Vulvar carcinoma, also known as vulvar cancer, is a rare malignant tumor disease of the external genital organs in women. The term vulvar carcinoma is derived from the Latin word "vulva" - meaning "female vulva" - and the Greek word "karkínos" - meaning "cancer". Here you will find further information and selected vulvar cancer specialists and centers.
Selected specialists and centers for the treatment of vulvar cancer
Brief overview:
- Anatomy: The female external primary sex organs are called the vulva. These include the outer and inner labia, the mons pubis, the vaginal vestibule and the clitoris.
- What is vulvar cancer? It is a rare malignant cancer of the external female genital organs.
- Localization: The labia minora are usually affected, more rarely the other areas of the vulva.
- Types: 90% are squamous cell carcinomas, which arise from the uppermost layer of the skin. There are also malignant melanomas, basal cell carcinomas, adenocarcinomas, sarcomas and carcinomas of the Bartholin glands.
- Frequency: It is the fourth most common cancer of the female reproductive organs. Around 1600 new cases are diagnosed in Germany every year.
- Causes: The causes are unknown. Risk factors include HPV infection and other sexually transmitted pathogens, nicotine, chronic illnesses and inflammation, advanced age, immunodeficiency and other illnesses.
- Symptoms: In the early stages, small white or reddish spots appear in the genital area, followed later by unpleasant itching, a feeling of soreness, weeping areas or cracks. In the advanced stage, bloody discharge and noticeable ulcers may also occur.
- Diagnosis: The suspected diagnosis is usually made during a gynecological examination. The doctor then takes a tissue sample to confirm the findings. Imaging procedures show the spread of the tumor.
- Treatment: Ideally, surgery can completely remove the tumor. Radiotherapy or chemotherapy may also be used.
- Prevention: Women should attend their annual check-ups. An HPV vaccination for girls between the ages of 12 and 17 provides additional protection.
Article overview
Vulvar cancer: definition
In principle, malignant vulvar tumors can occur in all regions of the female pubic area. In most cases, however, vulvar cancer affects the labia majora (outer labia) and, more rarely, the labia minora (inner labia). Overall, around 80 percent of all vulvar cancers occur in the labia majora and labia minora. The clitoris is only affected in around 10 percent of cases.
Depending on the cells from which a malignant tumor of the vulva originates, different forms of vulvar cancer can be distinguished.
Around 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 carcinomas in the true sense of the word.
Vulvar cancer can also develop from other cell types. For example, the remaining 10 percent of vulvar cancer cases include
- malignant melanoma (black skin cancer),
- basal cell carcinomas (white skin cancer),
- Adenocarcinomas (malignant tumors caused by cell changes in glandular tissue),
- Sarcomas (malignant tumors originating from the connective tissue of the vulva) and
- Carcinomas of the Bartholin glands.
Frequency of vulvar carcinomas
Vulvar carcinoma accounts for around 4 to 5 percent of all tumor diseases of the female genital tract. This makes it the fourth most common cancer of the female genital organs.
Overall, around two in every 100,000 women in Germany are diagnosed with vulvar cancer every year. This corresponds to around 1,600 new cases per year.
The majority of those affected are older women aged 70 and over. In recent years, however, the incidence rate has also increased among younger women.
Anatomy and function of the vulva
The term vulva covers the external, primary sexual organs of women. These include
- the labia majora (outer labia),
- the labia minora (labia minora),
- the mons pubis (mons veneris),
- the vaginal vestibule 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 skin bulges that surround the pubic cleft and join in front of the clitoris and 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 pubic cleft. The clitoris is located on the anterior fold.
The vaginal vestibule is surrounded by the labia minora and opens into the urethra, the 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 fully known. However, some risk factors can promote the development of a malignant tumor of the vulva.
These include, in particular, an infection with the so-called human papilloma virus (HPV), which is transmitted during sexual intercourse.
It is also being discussed that infections with othersexually transmitted pathogens such as
- Herpes viruses of type 2,
- chlamydia and
- Treponema pallidum, the causative agent of syphilis,
could be associated with an increased risk of vulvar cancer.
Other risk factors are
- chronic diseases or inflammation of the vulva and vagina,
- an advanced age,
- the use of immunosuppressive medication,
- a weakening of the immune system (for example after an organ transplant or in HIV patients),
- nicotine consumption and
- diseases such as leukoplakia (white callus disease) 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 symptoms.
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
- a constantly recurring, unpleasant itching,
- a feeling of soreness and
- red, non-healing, weeping spots or cracks in the genital area.
occur.
In addition, at an advanced stage there is often
- increased, possibly bloody discharge from the vagina and the formation of noticeable ulcers.
- the formation of noticeable ulcers or lumps with a cauliflower-like surface.
Various tumor stages are distinguished in vulvar carcinoma according to the so-called FIGO classification. They 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: Vulvar cancer is limited to the mucous membrane (so-called carcinoma in situ)
- FIGO Ia: Vulvar carcinoma is confined to the vulva and/or perineum and has a maximum diameter of 2 centimeters. The stromal invasion (penetration of cancer cells into the surrounding connective tissue) is a maximum of 1.0 millimeter.
- FIGO Ib: Vulvar carcinoma is confined to the vulva and/or perineum and has a maximum diameter of 2 centimeters. The stromal invasion (penetration of cancer cells into the surrounding connective tissue) is more than 1.0 millimeter.
- FIGO II: Vulvar carcinoma is confined to the vulva and/or perineum and is more than 2 centimeters in diameter.
- FIGO III: The vulvar carcinoma has spread to the lower urethra and/or the vagina or anus and/or there are lymph node metastases.
- FIGO IVa: The vulvar carcinoma has spread to the mucous membrane of the bladder and/or rectum and/or the upper ureter.
- FIGO IVb: The 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 and a gynecological examination.
During the anamnesis interview, the doctor first asks the patient about her medical history and complaints.
During the gynecological examination, the doctor will palpate the genital area. He also examines the vulva for any noticeable color and/or surface changes to the mucous membrane. For this purpose, he often uses a so-called colposcope, with which he can view the mucous membrane in multiple magnification.
The diagnosis is made as part of a gynecological examination © Mariakray | AdobeStock
In suspected cases, he takes a tissue sample (biopsy), which he then has examined histologically in the laboratory. If malignant cancer cells are detected under the microscope, the diagnosis of "vulvar carcinoma" is confirmed.
In the next step, the doctor uses various imaging techniques to determine how far the tumor has already spread. These include in particular
- Ultrasound examination of the vagina, inguinal lymph nodes and pelvic organs,
- a computer tomography of the pelvis and
- sometimes an endoscopy of the urinary tract and rectum.
Treatment of vulvar cancer
Vulvar cancer is treated by gynecologists specializing in gynecological oncology. They have various treatment options available for the treatment of vulvar cancer, including surgery, radiotherapy and chemotherapy.
Which form of therapy is used depends on the stage of the tumor.
Surgical treatment of vulvar cancer
During surgery, the vulvar carcinoma must be completely removed. In the case of vulvar carcinomas in the early stages, it is usually sufficient to generously excise the affected area of skin.
If the disease is already at an advanced stage, it may be necessary to remove the entire vulva and possibly also all or part of the vagina and/or urethra.
The question of lymph node involvement can often be clarified by means of a sentinel lymph node biopsy. Sometimes the lymph nodes in the groin must also be removed, both superficially and deeply. This may also involve the lymph nodes of the pelvis.
Radiotherapy and chemotherapy for vulvar cancer
In advanced cases, radiotherapy in combination with chemotherapy is necessary in addition to surgical treatment.
Prevention of vulvar cancer
To prevent vulvar cancer, it is advisable to have a check-up with your gynecologist at least once a year. Pre-cancerous stages of vulvar cancer can be detected and treated in good time.
An HPV vaccination has also been available for some time. It protects against infection with human papillomavirus types 16 and 18, two virus types that are considered risk factors for cervical cancer and vulvar cancer.
The vaccination is primarily recommended for girls aged 12 to 17.