Vulvar cancer is a rare cancer of the external female genitalia. Symptoms often include vulvar skin changes, itching, pain, or bleeding.
What is vulvar cancer?
Vulvar cancer is a type of cancer that occurs on the outer surface of the female genitalia. To help you understand where this cancer occurs, it is helpful to know the different parts of the vulva. The vulva includes the:
- Labia majora (outer lips)
- Labia minora (inner lips)
- Clitoris
- Opening of the vagina
Vulvar cancer is relatively rare, accounting for less than 1% of all cancers in women. It is more often diagnosed in older women, but can occur at any age, with the average age of diagnosis around 65. The treatment plan and survival outcomes vary depending on the stage at diagnosis. AHN is here to help you navigate a diagnosis and find the right care and treatment plan for your specific condition. At AHN, we see you.
AHN Gynecologic Oncology Center of Excellence
The AHN Gynecologic Oncology Center of Excellence is a specialized center dedicated to the comprehensive care of women with gynecologic cancers and complex gynecologic conditions. We focus on cancer care from diagnosis to treatment to ringing the bell. We collaborate with specialists throughout the cancer center and hospital system for customized treatment plans and exceptional care.
Why choose AHN for vulvar cancer treatment?
At the Cancer Institute, we take a collaborative approach to your care. You meet with a devoted team of cancer specialists, typically within three days of diagnosis. Your team quickly works to design an effective treatment plan tailored to the stage of the cancer. We routinely discuss your progress and determine if we should change the treatment to better meet your needs. At the Cancer Institute, you can expect:
- Patient-centered care: We meet with you and your family to discuss the details of your treatment plan, so you feel confident and informed. You’ll see the same team of physicians throughout your care. Your doctors get to know you, your family, and your personal preferences. We also offer robust support services to keep you living the best quality of life during treatment.
- Sophisticated treatment: Your specific treatment will largely depend on if, and how far, the cancer has spread. We offer an array of options to stop the cancer in its tracks. Treatments include cutting-edge surgery, radiation therapy, or systemic therapy, such as chemotherapy and immunotherapy.
- Access to clinical trials: We participate in many clinical trials, keeping you at the forefront of the latest treatment options.
Vulvar cancer symptoms and signs
It's important to be aware of the possible signs and symptoms of vulvar cancer, even though these symptoms can sometimes be caused by other, less serious conditions. Early detection is key to successful treatment, so it's essential to consult with your health care provider if you notice any unusual changes in your vulvar area. Some common symptoms and signs of vulvar cancer include:
- Persistent itching: This is one of the most common symptoms.
- Pain or tenderness: You might experience pain, burning, or tenderness in the vulvar area.
- Changes in skin: Look for changes in the color or texture of the skin on your vulva. This could include thickening, lumps, or wart-like growths.
- Open sore or ulcer: A sore that doesn't heal within a few weeks should be evaluated by a doctor.
- A lump: A lump or growth on the vulva.
- Bleeding or discharge: Any bleeding or discharge from the vulva should be checked.
- Enlarged lymph nodes: Swollen lymph nodes in the groin area can sometimes indicate that cancer has spread.
Causes and risk factors
The exact causes of vulvar cancer are not fully understood, but certain risk factors can increase the likelihood of developing the disease. It's important to remember that having one or more risk factors does not guarantee that you will develop vulvar cancer, but it does mean that you should be particularly vigilant about monitoring your vulvar health and consulting with your doctor about any concerns. Here's an overview of some common causes and risk factors associated with vulvar cancer:
- Age: The risk of vulvar cancer increases with age, with most cases diagnosed in women over 50.
- Human papillomavirus (HPV) infection: HPV is a common virus that can cause warts and other skin changes. Certain types of HPV are strongly linked to vulvar cancer.
- Smoking: Smoking increases the risk of developing vulvar cancer.
- Weakened immune system: A weakened immune system, such as from HIV/AIDS or certain medications, can increase the risk.
- History of precancerous conditions: Having a history of vulvar intraepithelial neoplasia (VIN) increases the risk of developing vulvar cancer.
- Skin conditions: Certain skin conditions, such as lichen sclerosus, can increase the risk of vulvar cancer.
- Other cancers: History of cervical cancer or other HPV-related cancers
Vulvar cancer screening and diagnosis
There are no widely accepted, routine screening test or guideline for vulvar cancer in average-risk women. This is primarily due to the rarity of the cancer and the lack of evidence that routine screening leads to earlier detection and improved outcomes. With that said, routine yearly gynecologic care is important to identify symptoms or skin changes early.
Women are generally encouraged to perform regular self-exams of their vulva to become familiar with its normal appearance. Any new lumps, bumps, sores, or changes in skin appearance should be reported to a health care provider. Women with certain risk factors such as human papillomavirus (HPV) infection or a history of vulvar intraepithelial neoplasia (VIN) may benefit from more diligent monitoring.
During routine pelvic exams, health care providers may visually inspect the vulva. However, this is not considered a specific screening test for vulvar cancer, but rather a part of a comprehensive gynecological assessment.
If a suspicious lesion or symptom is found, the following methods are used to diagnose vulvar cancer:
- Visual examination and palpation: The health care provider will carefully examine the vulva, noting the location, size, color, and texture of any abnormalities. They will also palpate the area to feel for any underlying masses.
- Colposcopy: This procedure uses a magnifying instrument called a colposcope to get a closer look at the vulvar skin. It's often used if there are abnormal areas detected during the visual exam. Acetic acid (vinegar) may be applied to the vulva to highlight abnormal cells.
- Biopsy: A biopsy is the most important step in diagnosing vulvar cancer. It involves removing a small tissue sample from the suspicious area, which is then examined under a microscope by a pathologist. There are different types of biopsies:
- Punch biopsy: A small, circular piece of tissue is removed using a special instrument.
- Incisional biopsy: A small wedge of tissue is removed.
- Excisional biopsy: The entire abnormal area, along with a small margin of surrounding normal tissue, is removed. This can be both diagnostic and therapeutic if the lesion is small and completely removed.
- Imaging tests: Imaging tests are not typically used to diagnose vulvar cancer, but they may be used to determine if the cancer has spread to other areas, such as the lymph nodes.
- MRI (magnetic resonance imaging): Can help assess the extent of the tumor and if it has spread locally.
- CT scan (computed tomography): May be used to evaluate lymph node involvement or distant spread.
- PET/CT scan (positron emission tomography/computed tomography): Can help detect metastatic disease.
Types and stages of vulvar cancer
Vulvar cancer is not a single disease; rather, it encompasses different types of cancer that can develop in the vulvar region. These types vary in their characteristics and how they behave. It's important to understand the different types of vulvar cancer, as this knowledge helps determine the most appropriate treatment approach. Here are the common types of vulvar cancer:
- Squamous cell carcinoma (SCC): This is the most common type, accounting for about 90% of vulvar cancers. SCC develops from the squamous cells, which are the flat cells that make up the surface of the vulvar skin.
- Vulvar melanoma: Melanoma is a type of skin cancer that develops from melanocytes, the cells that produce pigment. Vulvar melanoma accounts for about 5 – 10% of vulvar cancers.
- Adenocarcinoma: This type of cancer develops from glandular cells in the vulva. Adenocarcinomas are relatively rare, accounting for only a small percentage of vulvar cancers.
- Sarcoma: Sarcomas are cancers that develop from connective tissues, such as muscle, fat, or blood vessels. Vulvar sarcomas are very rare.
- Basal cell carcinoma: This is the most common type of skin cancer overall, but it is very rare in the vulva.
Staging of Vulvar Cancer
Staging is a process used to determine the extent of cancer in the body. It helps your care team plan the most effective treatment and estimate a patient's prognosis. The staging of vulvar cancer is based on several factors, including the size of the tumor, whether the cancer has spread to nearby lymph nodes, and whether it has spread to distant parts of the body.
The staging system commonly used for vulvar cancer is the TNM system, which stands for Tumor, Node, and Metastasis:
- T (Tumor): This describes the size and extent of the primary tumor in the vulva.
- (Node): This indicates whether the cancer has spread to nearby lymph nodes in the groin.
- M (Metastasis): This indicates whether the cancer has spread to distant parts of the body, such as the lungs, liver, or bones.
Based on the TNM classifications, vulvar cancer is assigned a stage from 0 to 4:
- Stage 0: The cancer is only found in the surface layer of cells in the vulva (VIN3 or carcinoma in situ).
- Stage 1: The cancer is confined to the vulva.
- Stage 2: The cancer has spread to nearby tissues, such as the lower part of the urethra, vagina, or anus.
- Stage 3: The cancer has spread to nearby lymph nodes in the groin.
- Stage 4: The cancer has spread to distant parts of the body.
The stage of vulvar cancer is a crucial factor in determining the most appropriate treatment plan and predicting the patient's outcome.
Vulvar cancer treatment
When you’re seeking cancer treatment, you want to find personalized care that is tailored to your specific diagnosis. At AHN, our focus is on making sure each patient experiences a personalized treatment and remarkable care. Our care providers, specializing in rare cancers, understand the demands these diseases present and are highly skilled in treating them.
Surgery
Surgery is the primary treatment for early-stage vulvar cancer. The goal is to remove the cancer and a margin of healthy tissue around it. Surgical procedures may include:
- Wide local excision/simple partial vulvectomy: Removal of the tumor along with a surrounding margin of normal tissue. This is typically reserved for the earliest stage vulvar cancer (Stage IA).
- Partial or radical vulvectomy: More extensive resection of the tumor with part or all the vulva. A radical vulvectomy excises the tumor with deeper layers of tissue, Surgery could require removal of the entire labia and/or clitoris. This is typically required for larger, more invasive tumors.
- Sentinel lymph node biopsy (SLNB): A procedure to identify and remove the first lymph node(s) to which the cancer is likely to spread. If the sentinel nodes are cancer-free, it's likely that the remaining lymph nodes are also cancer-free, avoiding the need for a more extensive lymph node dissection.
- Inguinal lymphadenectomy: Complete removal of lymph nodes in the groin to examine for cancer. This procedure may be necessary if there is a clinically enlarged lymph node indicating cancer spread, if a patient is ineligible for sentinel lymph nodes or the sentinel mapping is ineffective.
Radiation therapy
Radiation therapy uses high-energy rays to kill cancer cells. It may be used:
- As a primary treatment: For women who cannot undergo surgery due to other health conditions.
- After surgery: To kill any remaining cancer cells in the vulva or lymph nodes.
- To relieve symptoms: In advanced cases, radiation therapy can help shrink tumors and alleviate pain.
Chemotherapy
Chemotherapy uses drugs to kill cancer cells throughout the body. It is not typically used as the primary treatment for early-stage vulvar cancer. However, chemotherapy may be used:
- In combination with radiation therapy: To make the cancer cells more sensitive to radiation.
- For advanced or recurrent cancer: To shrink tumors and slow the spread of the disease.
Targeted therapy
Targeted therapy drugs attack specific vulnerabilities in cancer cells. These drugs are not commonly used for vulvar cancer but may be an option in certain cases, especially in clinical trials.
Immunotherapy
Immunotherapy drugs help the body's immune system fight cancer. Some immunotherapy drugs have been approved for use in advanced vulvar cancer that has recurred or spread.
Vulvar cancer FAQs
To help you in your conversations with your AHN care team, we’ve included some answer to our patients’ frequently asked questions. Your care team is available to answer any and all of your questions, but these can aid you in getting your thoughts together prior to any appointments you may have.
Can vulvar cancer kill you?
Unfortunately, yes, vulvar cancer can be fatal. However, the prognosis is significantly better when the cancer is detected and treated early. Regular checkups and awareness of potential symptoms are crucial for improving outcomes.
What causes vulvar cancer?
The exact cause of vulvar cancer isn't always clear, but certain factors can increase the risk. These include:
- HPV (human papillomavirus): Many vulvar cancers are linked to HPV infection, particularly HPV 16.
- Lichen sclerosis: This skin condition, which causes thin, itchy skin on the vulva, can increase the risk.
- VIN (vulvar intraepithelial neoplasia): This precancerous condition can develop into invasive cancer.
- Age: The risk increases with age, with most cases occurring in those over 60.
- Smoking: Smoking can also increase the risk of developing vulvar cancer.
How to recognize vulvar cancer?
It's important to be aware of any changes in the vulvar area. Some potential signs of vulvar cancer include:
- Persistent itching
- Pain or soreness
- A lump, bump, or growth
- Changes in skin color
- Open sores or ulcers that don't heal
- Bleeding not associated with menstruation
If you notice any of these symptoms, talk to your doctor right away.
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