Vulvar cancer: Information and specialists for vulvar cancer


Vulvar carcinoma, also known as vulvar cancer, is a rare malignant tumor disease of the female external genital organs. The term vulvar carcinoma is derived from the Latin word "vulva", meaning "female pubic area", and the Greek word "karkínos", meaning "cancer". Here you can find additional information and selected specialists and centers for the treatment of vulvar cancer.

ICD codes for this diseases: C51

Selected specialists and centers for the treatment of vulvar cancer

Brief overview:

  • Anatomy: The external primary female sex organs are called the vulva. These include the labia majora, labia minora, mons pubis, vulval vestibule, and clitoris.
  • What is vulvar cancer? It is a rare malignant cancer of the external female reproductive organs.
  • Localization: The labia majora are most often affected; other areas of the vulva are less commonly affected.
  • Types: Squamous cell carcinomas, which arise from the uppermost layer of the skin, account for 90% of cases. Furthermore, there are malignant melanoma, basal cell carcinoma, adenocarcinoma, sarcoma, and Bartholin gland carcinoma.
  • Frequency: It is the fourth most common cancer of the female reproductive organs. Every year, about 1,600 new cases are diagnosed in Germany.
  • Causes: The causes are unknown. Risk factors include HPV infection as well as other sexually transmitted pathogens, nicotine, chronic disease and inflammation, older age, immunodeficiency, and other conditions.
  • Symptoms: In the early stages, small white or reddish spots appear in the genital area, later an unpleasant itching, feeling of soreness, weeping spots, or cracks emerge. In advanced stages, bloody discharge and palpable ulcers may also appear.
  • Diagnosis: Most often, the suspected diagnosis is made during a gynecological examination. The doctor then takes a tissue sample, which can confirm the findings. Imaging techniques show the spread of the tumor.
  • Treatment: Ideally, a tumor can be completely removed by surgery. If necessary, additional radiotherapy or chemotherapy may also be employed.
  • Prevention: Women should take advantage of annual screenings. An HPV vaccination for girls between 12 and 17 years of age provides additional protection.

Article overview

Vulvar cancer: Definition

Malignant vulvar tumors can generally occur in all regions of the female pubic area. In most cases, however, vulvar cancer affects the labia majora (outer labia) and, less commonly, the labia minora (inner labia). Overall, about 80 percent of all vulvar cancers occur in the labia majora and minora. The clitoris is affected in only about 10 percent of cases.

Different types of vulvar cancer can be differentiated depending on the cellular origin of the malignant tumor.

About 90 percent of all malignant vulvar tumors are so-called squamous cell carcinomas. These tumors are of epithelial origin, i.e., they develop from cell changes in the uppermost layer of the skin. They are considered vulvar cancer in the true sense.

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

  • Melanoma (malignant melanoma),
  • Basal cell carcinoma (basal cell skin cancer),
  • Adenocarcinoma (malignant tumors formed by cell changes of glandular tissue),
  • Sarcoma (malignant tumors originating from the connective tissue of the vulva), and
  • Bartholin gland carcinoma.

Incidence of vulvar cancer

Vulvar cancer accounts for about 4 to 5 percent of all tumors of the female genital tract. It is thus the fourth most common cancer of the female reproductive organs.

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

The majority of those affected are older women aged 70 and over. In recent years, however, the rate of the disease has also increased among younger women.

Anatomy and function of the vulva

The term vulva refers to the external, primary sexual organs of women. These include

  • The labia majora (outer labia),
  • The labia minora (inner labia),
  • The mons pubis (mons veneris),
  • The vulval vestibule, and
  • The clitoris.

The outer labia, also known as the labia majora, forms the outer boundary of the vulva together with the mons pubis. They consist of two large skin bulges that surround the pudendal cleft and join in front of the clitoris and anus.

The inner labia, also known as the labia minora, are two thin, puckered folds of skin. They are located between the outer labia in the pudendal cleft. The clitoris is located at its anterior fold.

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

Causes and risk factors for vulvar cancer

The exact causes and correlations for the development of vulvar cancer are not yet completely known. However, some risk factors may increase the likelihood of developing a malignant tumor of the vulva.

In particular, this includes a so-called human papillomavirus (HPV) infection. These viruses are transmitted during sexual intercourse.

There is also a discussion regarding whether infections with other sexually transmitted pathogens such as

  • Type 2 herpes viruses,
  • Chlamydia, and
  • Treponema pallidum, the syphilis pathogen,

may be associated with an increased risk of vulvar cancer.

Other risk factors are

  • Chronic diseases or inflammation of the vulva and vagina,
  • An older age,
  • Taking immunosuppressant medications,
  • A weakening of the immune system (for example, after an organ transplant or in HIV patients),
  • Nicotine consumption, and
  • Diseases such as leukoplakia (idiopathic white patch) or lichen sclerosus in the genital area.

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

Symptoms and stages of vulvar cancer

In the early stages, vulvar cancer usually causes little or no discomfort.

Early symptoms include small white or reddish, slightly raised spots or indurations in the genital area that resemble warts or scars.

In the advanced stages of vulvar cancer, symptoms typically include

  • Constantly recurring, unpleasant itching,
  • A feeling of soreness, and
  • Red, non-healing, weeping spots, or cracks in the genital area.

In addition, the following symptoms often appear in the advanced stage:

  • An increased, possibly also bloody discharge from the vagina, and
  • The formation of palpable ulcers or nodules with a cauliflower-like surface.

In vulvar cancer, the different tumor stages are divided according to what is known as the FIGO classification system. The different stages are defined by the Fédération Internationale de Gynécologie et d’Obstétrique (French for: International Federation of Gynecology and Obstetrics).

The FIGO classification subdivides the stages of vulvar cancer as follows

  • FIGO 0: Vulvar cancer is confined to the mucosa (known as carcinoma in situ)
  • FIGO Ia: Vulvar cancer is confined to the vulva and/or perineum and has a maximum diameter of 2 centimeters. Stromal invasion (spread of cancer cells into surrounding connective tissue) is 1.0 millimeter or less.
  • FIGO Ib: Vulvar cancer is confined to the vulva and/or perineum and has a maximum diameter of 2 centimeters. Stromal invasion (spread of cancer cells into surrounding connective tissue) is greater than 1.0 millimeter.
  • FIGO II: Vulvar cancer is confined to the vulva and/or perineum and is greater than 2 centimeters in diameter.
  • FIGO III: Vulvar cancer has spread to the lower urethra and/or vagina or anus and/or there is lymph node metastasis.
  • FIGO IVa: Vulvar cancer has spread to the mucosa of the bladder and/or rectum and/or upper ureter.
  • FIGO IVb: Vulvar carcinoma has spread to distant organs (distant metastases).

Diagnosis of vulvar carcinoma (vulvar cancer)

The suspected diagnosis of vulvar cancer is usually made during a medical history interview (anamnesis) and a gynecological examination.

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

During the gynecological examination, the doctor palpates the genital area. They also examine the vulva for any noticeable color changes and/or surface changes in the mucosa. For this purpose, the physician often uses a so-called colposcope, with which they can view the mucosa at multiple magnifications.

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

If the gynecologist suspects vulvar carcinoma, they take a tissue sample (biopsy), which is then histologically examined in the laboratory. If malignant cancer cells are detected under the microscope, the diagnosis of "vulvar cancer" is confirmed.

As a next step, the physician uses various imaging techniques to determine how far the tumor has already spread. In particular, this includes

  • An ultrasound examination of the vagina, inguinal lymph nodes, and pelvic organs,
  • A CT scan of the pelvis, and
  • Sometimes an endoscopy of the urinary tract and rectum.

Treating vulvar cancer

The treatment of vulvar cancer is carried out by gynecologists specializing in gynecological oncology. Multiple options for treating vulvar cancer are available to these specialists, including surgery, radiation, and chemotherapy.

The choice of treatment depends on the stage of the tumor.

Surgical treatment of vulvar cancer

During surgery, the vulvar cancer must be completely removed. In the case of vulvar cancer in the early stages, it is usually sufficient to generously excise the affected area of the skin.

If the disease is already advanced, it may be necessary to remove the entire vulva and, if required, to additionally excise all or parts of the vagina and/or urethra.

The question of whether the cancer has metastasized to the lymph nodes can often be resolved by a sentinel lymph node biopsy. Sometimes, in the case of metastasis, the lymph nodes of the groin must also be removed on the surface and deep in the tissue. This may also affect the lymph nodes of the lesser pelvis.

Radiation and chemotherapy for vulvar cancer

In advanced cases, radiotherapy in combination with chemotherapy is necessary in addition to surgical treatment.

Preventing vulvar cancer

To prevent vulvar cancer, it is advisable to have a gynecological examination at least once a year. During this check-up, preliminary stages of vulvar cancer can be detected and treated in time.

In addition, the HPV vaccination has been available for some time. It protects against getting infected with human papillomavirus types 16 and 18. These two types of virus are considered risk factors for cervical cancer and vulvar cancer.

Vaccination is recommended especially for girls between the ages of 12 and 17.

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