better quality of life.

Frequently Asked Questions (FAQs) About Vulvar Cancer

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