Vulvar Carcinoma (Vulvar Cancer): Causes, Symptoms & Treatment

Vulvar carcinoma, also known as vulvar cancer, is a relatively rare but serious cancer of the female genital area. As with all forms of cancer, early detection is critical to successful treatment for vulvar cancer.

What is vulvar cancer?

Vulvar carcinoma is a malignant, or malignant, tumor in the area of a woman’s external genital organs. This area consists of the labia minora and labia majora, as well as the clitoris and vaginal vestibule. In about 90 percent of all cases, such vulvar carcinoma develops from the uppermost layers of the mucosa and is then called squamous cell carcinoma. However, all other types of tumors, such as the so-called black skin cancer, are also subsumed under the term vulvar carcinoma, provided they occur in the area of the vulva. Vulvar carcinomas are rather rare; every year about 3,400 to 4,000 women in Germany develop the disease. The risk of developing vulvar carcinoma increases significantly with age; those affected are usually older than 60 years.

Causes

The causes of vulvar carcinoma have not yet been definitively determined, but the disease is often associated with certain risk factors. The most significant of these is infection with the so-called human papilloma viruses, or HPV. These viruses are transmitted through sexual intercourse and are associated with numerous other diseases in the genital area, including cervical cancer. The viruses can lead to skin changes in the vulva area, which in turn can develop into vulvar carcinoma. The development of vulvar cancer can also be preceded by infection with herpes viruses, chlamydia or the syphilis pathogen Treponema pallidum, as well as the so-called white callus disease (leukoplakia). A weakening of the immune system, for example due to AIDS, can also promote the development of vulvar carcinoma. Increased nicotine consumption due to smoking has also been observed in connection with vulvar carcinoma.

Symptoms, complaints, and signs

Vulvar cancer statistically affects very few women. The disease is usually diagnosed only at an advanced stage. This is because pain does not occur until then. Sometimes a gynecologist manages to detect it in its early stages during a routine examination. Sustained removal of the tumor without pain is usually possible without problems in the early stage. A distinction must be made between incipient and advanced signs of the disease. Initially, affected women complain of unusual itching. This occurs on the labia minora or between the clitoris and the urethral opening. Reddish spots or scars have also sometimes formed there. Regularly, indurations resembling warts appear. However, increased vaginal discharge also indicates vulvar carcinoma. During or after the sexual act, unusual bleeding may occur. After the tumor reaches a certain size, women often feel pain when emptying their bladder. The genital area feels sore during the day and at night. Ulcers have formed. Nodules can also be felt. Vulvar cancer can cause discomfort in other parts of the body in its advanced stages. Pain in the back is just as common as in the legs. Nerves in the spinal cord are responsible for this.

Diagnosis and progression

Particularly in the early stages, vulvar carcinoma is hardly noticeable by specific symptoms; about one fifth of all affected women feel no discomfort at all. However, persistent itching is relatively common. Further indications may be skin changes in the form of reddish spots or wart-like elevations, as well as discharge, which may also be bloody. In the advanced stage, ulcers or nodules develop whose surface structure is comparable to that of a cauliflower. Initial examinations at the doctor’s office include palpation and colposcopy. With the colposcope, the mucous membrane of the vulva is shown several times enlarged and can thus be better viewed for abnormalities. A definitive diagnosis of vulvar cancer is made by biopsy, which is the removal and examination of a tissue sample. The earliest possible diagnosis is advantageous because vulvar carcinomas can form metastases in the neighboring lymph nodes even in early stages.

Complications

If vulvar cancer is detected in time, complications are usually not expected. However, if therapy begins at an advanced stage, the risk that the tumor will begin to spread increases. In these cases, metastases form, which often first affect the lymphatic system, especially the lymph nodes in the groin area. In the next step, carcinomas then form in the pelvis. If the patient does not respond to treatment attempts, other internal organs, especially the liver or kidneys, may also be affected in the further course. This can lead to further serious, possibly life-threatening, complications such as multiple organ failure. Complications can also result from the therapy itself. In the case of vulvar cancer, the tumor is usually removed surgically. As with any surgery, this can result in severe bleeding and infection of the surgical wound and, as a result, sepsis. Furthermore, the tumor is often subjected to radiation therapy prior to surgery, especially if it is already very large, in order to shrink it. The side effects of radiation therapy are for most patients much less stressful than those of chemotherapy, but there may be side effects that massively reduce the quality of life. For example, sensitive individuals and patients whose general condition is already severely weakened may suffer damage to the mucous membrane of the mouth and throat, as well as to the bladder and genital organs. Severe gastrointestinal disturbances are also occasionally observed.

When should you see a doctor?

Vulvar carcinoma can naturally develop exclusively in women or girls. Therefore, they belong to risk group of the disease. A doctor must be consulted already at the first disturbances and irregularities of the female sex. The disease can end in premature death without early medical and medical care. Therefore, acute action is required if visual abnormalities occur or discomfort is noticed. Swelling, itching or disturbances of the toilet should be discussed with a physician. If there are irregularities in the emptying of the bladder, this should be seen as an early warning signal. Pain, a general feeling of discomfort, or internal restlessness should be investigated. Hardening of the labia, the formation of scars or spots, and palpated warts and lumps should be examined and treated. If there are irregularities in the sexual act, changes in libido or disturbances in menstruation, a doctor should be consulted. If existing complaints on the body continue to spread or an increase in intensity is observed, action is required. A doctor should be consulted as soon as possible if problems of the back are noticed in addition to discomfort in the genital area. Irregularity of locomotion, discomfort in contact with textiles in the genital area, as well as an unusual discharge from the vagina must be investigated.

Treatment and therapy

In the overwhelming majority of cases, vulvar cancer is treated by vulvectomy, which means that the vulvar cancer is removed during surgery. How extensive this surgery is depends on the progression of the disease. Its goal is to remove the vulvar cancer completely, that is, including any metastases that may be present. Therefore, the surgery sometimes includes adjacent lymph nodes, uterus, urinary bladder or bowel. Depending on the location and size of the vulvar cancer, it may be advisable to irradiate the tumor before its surgical removal in order to reduce its size in this way. Radiation after surgery serves the purpose of reducing the risk of recurrence of vulvar cancer. Chemotherapy may be necessary to treat metastases. Depending on the extent of the vulvectomy, it may be followed by other procedures to restore and maintain the function of the reproductive organs. After surgery, follow-up is required every three months for the first two years and every six months for the next three years.

Prevention

Prevention of vulvar cancer is possible only to a limited extent. However, vaccination of young women against human papilloma virus, as recommended for cervical cancer screening, can eliminate a major risk factor for vulvar cancer as well.Regular cancer screening by a gynecologist is also crucial for detection as early as possible.

Follow-up care

Following the treatment of vulvar cancer, clinically oriented follow-up care is performed. In this process, the patient attends regular gynecological examinations. During the first three years, follow-up examinations are performed every quarter. In the following two years, they take place every six months, and thereafter at intervals of one year. The examining physician looks at the patient’s medical history and performs a symptom-related gynecological examination. If the follow-up examinations reveal abnormal findings, a tissue sample (biopsy) is taken. The goals of follow-up care also include the detection of secondary symptoms or concomitant diseases of vulvar cancer and their appropriate therapy. Possible relapses can also be detected and treated at an early stage in this way. It is therefore extremely important that the patient keeps regular appointments for check-ups. Part of the check-ups are clinical examinations of the vulvar region and examination of the groin area, which is usually done by sonography (ultrasound examination). If a recurrence is suspected, imaging procedures such as computed tomography (CT) or magnetic resonance imaging (MRI) take place.

Here’s what you can do yourself

The everyday life of affected women is usually severely restricted by the cancer. Due to the necessary treatments, many female patients are unable to pursue their professional activities for an extended period of time. In most cases, the disease also places a great psychological burden on the affected woman, her partner and the family. Those affected can find help in self-help groups or in Internet forums for cancer patients and their relatives, among other places. Vulvar cancer can also lead to difficulties in the partnership. Both the disease and its treatment can result in a complete loss of libido. An understanding partnership environment is therefore helpful. Depending on the location and course of the vulvar cancer, the disease is treated surgically by partial removal of the vulva. This is usually very stressful for the women affected. In some cases, the involvement of a couples therapist or psychologist is recommended. During and after treatment of vaginal carcinoma, patients should strengthen their immune system by adopting a healthy lifestyle. A healthy diet rich in vitamins is particularly important. In most cases, the pelvic floor is weakened after surgical treatment. It is therefore advisable to exercise it in a targeted manner.