Vulvar cancer

Types of Vulvar Cancer

Vulvar cancer can be classified into two main types based on the type of cells from which the cancer arises:

1. Squamous Cell Carcinoma (SCC):
   • The most common type of vulvar cancer, accounting for approximately 90% of cases.
   • It originates from the squamous cells that line the outer surface of the vulva.
   • This type of cancer is often associated with chronic irritation, HPV infection, and vulvar lichen sclerosus (a skin condition that causes thinning and scarring of the vulvar skin).
2. Melanoma:
   • A rarer and more aggressive form of vulvar cancer that develops from the pigment-producing cells (melanocytes) in the vulva.
   • Vulvar melanoma tends to be more aggressive and has a higher potential for metastasis (spreading to other parts of the body).
3. Adenocarcinoma:
   • This cancer originates in the glandular cells, which produce mucus or other fluids. It is quite rare in the vulva and typically affects women who were exposed to diethylstilbestrol (DES) in utero.
4. Basal Cell Carcinoma:
   • A rare type of skin cancer that can develop in the vulvar area, usually in the labia. It is often less aggressive than squamous cell carcinoma and tends to have a good prognosis when treated early.
5. Sarcoma:
   • Rare in the vulva, sarcomas are cancers that arise from the connective tissue, muscles, or blood vessels. Leiomyosarcoma and rhabdomyosarcoma are examples of sarcomas that may occur in the vulva.

Risk Factors for Vulvar Cancer

The exact cause of vulvar cancer is not fully understood, but certain factors increase the risk of developing it. These include:

1. Human Papillomavirus (HPV) Infection:
   • Infection with high-risk strains of HPV (especially HPV types 16 and 18) is a significant risk factor for vulvar squamous cell carcinoma. HPV is the same virus that is linked to cervical cancer.
   • HPV vaccination can reduce the risk of developing vulvar cancer in women.
2. Age:
   • Vulvar cancer is most commonly diagnosed in older women, particularly those over the age of 60. The risk increases with age, possibly due to prolonged exposure to risk factors like HPV or chronic conditions such as lichen sclerosus.
3. Lichen Sclerosus:
   • This is a chronic skin condition that causes thinning, whitening, and scarring of the vulvar skin. Women with untreated or poorly managed lichen sclerosus are at an increased risk of developing vulvar cancer.
4. Smoking:
   • Smoking is a major risk factor for vulvar cancer, as it is for many other types of cancer. It weakens the immune system and makes it harder for the body to clear HPV infections, which can increase the risk of developing cancer.
5. Chronic Inflammation or Irritation:
   • Conditions that cause chronic irritation or inflammation in the vulvar region, such as vulvar intraepithelial neoplasia (VIN) or lichen planus, may increase the risk of vulvar cancer.
6. Weakened Immune System:
   • Women with a compromised immune system, such as those with HIV/AIDS or who are on immunosuppressive therapy, are at a higher risk of developing vulvar cancer.
7. History of Cervical or Vaginal Cancer:
   • Women who have had cervical or vaginal cancer are at a higher risk of developing vulvar cancer, likely due to shared risk factors like HPV.

Symptoms of Vulvar Cancer

Vulvar cancer may not cause noticeable symptoms in its early stages. As the disease progresses, common symptoms may include:

• Abnormal Vulvar Itching (Pruritus):
  • Persistent itching in the vulvar area is one of the most common early symptoms of vulvar cancer.
• Visible Changes in the Vulva:
  • The development of lumps, bumps, or sores on the vulva that do not heal may indicate the presence of vulvar cancer. These changes can be painless in the early stages but may become painful as the cancer progresses.
• Painful Urination:
  • If the tumor grows and puts pressure on the urethra (the tube that carries urine out of the body), painful urination may occur.
• Painful Sexual Intercourse (Dyspareunia):
  • Pain during intercourse is common in women with vulvar cancer.
• Vulvar Bleeding:
  • Unexplained vaginal bleeding or bleeding from the vulva, especially after intercourse, may be a sign of vulvar cancer.
• Changes in Skin Appearance:
  • The skin of the vulva may become thickened, ulcerated, or have abnormal color changes, such as becoming red, white, or darkened.
• Swelling in the Groin or Pelvic Area:
  • If the cancer spreads to the lymph nodes, swelling in the groin or pelvic area may occur.

Diagnosis of Vulvar Cancer

Early diagnosis is key to better outcomes. The process for diagnosing vulvar cancer typically involves:

1. Physical Examination:
   • A pelvic exam is often the first step in diagnosing vulvar cancer. The doctor will visually inspect the vulva for abnormal lumps, sores, or skin changes.
2. Biopsy:
   • If abnormal tissue is detected, a biopsy will be performed to remove a small sample of tissue from the affected area for examination under a microscope. This is the definitive way to diagnose vulvar cancer.
3. Colposcopy:
   • If suspicious areas are found, a colposcopy may be performed. This involves using a special magnifying instrument to examine the vulva more closely.
4. Imaging Tests:
   • Ultrasound, CT scans, or MRI may be used to determine the size and extent of the cancer, as well as whether it has spread to nearby lymph nodes or other organs.
5. Lymph Node Biopsy:
   • Since vulvar cancer can spread to the lymph nodes in the groin, a sentinel lymph node biopsy may be performed to check for cancer spread. If cancer cells are found in the lymph nodes, this may indicate the need for further treatment.

Treatment of Vulvar Cancer

The treatment for vulvar cancer depends on several factors, including the stage of cancer, the size and location of the tumor, and the patient’s overall health. The main treatment options are:

1. Surgery:
   • Vulvectomy: The most common treatment for vulvar cancer is surgery, which involves removing part or all of the vulva. The extent of the surgery depends on the size and location of the tumor.
   • Sentinel Lymph Node Biopsy: If the cancer has spread to the lymph nodes, removal of nearby lymph nodes may be necessary.
   • Reconstructive Surgery: After a vulvectomy, reconstructive surgery may be performed to improve cosmetic appearance and restore function.
2. Radiation Therapy:
   • Radiation therapy may be used after surgery to target any remaining cancer cells or to treat advanced cases where surgery is not possible. It may also be used to shrink tumors before surgery.
   • External Beam Radiation is typically used for larger tumors or those that have spread to the lymph nodes.
   • Brachytherapy (internal radiation) may be used in some cases, where radioactive sources are placed inside the body near the tumor.
3. Chemotherapy:
   • Chemotherapy is often used for advanced vulvar cancer or if the cancer has spread beyond the vulva. Common drugs used for vulvar cancer include cisplatin and 5-fluorouracil (5-FU).
   • Chemotherapy may be used in combination with radiation or surgery.
4. Immunotherapy:
   • Immunotherapy is still under investigation for vulvar cancer, but it may be an option for certain advanced or recurrent cancers. It works by boosting the body’s immune system to help fight the cancer.
5. Targeted Therapy:
   • In some cases, targeted therapies that specifically target cancer cells or cancer-related pathways may be used, particularly in cases where the cancer has recurred or spread.

Prognosis and Survival Rates

The prognosis for vulvar cancer depends largely on the stage at diagnosis, the type of cancer, and the patient’s overall health.

• Early-Stage Vulvar Cancer: If the cancer is detected early and confined to the vulva, the 5-year survival rate is generally high (around 80-90%).
• Advanced Vulvar Cancer: When vulvar cancer has spread to nearby lymph nodes or distant organs, the prognosis is poorer, and the survival rate drops significantly. The 5-year survival rate for advanced stages can be as low as 30-40%.

Regular follow-up care after treatment is important to monitor for recurrence and manage any long-term side effects from treatment.

Conclusion

Vulvar cancer is a rare but serious condition that can significantly impact a woman’s health and quality of life. Early detection and treatment are essential for improving outcomes. Women should be aware of the symptoms of vulvar cancer and consult a healthcare provider if they notice any unusual changes in the vulvar area. Risk factors such as HPV infection, smoking, and lichen sclerosus can be managed or reduced, which may help in the prevention and early detection of this type of cancer.