Vulvar Cancer – Symptoms of This Gynecological Condition, Diagnosis, and Treatment of Vulvar Cancer and Malignant Tumors of the Vulva

Malignant vulvar tumors can occur in any region of the female genital area. In most cases, however, vulvar cancer affects the labia majora (outer labia); less commonly, it affects the labia minora (inner labia).

Overall, about 80 percent of all vulvar cancers occur on the labia majora and labia minora. The clitoris is affected in only about 10 percent of cases.

Depending on the type of cells from which a malignant vulvar tumor originates, different forms of vulvar cancer can be distinguished.

About 90 percent of all malignant vulvar tumors are so-called squamous cell carcinomas. These tumors are of epithelial origin, meaning they develop from cellular changes in the outermost layer of the skin. Doctors refer to them as vulvar carcinoma in the strict sense.

In addition, vulvar cancer can also arise from other cell types. The remaining 10 percent of vulvar cancer cases include, among others:

• malignant melanomas (black skin cancer)
• basal cell carcinomas (white skin cancer)
• adenocarcinomas (malignant tumors arising from cell changes in glandular tissue)
• sarcomas (malignant tumors originating in the connective tissue of the vulva) and
• carcinomas of the Bartholin’s glands

Vulvar carcinoma accounts for about 4 to 5 percent of all tumors of the female genital tract. This makes it the fourth most common cancer of the female reproductive organs.

Overall, about two out of every 100,000 women in Germany are diagnosed with vulvar cancer each year. This corresponds to approximately 1,600 new cases per year.

The disease primarily affects older women aged 70 and older. In recent years, however, the incidence rate has also increased among younger women.

The term “vulva” refers to the external, primary female genital organs.

These include:

• the labia majora (outer labia)
• the labia minora (inner labia)
• the mons pubis (mound of Venus)
• the vestibule of the vagina, and
• the clitoris

The labia majora, also known as the labia majora pudendi, together with the mons pubis, form the outer boundary of the vulva. They consist of two large folds of skin that enclose the vulval cleft and meet in front of the clitoris and the anus.

The labia minora, also known as the labia minora pudendi, are two thin, wrinkled folds of skin. They are located between the labia majora in the vulval cleft. The clitoris is situated at the anterior fold of the labia minora.

The vestibule of the vagina is surrounded by the labia minora and opens into the urethra, the vagina, and several glandular ducts.

The exact causes and mechanisms underlying the development of vulvar cancer are not yet fully understood. However, certain risk factors may increase the likelihood of developing a malignant tumor of the vulva.

These include, in particular, infection with human papillomaviruses (HPV), which can be transmitted during sexual intercourse.

It is also being discussed that infections with other sexually transmitted pathogens may be associated with an increased risk of vulvar cancer.

These include:

• Herpes virus type 2
• Chlamydia, and
• Treponema pallidum, the pathogen that causes syphilis

Other risk factors include:

• chronic conditions or inflammation of the vulva and vagina
• advanced age
• taking immunosuppressive medications
• a weakened immune system (for example, following an organ transplant or in HIV patients)
• nicotine use, as well as
• conditions such as leukoplakia (white patches) or lichen sclerosus in the genital area

Smoking is considered a risk factor for vulvar cancer © Nopphon | AdobeStock

In its early stages, vulvar cancer usually causes no symptoms at all or only mild ones. Early symptoms include, among others:

small white or reddish, slightly raised patches or hardened areas in the genital region that resemble warts or scars

In the advanced stages of vulvar cancer, the following symptoms typically occur, such as:

• a persistent, uncomfortable itching
• a feeling of soreness and
• red, non-healing, weeping areas or cracks in the genital area

In addition, the following often occurs in advanced stages:

• increased vaginal discharge, which may be bloody, as well as
• the formation of palpable ulcers or lumps with a cauliflower-like surface

In the case of vulvar cancer, physicians classify the different tumor stages according to the FIGO classification.

These are defined by the Fédération Internationale de Gynécologie et d'Obstétrique (French for: International Federation of Gynecology and Obstetrics).

The FIGO classification for vulvar cancer is as follows:

• FIGO 0: The vulvar carcinoma is confined to the mucosa (so-called carcinoma in situ)
• FIGO Ia: The vulvar carcinoma is confined to the vulva and/or the perineum and has a maximum diameter of 2 centimeters. Stromal invasion (the penetration of cancer cells into the surrounding connective tissue) is no more than 1.0 millimeter.
• FIGO Ib: Vulvar carcinoma is confined to the vulva and/or perineum and has a diameter of no more than 2 centimeters. Stromal invasion (the penetration of cancer cells into the surrounding connective tissue) is more than 1.0 millimeter.
• FIGO II: The vulvar carcinoma is confined to the vulva and/or the perineum and has a diameter of more than 2 centimeters.
• FIGO III: Vulvar carcinoma has spread to the lower urethra and/or the vagina or anus. Lymph node metastases may be present.
• FIGO IVa: The vulvar carcinoma has invaded the mucosa of the bladder and/or the rectum and/or the upper ureter.
• FIGO IVb: Vulvar cancer has spread to distant organs (distant metastases).

Doctors usually make a preliminary diagnosis of vulvar cancer during a medical history interview as part of a gynecological examination.

During the medical history interview, the doctor first asks the patient about her medical history and symptoms.

As part of the gynecological examination, the doctor palpates the genital area. The doctor also examines the vulva for noticeable changes in color and/or surface texture of the mucous membrane. To do this, the doctor often uses a colposcope, which allows for viewing the mucous membrane at high magnification.

The diagnosis is made during a gynecological examination © Mariakray | AdobeStock

If cancer is suspected, the doctor takes a tissue sample (biopsy), which is then sent to a laboratory for histological examination. If malignant cancer cells are detected under the microscope, the diagnosis of vulvar carcinoma is confirmed.

In the next step, the doctor uses additional imaging techniques to determine how far the tumor has already spread.

These include, in particular:

• an ultrasound examination of the vagina, the inguinal lymph nodes, and the pelvic organs,
• a CT scan of the pelvis, and
• sometimes an endoscopic examination of the urinary tract and the rectum

Vulvar cancer is treated by gynecologists specializing in gynecologic oncology. These specialists have various treatment options available for vulvar cancer, including surgery, radiation therapy, and chemotherapy. The specific treatment approach depends on the stage of the tumor.

Surgical Treatment of Vulvar Cancer

During surgery, doctors must completely remove the vulvar carcinoma. For vulvar carcinomas in the early stages, it is usually sufficient to excise the affected area of skin generously. In cases of advanced disease, it may be necessary to remove the vulva, parts of the vagina, and/or the urethra.

The question of lymph node involvement can often be clarified through a sentinel lymph node biopsy. If lymph nodes are involved, doctors may also need to remove both superficial and deep inguinal lymph nodes. In some cases, this may also involve the lymph nodes of the pelvis.

Radiation Therapy and Chemotherapy for Vulvar Cancer

In advanced cases, radiation therapy combined with chemotherapy is necessary in addition to surgical treatment.

To prevent vulvar cancer, it is recommended to have a preventive examination by a gynecologist at least once a year. During this examination, the doctor can detect and treat precancerous changes in the vulva in a timely manner.

In addition, an HPV vaccine has been available for some time. It protects against infection with human papillomavirus types 16 and 18. These two virus types are considered risk factors for cervical cancer and vulvar cancer. The vaccine is recommended for girls aged 12 to 17.

What is vulvar cancer?

Vulvar cancer is a malignant tumor of the vulva and is classified as a gynecological cancer. It usually develops from abnormal cells in the skin or mucous membranes of the external genitalia.

What are the symptoms of vulvar cancer?

Typical symptoms include persistent itching, pain, skin changes, small lumps, non-healing sores, or bleeding in the vulva area. The symptoms can be nonspecific and are therefore often detected late.

How is vulvar carcinoma diagnosed?

The diagnosis is made through a gynecological examination of the vulva. Abnormal areas are evaluated via biopsy to definitively detect malignant cellular changes.

How is vulvar cancer treated?

Treatment for vulvar cancer usually consists of surgery. Depending on the stage, lymph nodes may also be removed, and radiation therapy or chemotherapy may be used.

What is the prognosis for vulvar cancer?

The prognosis depends heavily on the stage of the disease. If vulvar cancer is detected early, the chances of a cure are good. In advanced stages, close follow-up care is particularly important.