Vulvar cancer used to almost only affect older women but is now affecting a rising number of younger women too. It is often triggered by human papillomaviruses (HPV), which can be prevented by an HPV vaccine.
At a glance
- The precursors of vulvar cancer are often caused by human papillomaviruses (HPV).
- HPV vaccines can prevent HPV-related tumors.
- Precursors of vulvar cancer have become increasingly common in recent years and also affect many younger women.
- If vulvar cancer is detected early enough and the tumor is completely removed, the chances of recovery are good.
- The prognosis largely depends on whether lymph nodes have been affected.
Note: The information in this article cannot and should not replace a medical consultation and must not be used for self-diagnosis or treatment.
What is vulvar cancer?
Vulvar cancer is a form of cancer that affects the external female reproductive organs. These include the labia majora and minora, the clitoris, the vestibule, the external urethral opening, the mons pubis and the perineum.
Vulvar cancer can develop from precursors. Medical specialists refer to these by the abbreviation “VIN”, which stands for vulvar intraepithelial neoplasia. Most precursors are caused by human papillomaviruses (HPV). If left untreated, precursors can remain unchanged, go away or develop into cancer that spreads into the surrounding tissue.
There are also much less common precursors that present themselves irrespective of HPV. One such example is Paget’s disease.
In the case of vulvar cancer, the cancer cells have spread from the skin and mucous membrane of the vulva into the connective tissue.
Vulvar cancer primarily affects older, post-menopausal women. In such cases, it often has no link to an HPV infection. However, HPV-induced vulvar cancer is becoming more prevalent – especially among younger women.
What are the symptoms of vulvar cancer?
The symptoms of vulvar cancer rarely present themselves at an early stage of the disease. There is no typical appearance. However, itchiness often occurs, even in the precursor stages.
Other possible symptoms include:
- burning sensation
- problems when urinating
- visible and palpable changes in the genital area.
Symptoms such as itching in the genital area or problems when urinating do not only occur with vulvar cancer, but often also with benign conditions. It is advisable to consult a doctor if these symptoms persist. General practitioners or gynecologists can quickly narrow down the causes of the symptoms and initiate further diagnostic steps if necessary.
Vulvar cancer: what are the causes and risk factors?
The main risk factors for vulvar cancer and its precursors include human papillomavirus (HPV) infections to the external female reproductive organs.
Women with HPV-related precursors and cancers of the cervix, vagina and anus also have an increased risk of tissue changes to the vulva.
Patients whose immune system has been weakened as a result of an organ transplant or HIV, for example, are also at increased risk of vulvar cancer.
Experts have found that vulvar cancer is more common among smokers and patients with chronic vulvar skin conditions, such as lichen sclerosus.
How common is vulvar cancer?
Vulvar cancer is the fourth most common cancer of the external female reproductive organs. Around 3,300 new cases were registered in 2016.
Most cases affect women over the age of 70. In recent years, however, there has been an increase in the number of new cases, especially among younger women. This has been attributed to the rise in human papillomavirus (HPV) infections.
Is it possible to prevent vulvar cancer?
Vaccinations against human papillomaviruses (HPV) can prevent HPV infections and thus the precursors of vulvar cancer. The vaccine is recommended for girls (and boys) between the ages of 9 and 14 years. Statutory health insurance providers cover the costs of repeat vaccinations up to the age of 17.
Important: Vaccination does not cover all types of HPV that can trigger cancer. Even women who have been vaccinated should therefore continue to keep an eye out for early signs of cancer.
Answers to frequently asked questions about HPV vaccination can be found on the German Cancer Research Center’s Cancer Information Service website (in German).
Is it possible to screen for vulvar cancer?
At present, there is no targeted, statutory screening program for vulvar cancer and its precursors in Germany.
From age 20, however, the free annual gynecological examination includes the doctor checking the entire vulva.
How is vulvar cancer diagnosed?
A doctor carefully checks the external reproductive organs by performing a gynecological examination. This enables the medical practitioner to detect any changes to the tissue in the vulva area. Gynecologists may also use a magnifying glass for a closer examination. If signs of suspected cancer or a cancer precursor are found, they take a tissue sample (punch biopsy) for laboratory examination.
If the condition is already at an advanced stage and has spread beyond the skin and mucous membrane of the vulva, further investigations are performed, including imaging procedures such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) or an examination of the neighboring organs, such as the bladder and rectum.
The question of whether and how many lymph nodes have been affected is also important. The higher the number of affected lymph nodes, the worse the prognosis. The doctor can either externally feel changes to the lymph nodes or assess them with a CT or MRI scan.
How is vulvar cancer treated?
Patients with vulvar cancer or a precursor should ideally be treated in a specialist center that employs doctors from various disciplines who specialize in cancer treatment.
The doctors and patients jointly decide which treatment is appropriate and discuss which short and long-term consequences are to be expected. In addition to the patient’s tumor stage, personal circumstances and general health, her wishes are also important.
As the tumor cells with vulvar cancer and its precursors can penetrate healthy tissue to a varying extent, there are some significant differences when it comes to treatment.
Important: Vaginal cancer and its precursors are treated in a similar way to vulvar cancer.
If you have questions regarding the treatment of vaginal cancer, you can turn to the Cancer Information Service of the German Cancer Research Center.
Mild precursors of vulvar cancer that have been caused by a human papillomavirus (HPV) infection do not initially require treatment. It is important that the cancer patient agrees to this approach and keeps an eye out for any new symptoms and problems. A doctor will regularly check the affected areas.
Doctors’ preferred method for removing HPV-induced cancer precursors that have changed more significantly is with a laser.
If there is no evidence of an HPV infection and Paget’s disease, doctors use surgery to remove cancer precursors.
Invasive vulvar cancer treatment
If the tumor cells have already spread into the surrounding tissue, the doctor surgically removes the tumor as completely as possible. Gynecological surgeons try to restore the vulva using plastic surgery.
In the case of large tumors, doctors sometimes recommend chemoradiotherapy (a combination of chemotherapy and radiotherapy) to reduce the size of the tumor prior to surgery. Chemoradiotherapy can also be an option if surgery is refused by the patient or not possible.
If the tumor has spread further in the pelvic area, chemoradiotherapy is the treatment of choice. If the cancer is spreading to the neighboring organs, it may be necessary to completely or partially remove the pelvic organs.
If the cancer cells have attacked the lymph nodes in the groin and pelvis, the doctors initially remove them surgically. Depending on the stage of the tumor, other treatment can also be required, for example chemoradiotherapy or just radiotherapy.
Remote metastasis treatment
If the cancer has spread to more distant organs, i.e. remote metastases have formed, a complete cure is not usually possible. In some situations, chemotherapy may be considered. During this phase, doctors primarily try to alleviate the symptoms in consultation with the patient and to optimally support her.
Treatment in the event of a relapse
If the cancer returns following successful treatment, i.e. if a relapse occurs, the treatment focuses on the following factors:
- What initial treatment was used?
- Is it a local relapse?
- Has the tumor spread to other organs (remote metastases)?
- How many lymph nodes are affected?
In the case of a relapse, the doctors usually remove the newly developed tumor through surgery. If this is not possible, chemoradiotherapy or radiotherapy on the vulva may be used. In the case of lymph node recurrence, surgery, radiotherapy and chemotherapy are all potential treatment options. Remote metastases in the case of a relapse are treated in the same way as during an initial diagnosis.
How is cancer treated?
The video below explains how cancer can be treated.
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How do the aforementioned treatments work, what are their potential side-effects and how can these be combated? Detailed descriptions of the aforementioned treatment options can be found on the website of the Cancer Information Service of the German Cancer Research Center (in German).
What aftercare is available for vulvar cancer and its precursors?
Aftercare primarily focuses on
- detecting a possible relapse at an early stage
- identifying and treating consequences of the disease and the treatment
- restoring and preserving a good quality of life
If the patient also has the chronic skin condition lichen sclerosus, life-long aftercare is required. This also applies after the treatment of vulvar cancer precursors.
In the first 5 years, aftercare is very regular. Patients have several aftercare appointments every year. From the 6th year after diagnosis, women can then receive aftercare just once a year.
Aftercare involves the doctor physically examining the patient, performing imaging procedures and taking tissue samples (punch biopsy) from suspected lesions on the vulva.
What can aftercare achieve, and what not? And who coordinates it? You can read about important aspects of oncological aftercare on the website of the Cancer Information Service of the German Cancer Research Center (in German).
What is life like with and after vulvar cancer?
Anatomically, the vulva is located near the bladder and the intestine. As a result, some patients find that they are no longer able to properly control their urine or stool excretions following treatment. This is referred to in medical terms as incontinence.
One measure for combating incontinence is to do targeted pelvic floor exercises. These can be provided by physiotherapists, for example. Pads, special incontinence underwear and, in some cases, a catheter for draining urine, can also help.
Further possible side-effects of the treatment can be alleviated with targeted supportive therapy. These include, for example, changes to the appearance of the vulva, a narrowing of the vagina, inflammation of the mucous membranes or lymphedema.
In addition to the physical problems, cancer also always causes psychological stress. Support from psycho-oncologists can help patients adapt to the changes in their life and deal with adverse effects and concerns. Contact with a self-help group can also be helpful.
How is a patient’s sex life affected?
Cancer in the genital area often directly affects a patient’s sex life. The disease itself and the necessary treatment can disrupt the libido and lead to sexual function limitations.
Depending on their personal circumstances this can put great strain on the woman and her partner.
In addition to treating physicians, psycho-oncologists offering sexual therapy can provide valuable professional help. There are also advice centers that offer support on sex matters.
Who can be contacted for information about vulvar cancer?
Doctors from a variety of disciplines work together closely to treat cancer of the external female reproductive organs. Hospitals with a great deal of experience in treating patients with gynecological tumors can demonstrate this through certification. The German Cancer Society (DKG) regularly checks their compliance with certain professional requirements.
You can find the addresses of the certified centers on the OncoMAP website.
Do you have further questions about dealing with the condition in everyday life and additional support options? You will find relevant information on the website of the Cancer Information Service of the German Cancer Research Center (in German).
If you have any questions about vulvar cancer, you can also contact the Cancer Information Service on 0800 - 420 30 40 (toll free) or by e-mail at email@example.com.
- Deutsche Gesellschaft für Gynäkologie und Geburtshilfe e.V. (DGGG). Diagnostik, Therapie und Nachsorge des Vulvakarzinoms und seiner Vorstufen. S2k-Leitlinie. AWMF-Registernummer 015/059. Aufgerufen am 24.06.2020.
- Zentrum für Krebsregisterdaten (ZfKD) des Robert Koch-Instituts (RKI): Krebs der Vulva (Vulvakarzinom). Aufgerufen am 19.06.2020.
In cooperation with the Cancer Information Service of the German Cancer Research Center (Krebsinformationsdienst des Deutschen Krebsforschungszentrums). As at: