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Radiation therapy uses high-energy rays (such as gamma rays or x-rays) and particles (such as electrons, protons, or neutrons) to kill cancer cells. In treating vulvar cancers, radiation is delivered from outside the body in a procedure that is much like having a diagnostic x-ray. This is called external beam radiation therapy. It is sometimes used along with chemotherapy to treat more advanced cancers to shrink them so they can be removed with surgery. This can sometimes allow the cancer to be removed with a less extensive surgery. Radiation alone may be used to treat lymph nodes in the groin and pelvis.
Common side effects of radiation therapy include
Tiredness
Upset stomach, nausea, or vomiting
Loose bowels or diarrhea
Serious fatigue, might also occur, but sometimes not until about 2 weeks after treatment begins. Diarrhea can usually be controlled with over-the-counter medicines. Nausea and vomiting can also be treated with medicines. These side effects tend to be worse when chemotherapy is given with radiation.
Radiation to the pelvis can also irritate the bladder (radiation cystitis), causing discomfort and an urge to urinate often. Pelvic radiation can also lead to premature menopause.
Skin changes are also common. As the radiation passes through the skin to the cancer, it may damage the skin cells. This can cause irritation ranging from mild, temporary redness to permanent discoloration. This can affect the vulvar area, making the area sensitive and sore. The skin may release fluid, which can lead to infection, so the area exposed to radiation must be carefully cleaned and protected.
Radiation can also lead to low blood counts, causing anemia (low red blood cells) and leukopenia (low white blood cells). The blood counts usually return to normal after radiation is stopped.
Women who receive radiation to the inguinal (groin) area after a lymph node dissection may have problems with the surgical wound site. It may open up or have trouble healing.
Radiation to the lymph nodes also increases the risk of lymphedema.
If you have side effects from radiation, discuss them with your cancer care team. There are often methods to relieve these symptoms.
Chemotherapy for vulvar cancer
Chemotherapy (chemo) uses anti-cancer drugs that are usually given intravenously (into a vein), by mouth, or applied to the skin in an ointment. Drugs taken by mouth or injected into a vein, called systemic chemo, enter the bloodstream and reach throughout the body, making this treatment potentially useful for cancer that has spread to distant sites.
Drugs most often used in treating vulvar cancer include cisplatin with or without fluorouracil (5-FU). Another chemo drug, mitomycin, may rarely be used.
The role of chemo in treating vulvar cancer remains to be determined. In more advanced disease, chemo might be given with radiation therapy before surgery. This combined treatment may shrink the tumor, making it easier to remove it with surgery. So far, the results of treating vulvar cancers that have spread to other organs with chemo have been disappointing.
Many of the drugs used in cancer chemo work by attacking cells that are rapidly dividing. This is helpful in killing cancer cells, but these drugs can also affect normal cells, leading to side effects. Side effects of chemo depend on the type of drugs, the amount taken, and the length of time you are treated. Common side effects include:
Nausea and vomiting
Loss or increase of appetite
Temporary loss of hair
Mouth or vaginal sores
Changes in the menstrual cycle, premature menopause, and infertility (inability to become pregnant). Most women with vulvar cancer, however, have already gone through menopause.
Chemo often affects the blood-forming cells of the bone marrow, leading to low blood counts. This can cause:
Increased chance of infections (due to low white blood cell count)
Increased chance of bleeding and bruising (due to low blood platelet count)
Tiredness (due to anemia, that is, low red blood cell count)
Other side effects can occur depending on what drug is used. Most side effects are temporary and stop when the treatment is over, but chemo drugs can have some long-lasting or even permanent effects. For example, cisplatin can cause nerve damage (called neuropathy). This can lead to numbness, tingling, or even pain in the hands and feet.
Ask your cancer care team about the chemo drugs you will receive and what side effects you can expect. Also be sure to talk with them about any side effects you do have so that they can be treated. For example, you can be given medicine to reduce or prevent nausea and vomiting.
Topical therapy for vulvar cancer
Topical therapy applies the drug directly onto the cancer. This is another way to treat vulvar intraepithelial neoplasia (VIN), but is not used to treat invasive vulvar cancer.
One choice is to apply the chemotherapy drug, fluorouracil (5-FU), directly to the skin of the vulva. This is called topical chemotherapy. Chemotherapy applied directly to the skin as an ointment will cause local irritation and peeling. This is normal and is part of the local destruction of cancer cells. Medicated ointments suggested by the health care team can help relieve the discomfort of this treatment. Topical chemotherapy for VIN is less effective than laser treatment or surgery.
A second drug that can be used topically is called imiquimod. This drug comes in a cream to be applied to the area of VIN. Imiquimod is not a chemotherapy drug. Instead, it acts by boosting the body's immune response to the area of abnormal tissue. This treatment has improved VIN, and in some women, it has caused VIN to go away completely.
Treatment options for squamous cell vulvar cancer by stage
The stage of a vulvar cancer is the most important factor in choosing treatment. However, other factors that affect this decision include the exact location of the cancer on the vulva, the type of cancer, your age, and your overall health.
Stage 0 (carcinoma in situ): Treatment options for carcinoma in situ and for less advanced pre-cancerous changes (vulvar intraepithelial neoplasia, or VIN) are the same. If left untreated, nearly all will progress to invasive vulvar cancer. Surgery, such as laser surgery, wide local excision, or a skinning vulvectomy may be used, depending on the size and location of the cancer. A topical therapy such as Fluorouracil (5-FU) ointment or imiquimod cream may be applied to the abnormal areas. Even if treated, stage 0 cancers may recur (come back) or new stage 0 cancers may form on other areas of the vulva, so good follow-up is important. The 5-year survival rate is nearly 100%, similar to pre-invasive skin cancers in other body sites.
Stage I: Treatment options depend on the size and depth of the cancer and whether the patient also has VIN. If the depth of invasion is 1 mm or less (stage IA) and there are no other areas of cancer or VIN, the cancer is surgically removed along with a 1-cm (less than half an inch) margin of the normal tissue around it.
For stage IB cancers, treatment may include a partial radical vulvectomy and inguinal lymph node dissection (removal of nearby groin lymph nodes). Sentinel lymph node biopsy may be done instead of the lymph node dissection.
Another option that is rarely used for cancers that are larger and quite extensive is a complete radical vulvectomy and removal of the groin lymph nodes.
If the lymph nodes are not removed because the patient is not healthy enough to withstand the surgery, radiation therapy to the groin areas may be given. If the lymph nodes are enlarged, a needle biopsy may be done before treatment to see if the nodes contain cancer cells.
Patients who are not healthy enough to have any surgery may be treated with radiation therapy alone.
Stage II :Stage II cancers have spread to structures near the vulva, such as the anus, the lower third of the vagina, and/or the lower third of the urethra. One option for treatment is partial radical vulvectomy (removal of the tumor, nearby parts of the vulva, and other tissues containing cancer). Surgery may also include removal of the lymph nodes in the groin on both sides of the body (or sentinel node biopsies). Radiation therapy to the area of surgery may be needed if cancer cells are at or near the margins (edges of the tissue removed by surgery).
For women who are too sick or weak from other medical problems to have surgery, radiation (with or without chemotherapy) may be used as the main treatment.
Stage III: Stage III cancers have spread to nearby lymph nodes. Treatment may include surgery to remove the cancer (either a radical wide local incision or partial or complete radical vulvectomy) and lymph nodes in the groin. This may be followed by radiation therapy. Sometimes chemotherapy (chemo) with 5-FU or cisplatin is given along with the radiation to help it work better.
These cancers may also be treated with radiation (with or without chemo) first, followed by surgery to remove any remaining cancer. This is often done to try to preserve normal structures such as the vagina, urethra and anus.
Radiation and chemo (without surgery) may be used as the main treatment for patients who cannot have surgery due to underlying medical problems.
Stage IVA: These cancers have spread more extensively to organs and tissues in the pelvis, such as the rectum (above the anus), the bladder, the pelvic bone, the upper part of the vagina, and the upper part of the urethra. When treated with surgery, the goal is to remove as much of the cancer as possible. The extent of the surgery beyond a radical vulvectomy depends on what organs contain cancer cells. Pelvic exenteration is an option, although it is used rarely. This operation includes vulvectomy and removal of the pelvic lymph nodes plus removal of some of the following: the lower colon, rectum, bladder, uterus, cervix, and vagina.
The standard approach is to combine surgery, radiation, and chemo. Radiation therapy may be done before or after surgery. Chemo may also be given before surgery. Radiation and possibly chemo can also be given to women who cannot have surgery because of prior medical problems.
Stage IVA also includes T1 and T2 tumors with less severe nearby spread but extensive spread to nearby lymph nodes that has caused the lymph nodes to become fixed (stuck to the underlying tissue) or ulcerated (become open sores). These cancers are often treated with radical vulvectomy and removal of the groin lymph nodes. Radiation (often with chemo) may be given either before or after surgery.
Stage IVB: These cancers have spread to lymph nodes in the pelvis or to organs and tissues outside the pelvis (like the lungs or liver). Surgery is not expected to cure these cancers, but may be helpful in relieving symptoms of bowel or bladder blockages. Radiation may also be helpful in shrinking the cancer and improving symptoms. Chemo may also be an option, as is enrolling in a clinical trial.
Recurrent vulvar cancer
When cancer comes back after treatment, it is called recurrent. Treatment options will depend on how soon the cancer comes back and whether the recurrence is local (in the vulva), regional (in nearby lymph nodes), or distant (has spread to organs such as the lungs or bone).
If the recurrence is local, it may still be possible to remove the cancer by surgery or by using combinations of chemo, radiation therapy, and surgery. Vulvar cancer that comes back locally more than 2 years after the initial treatment has a better prognosis (outlook) than cancers that recur sooner.
When the cancer is unresectable (has grown too large or spread too far to be surgically removed), chemo and/or radiation therapy may be used to help relieve symptoms such as pain caused by the cancer, or to shrink the tumor so that surgery may become an option. If treatment is given only to relieve pain or bleeding, it is called palliative (symptom relief) therapy.
It's very important to understand that palliative treatment is not expected to cure a cancer. Women with advanced vulvar cancer are encouraged to enter a clinical trial where they may receive new forms of therapy that may be helpful but are as yet unproven.
What will happen after treatment for vulvar cancer?
For some people with vulvar cancer, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about cancer coming back. (When cancer comes back after treatment, it is called recurrence.) This is a very common concern in people who have had cancer.
It may take a while before your fears lessen. But it may help to know that many cancer survivors have learned to live with this uncertainty and are living full lives.
For other people, the cancer may never go away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other therapies to try to help keep the cancer in check. Learning to live with cancer that does not go away can be difficult and very stressful. It has its own type of uncertainty.
Follow-up care
When treatment ends, your doctors will still want to watch you closely. It is very important to go to all of your follow-up appointments. During these visits, your doctors will ask questions about any problems you may have and may do exams and lab tests or x-rays and scans to look for signs of cancer or treatment side effects. Almost any cancer treatment can have side effects. Some may last for a few weeks to months, but others can last the rest of your life. This is the time for you to talk to your cancer care team about any changes or problems you notice and any questions or concerns you have.
It is important to keep health insurance. Tests and doctor visits cost a lot, and even though no one wants to think of their cancer coming back, this could happen.
If treatment for vulvar cancer stops working
If cancer keeps growing or comes back after one kind of treatment, it is possible that another treatment plan might still cure the cancer, or at least shrink it enough to help you live longer and feel better. But when a person has tried many different treatments and the cancer has not gotten any better, the cancer tends to become resistant to all treatment. If this happens, it's important to weigh the possible limited benefits of a new treatment against the possible downsides. Everyone has their own way of looking at this.
This is likely to be the hardest part of your battle with cancer -- when you have been through many medical treatments and nothing's working anymore. Your doctor may offer you new options, but at some point you may need to consider that treatment is not likely to improve your health or change your outcome or survival.
If you want to continue to get treatment for as long as you can, you need to think about the odds of treatment having any benefit and how this compares to the possible risks and side effects. In many cases, your doctor can estimate how likely it is the cancer will respond to treatment you are considering. For instance, the doctor may say that more chemo or radiation might have about a 1% chance of working. Some people are still tempted to try this. But it is important to think about and understand your reasons for choosing this plan.
No matter what you decide to do, you need to feel as good as you can. Make sure you are asking for and getting treatment for any symptoms you might have, such as nausea or pain. This type of treatment is called palliative care.
Palliative care helps relieve symptoms, but is not expected to cure the disease. It can be given along with cancer treatment, or can even be cancer treatment. The difference is its purpose - the main purpose of palliative care is to improve the quality of your life, or help you feel as good as you can for as long as you can. Sometimes this means using drugs to help with symptoms like pain or nausea. Sometimes, though, the treatments used to control your symptoms are the same as those used to treat cancer. For instance, radiation might be used to help relieve bone pain caused by cancer that has spread to the bones. Or chemo might be used to help shrink a tumor and keep it from blocking the bowels. But this is not the same as treatment to try to cure the cancer.
At some point, you may benefit from hospice care. This is special care that treats the person rather than the disease; it focuses on quality rather than length of life. Most of the time, it is given at home. Your cancer may be causing problems that need to be managed, and hospice focuses on your comfort. You should know that while getting hospice care often means the end of treatments such as chemo and radiation, it doesn't mean you can't have treatment for the problems caused by your cancer or other health conditions. In hospice the focus of your care is on living life as fully as possible and feeling as well as you can at this difficult time. You can learn more about hospice in our document called Hospice Care.
Staying hopeful is important, too. Your hope for a cure may not be as bright, but there is still hope for good times with family and friends -- times that are filled with happiness and meaning. Pausing at this time in your cancer treatment gives you a chance to refocus on the most important things in your life. Now is the time to do some things you've always wanted to do and to stop doing the things you no longer want to do. Though the cancer may be beyond your control, there are still choices you can make.
What`s new in vulvar cancer research and treatment?
Research is being done to find new ways to prevent and treat cancer of the vulva. There are some promising new developments.
Oncogenes and tumor suppressor genes: Scientists are learning more about how certain genes called oncogenes and tumor suppressor genes control cell growth and how changes in these genes cause normal vulvar cells to become cancerous. This information is already being used to develop new drugs that counteract the effects of these gene changes. The ultimate goal of this research is gene therapy. Gene therapy involves replacing the damaged genes in cancer cells with normal genes in order to stop the abnormal behavior of these cells.
HPV vaccines: Vaccines for preventing and treating vulvar and cervical cancer are being developed and tested.
Some of these vaccines are meant to prevent infection with certain types of HPV by boosting the body’s immunity to them. Two HPV vaccines, Gardasil and Cervarix, are available. Both vaccines are able to prevent infection with HPV types 16 and 18 and prevent pre-cancerous changes in the cervix. Studies have shown that Gardasil can also prevent anal, vulvar, and vaginal cancers caused by HPV types 6 and 11. Other preventive vaccines are also under study.
Some vaccines being studied are intended to help the immune systems of women with HPV infections destroy the virus and cure the infection before a cancer develops.
Other vaccines are meant to help women who already have a cancer or pre-cancer. These vaccines attempt to produce an immune reaction to the parts of the virus (E6 and E7 proteins) that specifically contribute to the abnormal growth of cancer cells. It is hoped that this immunity will kill the cancer cells or stop them from growing. A vaccine of this type was tested in women with grade 3 vulvar intraepithelial neoplasia (VIN3) that tested positive for HPV-16. Most women treated had their VIN lesions shrink, and in some the lesions even went away completely. More studies of this vaccine are planned.
Drug treatment:
There have been case reports of using drugs known as targeted therapies to treat vulvar cancers. These drugs do not have the same kind of side effects as traditional chemo drugs do. So far, the drugs cetuximab and erlotinib have been tried and doctors have reported some success in a few patients. Sometimes cetuximab is combined with cisplatin chemotherapy for treatment. Further studies of these drugs are needed.
Combining surgery, radiation therapy, and chemotherapy: Clinical trials are underway to determine the best way to combine surgery, radiation therapy, and chemotherapy. For example, these trials will provide information about whether certain groups of patients benefit from radiation after surgery and whether patients with cancer that has spread to lymph nodes benefit from chemotherapy or pelvic radiation therapy.
