What is Vulvar Cancer?

Cancer can occur in anyone in any part of the body and is caused by abnormal tissue cells that are rapidly reproducing. The symptoms and treatments of each type of cancer are different depending on where in the body it is located. One type of cancer that is common among women is vulvar cancer. Vulvar cancer is a cancer of females’ external genitals or vulva. The vulva encompasses both the inner and outer lips of the vagina, the clitoris, the opening of the vagina (or introitus), and glands near the introitus. While most vulvar cancers will affect the outer lips of the vagina, there are other areas of the vulva that may be affected by cancer.

Additionally, all cancers can move to other areas in your body. For example, there are five different types of vulvar cancer, including:

• Squamous Cell Carcinoma – Almost 90% of vulvar cancers in women are this type of cancer. Squamous cell carcinoma can take multiple years to develop any symptoms and are frequently mistaken for normal body concerns.
• Vulvar Melanoma – This type of cancer comprises around 5% of all vulvar cancers. Vulvar Melanoma acts like melanomas in other spots in the body and can affect women under the age of thirty-five. Additionally, there is a higher chance of Vulvar Melanoma metastasizing or spreading to other areas in the body.
• Adenocarcinoma – Adenocarcinoma starts in women’s glandular tissues around the vulva and only makes up around 2% of vulvar cancers.
• Sarcoma – Sarcoma cancers start in the connective tissue in the vulva and are usually malignant. This means that it can multiply rapidly and uncontrollably and are prone to metastasize. This type of vulvar cancer is very rare.
• Verrucous Carcinoma – Verrucous carcinoma is a type of squamous cell carcinoma that is not commonly seen. It usually begins as a slow-growing wart on the genitals and then grows larger with time.

In general, most of these types of cancers will develop slowly and over time. Many times, cancer can be caught before it becomes too dangerous, so pelvic examinations are so important. Without treatment, all types of vulvar cancer can eventually be fatal.

History

Vulvar cancer accounts for about one in every twenty-five cancers in women, which has caused many health authorities to classify it as a more rare type of cancer. This cancer also tends to affect older women, with most of the women diagnosed with vulvar cancer being over the age of 65.

In 2014, the American Cancer Society reported that there were 4,850 new cases of vulvar cancer in women, with around 1,030 women dying of cancer. This means that vulvar cancer only makes up .6% of the cancers that are diagnosed in the United States every year. This number is even lower in the United Kingdom, with the National Health Service reporting that only 1,000 or so women are diagnosed with vulvar cancer every year. Overall, research has shown that the five-year survival rate for vulvar cancer is around 78% in the United States.

This is affected by a variety of individual factors, including the stage of cancer, the type of cancer, the patient’s age, and the patient’s general health before and during cancer treatments. The five-year survival rates for those with vulvar cancer in Stage 0 or 1 (the beginning stage) have a 90% survival rate. However, that rate drops to 20% when the lymph nodes within the pelvis are affected by vulvar cancer. Doctors state that the involvement or un-involvement of the lymph nodes is the most important predictor of survival and overall prognosis.

Hence, early diagnosis of vulvar cancer is very important for surviving this type of cancer.

Risk Factors

Although the cause of vulvar cancer is still unknown, certain risk factors will increase your chances of contracting this type of cancer.

These include:

• Aging

Unfortunately, getting older is a risk factor for this type of cancer. The chances of getting vulvar cancer increase as women age, even though a female can get vulvar cancer at any age. The average age at the vulvar cancer is diagnosed in women is 65.

• Human Papillomavirus

The human papillomavirus, or HPV as it is commonly known, is a sexually transmitted infection that greatly increases the risk of different types of cancer. These include both vulvar cancer and cervical cancer. While much younger, sexually active women are exposed to this virus, most are found to fight the infection off over time; however, some are not able to do so. In these women, the HPV infection causes changes within the cells that can lead to cancer in the future.

Regular pap smears are suggested for those who contract HPV to watch for these types of cancers.

• Smoking

Smoking any nicotine-based product increases your chances of getting cancer in any part of your body. This includes your vulva, even though smoking is nowhere near your genitals.

• Human Immunodeficiency Virus

Human Immunodeficiency Virus (HIV) is a sexually transmitted disease that greatly weakens your immune system and leaves your body vulnerable to various infections.

Having HIV or AIDS will make your body more susceptible to the HPV virus, while your lowered immune system will lessen the chances of fighting off the infection.

This boosts your chances of contracting vulvar cancer.

• Having a History of Precancerous Cells

Those who have a history of precancerous conditions of their vulvas have an increased risk of developing vulvar cancer. The most common vulva condition that leads to cancer is vulvar intraepithelial neoplasia, but the risk is small. Nevertheless, your doctor may recommend that you undergo treatment to remove any abnormal cells and come in for regular treatments if you have this condition.

• Having Vulvar Skin Conditions

Certain skin conditions of the vulva can increase your chances of developing vulvar cancer Lichen sclerosis is one of these conditions. It causes the skin of the vulva to thin and can make it very itchy, which can increase your risk of developing vulvar cancer. While cancer itself cannot be prevented, it can frequently be stopped before it does major damage to your body. That includes vulvar cancers that are not causing any symptoms.

To screen for cancers of the vulva, you can perform a self-exam of your vulva or go in for your yearly Pap test and pelvic examination.

Screening

• Self-Examination

The best way to check for vulvar cancer for many women is to report any symptoms to your primary care provider as soon as you notice them and to ensure that you go in for your yearly well-women exam.

For those who have many risk factors for vulvar cancer, it can also help to check your vulva every few weeks for any signs of cancer of the vulva. Most women conduct this self-examination with a mirror, checking to see if there are any changes in the way their vulva looks.

When a woman does this, she should be looking for any areas on their vulva that are darkly pigmented, white, irritated, and red. Keep an eye out for growths, nodules, and bumps on and around your vulva. Any ulcers or open sores that are on your vulva are cause for concern if you have not recently injured yourself. If you see any of these signs during your self-examination, you should immediately schedule an examination with your doctor to check for cancer or pre-cancer.

• Pelvic Examination by Physician

A doctor examines a women’s vulva at the same time that she has a pelvic examination completed. This is frequently done simultaneously as a Pap test or an HPV test. Be aware that neither the HPV test nor the Pap test is designed to look for vulvar cancer. To ensure that you are getting the examination you need, request a pelvic examination every year if it is not automatically done. A pelvic examination is a relatively personal exam, but it is necessary.

First, your physician will examine the outer and inner lips for any skin abnormalities. After this step, they will place a speculum inside of your vagina to spread it open. The speculum is made out of either plastic or metal and is used to ensure that the doctor can clearly see your cervix without any obstruction in the way.

Next, the physician will use a small spatula to gently scrape a sample of mucus and cells away from the exocervix. The exocervix is the surface of your cervix that is the closest to the vaginal opening. After the sample has been taken, the doctor will use a cotton-tipped swab or small brush to take a sample from inside the cervical opening. The speculum will be removed after this sample is taken.

Most women don’t find this process painful, but some find it uncomfortable due to having a sensitive cervix. After the samples have been taken, your physician will ensure that the organs in your pelvis are in good health. This is done by inserting one or two gloved fingers into the vagina while the physician palpitates the lower abdomen above with pubic bone. The doctor may also conduct a rectal exam at this time to ensure that you are in good health.

It is important to note the Pap and HPV test is not always done during a pelvic examination. This is because both the Pap and HPV tests help screen for cervical cancers and pre-cancers instead of vulvar cancer; however, many women choose to have all of these tests done simultaneously to avoid having to return to the doctor’s office, too frequently.

Symptoms

The signs and symptoms of vulvar cancer will vary depending on whether it is cancer or a pre-cancer and the type of cancer it is. Here we will go over the various symptoms that you may see if you are suffering from vulvar pre-cancer or vulvar cancer.

• Vulvar Intraepithelial Neoplasia

Vulvar Intraepithelial Neoplasia (VIN) is a pre-cancer of the vulva that frequently does not show any symptoms at all. If a woman with VIN does have any symptoms, it tends to be itching that will not go away or get better with over-the-counter remedies.

Additionally, the area of VIN can look different than normal vulvar skin, appearing thicker and lighter than the normal skin around it. Unfortunately, VIN can also be a red or pink color or darker than the area around it. This makes it difficult for women to diagnose their condition without help from a physician.

• Squamous Cell Carcinoma and Sarcoma’s

These two types of cancers are cancers that will normally present themselves with various symptoms. These can include:

• An area on the vulva that looks different from the skin around it – the abnormal skin can be darker or lighter than the rest of your skin or look red or pink.
• A lump or bump on your vulva that could feel rough. It can also feel raw, like an ulcer or like a devolving wart.
• A thickening in the skin of your vulva.
• Generalized pain and burning in your genitals that are centered on your vulva.
• Abnormal discharge and bleeding that is not linked with your normal menstrual cycle.
• An open sore that resists treatment and lasts three weeks or longer.

Verrucous carcinoma is a subtype of this type of cancer and has the same symptoms but tends to present itself with growths that are similar in consistency to genital warts. A sudden outbreak of cauliflower-like growths on the cervix, especially if you haven’t had any recent sexual activity, is cause to visit your physician.

• Vulvar Melanoma

Vulvar melanoma has many of the same symptoms as squamous cell carcinoma, but they differ in how they appear. Most vulvar melanomas are dark brown or black, but there have been melanomas that have been red, pink, white, and other colors. While they can be found on the vulva, that is somewhat rare. They are usually found on the labia major or minor and the clitoris.

Regardless of where they are found, vulvar melanomas have been found to begin as a mole on the skin, so any changes in a mole that has been present for years could be a melanoma. A good way to tell if a mole is a problem or not is to use the ABCDE rule.

A mole might be a melanoma if it has:

• One side of the mole is not the same as the other side.
• Border Irregularity – the edges of the mole are notched or ragged.
• There are color differences throughout the mole. That may mean that the mole is different shades of brown or black, or there may be pink and white skin patches.
• The mole is larger than ¼ inch, especially if it has recently grown.
• The mole has changed its size, shape, or color recently.

The most common sign of a mole that has become a melanoma is a change in your mole’s size, shape, or color; however, not all melanomas will follow the ABCDE rule. If you have noticed a change in your mole, you should ask your doctor to take a look.

• Adenocarcinoma Cancer

Adenocarcinoma, or Bartholin gland cancer, presents as a distinct mass or lump that is on either side of the vaginal opening; however, this type of cancer is very rare. Most women who visit their doctors with a lump such as this find that they are suffering from a Bartholin gland cyst, which is not cancer at all. Many of the symptoms that could mean vulvar cancer can frequently be attributed to another issue that is not life-threatening. If you are concerned about your vaginal health, you should make an appointment with your doctor for a pelvic screening.

Diagnosis and Stages

The first step to any diagnosis of vulvar cancer is a pelvic examination by your physician.

After this has been completed, if there is any question about whether or not you have cancer, a biopsy will be done to ensure there is a problem. While your body may have the symptoms of vulvar cancer, it is not a guarantee that you have cancer. The only way to make sure that growth or abnormal skin spot is cancer is to conduct a biopsy.

In this procedure, you will have a small piece of tissue removed from the abnormal area for further examination and testing. First, a pathologist will examine this sample to determine if you have pre-cancer, cancer, or no cancer at all. If the biopsy shows that you have vulvar cancer, further tests will be conducted to see your type of cancer and whether it has spread. Then, you will be sent to a gynecologic oncologist who specializes in cancers of the female reproductive system.

There you may receive further tests such as:

• Cystoscopy – This examination utilizes a lit tube to check the bladder for cancers.
• Proctoscopy – This examination also uses a lit tube to check the rectum for cancers.
• Further Examination – More examination of the pelvis may be conducted while you are under anesthesia. This allows your doctor to do a more in-depth exam of your pelvis to see how much, if at all, cancer has spread.
• Computed Tomography (CT Scan) – A CT scan may be used to find any enlarged lymph nodes that your cancer may have spread to.
• Magnetic Resonance Imaging (MRI) – An MRI may be done to examine cancerous tumors within the pelvis. They are also helpful in determining if vulvar cancer has moved into the spinal cord or the brain as well as the pelvis.
• Positron Emission Tomography (PET) – PET is used to help a physician who believes that cancer has spread but cannot locate where it has spread to.

Depending on what type of cancer you have and where it is located, you may receive some or all of these tests to determine the best course of treatment for your vulvar cancer. These tests also help to determine what stage your vulvar cancer is in.

Determining the stage of your cancer is a rather complicated process, but it is broken down into different categories, which include:

The Extent of the Tumor (T)

This category includes tumors that have not grown into any other tissue, cancer that is only growing through the vulva or perineum, cancers that are growing into the lower third of the vagina or urethra or into the anus, as well as cancers that have grown into the rectum, bladder, upper urethra, or pubic bone.

The Spread Into Lymph Nodes (N)

This category includes no spread of cancer to the lymph nodes, the spread of cancer into one or two lymph nodes through the groin, the spread of cancer into more groin lymph nodes and their relative size, and the spread of cancer into all groin lymph nodes that have caused ulcers through the vulva.

Distant Spread of Cancer (M)

This category includes cancers that have not spread beyond the vulva and those that have spread to distant sites, including the lymph nodes in the pelvis.

The grouping of these three categories and their subcategories determines the stage of your vulvar cancer. They begin at Stage 0, which is early cancer that is found on the surface of the vulva only, and end at Stage IVA, which includes three different mixtures of categories that are all late-stage vulvar cancers.

Melanoma cancers of the vulva are staged slightly differently than the rest of the vulvar cancers, but the categories are the same, with the stages differing slightly and running from Stage 0 to Stage IV.

Treatments and Drugs

Depending on the type and the stage of your vulvar cancer, you may require more than one type of treatment to get rid of your cancer. You may have a variety of doctors that are supporting you and who will work together to devise the best possible plan for your body and your type of cancer.

Treating Vulvar Pre-Cancer

If your doctor had caught the abnormal cells before they became cancer, you would usually be provided with a topical therapy that is applied directly to the vulva.

There are two standard choices for this treatment, including:

• Fluorouracil – This topical drug is a form of chemotherapy that is applied directly to the skin of your vulva. It will cause irritation and peeling wherever it is placed as it destroys the pre-cancerous cells on your vulva. Your health care team will usually alleviate that pain with medicated ointments and creams that will help you to get through the process.
• Imiquimod – Imiquimod is another drug, not a topical chemotherapy option, that comes in a cream form. It is used to treat VIN, and it works to boost your body’s immune system and normal response to an area with abnormal tissues. In many women, this has caused their VIN to be eradicated entirely.

There are other ways to treat VIN, but that will not treat any vulvar pre-cancer cells.

Radiation Therapy

Radiation therapy is commonly used for many women that have vulvar cancer. It utilizes particles and high-energy rays to kill any cancer cells in your vulva. It is applied by delivering radiation into the body in a procedure that is very similar to receiving an X-ray. Radiation can be used by itself to treat cancers in the lymph nodes of the pelvis and groin or in conjunction with chemotherapy to shrink cancer to a smaller size.

The side effects of receiving radiation therapy for vulvar cancer include:

• General Tiredness
• Issues with the stomach, including nausea and vomiting
• Changes in the skin that may be temporary or permanent
• Loose bowels or diarrhea
• Low blood counts

Some of these side effects, such as stomach ailments and issues with the bowels, can be offset by medication, but the others will frequently stay with those receiving treatment until the therapy is ended.

These side effects are also frequently worse when a patient receives chemotherapy.

Chemotherapy

Chemotherapy is an anti-cancer drug offered in topical versions for less serious cancers or by mouth or intravenously for those with more serious cancers.

Chemotherapy injected or taken orally can enter the bloodstream and reach different areas throughout the body, making it a better option for those who have cancer that has metastasized. The drugs that are used most commonly for chemotherapy include the more commonly used cisplatin and the rarely used mitomycin. In addition, chemotherapy for vulvar cancer and radiation therapy has been used to shrink cancers within the pelvis before surgery. Still, chemotherapy results for this type of cancer have been up for debate.

If your doctor chooses to include chemotherapy in your vulvar therapy, there will be side effects. That is due to the way that chemotherapy drugs work in the body.

Many of these drugs attack cells that divide rapidly, but they can also affect normal cells, which can lead to side effects such as:

• Nausea and vomiting
• A loss of hair (temporary)
• Sores in the vagina or the mouth
• Menstrual cycle changes, premature menopause, and an inability to become pregnant
• Loose stools and diarrhea
• An increased chance of getting an infection
• An increased chance of bruising and bleeding
• Overall tiredness

Other side effects have been known to occur depending on what chemotherapy drugs are being used; however, most of these side effects are only temporary and will end when the drugs are no longer being administered.

Coping and Support

Coping with vulvar cancer can be difficult for many people regardless of what stage and type of cancer you are suffering from. Suffering from the side effects of your cancer treatment and the fear that comes with your diagnosis may be a struggle, but there are ways that you can manage this diagnosis.

Dealing with Side Effects from Medication

The side effects of many vulvar cancer treatments are very harsh on the body and can cause you a lot of stress.

It is important to discuss these side effects with your doctor to see if any of them can be alleviated through a change in medication or the addition of other medications. For any side effects that cannot be removed, pain medication may assist you.

Additionally, outside support is recommended to help you with day-to-day activities during your cancer treatment. Ask your doctor how much support they think you will need depending on the medication you are on to ensure that you can continue as much of your daily life as possible. It is also suggested that those undergoing cancer treatments attend support groups.

Coping with Emotional Issues During Treatment

In addition to dealing with the worries and stress of a vulvar cancer diagnosis, those with cancer and their families have to deal with harsh treatments to remove the abnormal cells.

This process can be incredibly demanding and painful, causing those receiving treatment to suffer from emotional distress during their treatment. These issues can become even more difficult to deal with if there are other stressors outside of the diagnosis, such as a lack of health insurance, a low income, or weak or completely absent social support.

It is important to find the support you need while you are undergoing treatment for your vulvar cancer. There are support groups that have been created specifically for those who have been diagnosed with cancer and who need the community.

Other advocates can work with you to ensure that your needs are being met during your treatment so that you are not forced to attempt to solve all of your issues by yourself.

Vulvar Cancer Survivor Aftercare Support

Many cancer survivors struggle with a return to a cancer-free lifestyle after they have successfully beat their diagnosis. Many survivors are worried about a recurrence of cancer and are adjusting to their new normal, which can include:

• Changes in what you eat and do
• Changes in your support system
• New and permanent scars
• Inability to do things that you used to be able to do
• Emotional scars from the process

Because of these struggles, many who have had vulvar cancer join a cancer survivor’s support group to ensure that they have the support that they need.

As hard as the treatment may be for vulvar cancer, many survivors say that their diagnosis helped them to make important changes in their lives. Many also state that they found the support and friends that they needed through the process.