Brief overview
- What is vulvar carcinoma? Malignant disease of the external genital organs of women. Usually arises from skin cells and only rarely from other parts of the female vulva (e.g. the clitoris).
- How common is vulvar cancer? Vulvar cancer is rare. In 2017, there were approximately 3,300 new cases in Germany, median age of onset 73 years. However, younger women are also becoming increasingly ill.
- How do you recognize vulvar carcinoma? First signs are non-specific (such as itching, pain, small skin lesions). Later, a visible tumor appears, which grows faster and faster and sometimes bleeds. Possibly also discharge with unpleasant odor.
- What is the treatment? If possible, surgical removal; complementary or alternative radiotherapy and/or chemotherapy.
- Is vulvar carcinoma curable? Vulvar carcinoma in the early stages has good chances of cure. However, these decrease very quickly if lymph nodes are affected. If other organs are affected, vulvar carcinoma is considered incurable.
Vulvar cancer: symptoms
The vulvar cancer symptoms in the early stages are very unspecific – many affected women therefore do not even think of a serious disease such as vulvar cancer. The first signs that may occur are:
- persistent itching in the vulva
- Pain, either spontaneously or, for example, during urination (dysuria) or during sexual intercourse
- Vaginal bleeding or bloody discharge
- Skin/mucous membrane lesions in the vulvar area, e.g., small, reddish, slightly raised patches or white, thickened indurations or oozing, non-bleeding small erosions
Sometimes persistent itching is also the only vulvar cancer sign in the early stages. In addition, there are many women who have no symptoms at all in this early tumor stage.
As the disease progresses, a tumor then becomes visible, for example as a palpable lump or as an ulcer with a cauliflower-like appearance. It grows slowly at first, later more and more rapidly, and may also bleed.
Other possible vulvar cancer symptoms at an advanced stage are increasing pain and an unpleasant smelling discharge. The latter is caused by dying tumor cells that are decomposed by bacteria.
Where does vulvar carcinoma develop?
In principle, a malignant tumor can develop anywhere in the vulva. For some years, however, most vulvar carcinomas have been localized in the anterior vulvar region, i.e. in the area of the labia minora, between the clitoris and urethra, or directly on the clitoris. In the remaining cases, the tumor arises in the posterior vulvar region, such as to the side of the labia majora, at the posterior vaginal entrance, or at the perineum (perineum = area between the external genital organs and the anus).
Vulvar cancer: chances of cure
Several factors influence the prognosis for vulvar cancer. The main factors are the size of the tumor, how deeply it has penetrated the underlying tissue, and the extent to which it has already spread.
Vulvar carcinoma & survival rate: What the statistics say
In the case of vulvar carcinoma, the relative 5-year survival rate is 71 percent, which means that in 71 percent of affected women, the malignant tumor has not led to death even five years after diagnosis (sources: Center for Cancer Registry Data and vulvar carcinoma guideline).
This figure refers to disease across all stages. Looking at the prognosis at different tumor stages, the same applies as for other cancers: The earlier treatment is given, the more likely vulvar cancer is curable.
In fact, in most cases (about 60 percent) vulvar cancer is detected at an early stage (stage I). The vast majority of affected women can then be cured. However, as soon as the cancer has spread to lymph nodes in the groin and possibly also in the pelvis, the prognosis deteriorates very quickly. If other organs (such as lungs, liver, bones, brain) are already affected by the cancer, vulvar cancer is considered incurable.
Prognosis may vary in individual cases
Vulvar cancer: causes & risk factors
Vulvar cancer occurs when cells in the pubic area degenerate and begin to multiply uncontrollably. Depending on which cells these are, a distinction is made between different types of vulvar cancer:
In about nine out of ten cases, cells of the uppermost layer of skin or mucous membrane (squamous epithelium) in the vulva degenerate – then the vulvar cancer is a so-called squamous cell carcinoma, i.e. a form of white skin cancer. The tumor usually forms a horny layer on the surface (keratinizing squamous cell carcinoma), but it can also remain unkeratinized (non-keratinizing squamous cell carcinoma).
The most common form of vulvar cancer – keratinizing squamous cell carcinoma – usually develops independently of infection with human papillomavirus (HPV; see Risk Factors) and preferentially in older women. Second most common are nonkeratinizing squamous cell carcinomas, which are more HPV-dependent and mostly affect younger women (mean age: 55 years).
Causes unclear
Whether squamous epithelium, basal cell layer or Bartholin’s glands – until now it has not been known exactly why cells in the vulva area suddenly degenerate in some women and lead to vulvar cancer. However, as with other cancers, it is very likely that an interaction of several factors is necessary for tumor development.
Risk factors for vulvar cancer
These risk factors include the so-called vulvar intraepithelial neoplasia (VIN). These are cell changes in the uppermost cell layer (epithelium) of the vulva. They can become precancerous. Doctors distinguish between three VIN stages:
- VIN I: Mild tissue changes limited to the lower third of the vulvar epithelium.
- VIN II: Moderate tissue changes affecting the lower two-thirds of the vulvar epithelium.
- VIN III: Severe tissue changes affecting the entire vulvar epithelium.
Stage VIN I is not considered a precancerous condition, but regresses in most cases. VIN II and VIN III, on the other hand, can progress to vulvar cancer over the course of several years.
A special form of VIN is Paget’s disease of the vulva, a malignant tissue change that originates from skin appendages. It is also considered a precursor of vulvar cancer.
The same applies to other cancers (or precancerous lesions) in the genital or anal area, the development of which may also be linked to human papillomaviruses. These include vaginal cancer, cervical cancer and anal carcinoma.
The fact that a persistent immune deficiency can also promote vulvar carcinoma is usually also related to HPV: If the immune system is permanently weakened, for example, by an HIV infection or by taking immunosuppressive drugs (after organ transplantation or in the case of autoimmune diseases), a chronic HPV infection can develop more easily, which in turn then favors the development of vulvar carcinoma.
In addition to HPV, however, several other sexually transmitted pathogens can also contribute to the development of vulvar cancer – herpes viruses (genital herpes), chlamydia and the pathogens that cause syphilis.
Also independent of HPV infection, autoimmune processes such as those associated with the chronic inflammatory skin disease lichen sclerosus can increase the risk of vulvar cancer – more specifically, the most common form of vulvar cancer, keratinizing squamous cell carcinoma of the vulva, which occurs primarily in older women.
Incorrect genital hygiene is also considered unfavorable: lack of hygiene in the genital area can be just as harmful as the frequent use of vaginal lotions or intimate sprays.
Vulvar cancer: examinations & diagnosis
The right person to contact if you suspect vulvar cancer is your gynecologist. He can determine whether a woman actually has a malignant vulvar tumor by means of various examinations:
Inspection and palpation.
As part of a comprehensive gynecological examination, the doctor will first closely inspect the vulva, the vagina and the cervix – pathological tissue changes often occur in several places at once. During the inspection, the doctor looks at the coloration of the skin and any abnormalities in the tissue, such as spots, cracks, thickening, scaling or ulcers.
In addition, the type feels the entire genital area. He pays attention to any lumps or thickenings in the tissue. The lymph nodes in the groin area are also included in the palpation examination. If they are enlarged and/or painful, this may indicate an infestation with cancer cells, but may also have many other reasons.
Colposcopy
The doctor can examine conspicuous tissue areas more closely by means of colposcopy. For this purpose, he uses a special magnifying glass with 10 to 20 times magnification (colposcope).
Note: Unlike VIN lesions, Paget’s disease of the vulva does not show white staining on acetic acid testing!
Biopsy
The physician takes one or more tissue samples (biospie) from each unclear tissue change – either as a punch biopsy or as an excision biopsy:
In a punch biopsy, a cylinder of tissue is punched out of the suspicious area using a special instrument. (e.g., a punch). In excisional biopsy, the entire suspicious area is excised immediately (e.g., in pigmented lesions that may be black skin cancer).
Fine tissue (histological) examination of the samples in the laboratory can definitively clarify whether it is cancer or a precancerous lesion.
Tissue removal is usually done under local anesthesia. The doctor can close the resulting wound with a suture.
Further examinations in the case of confirmed vulvar carcinoma
Once the diagnosis of vulvar cancer has been established, the doctor will order various further examinations depending on the individual case. These may include the following examinations:
A comprehensive gynecological examination of the entire genital and anal region helps to determine the tumor size and location more precisely.
The urinary tract can also be examined endoscopically (urethrocystoscopy) if an infestation with cancer cells is suspected.
Ultrasound examinations of the vagina, groin region, pelvic organs and liver can also provide information about tumor spread.
If lung metastases are suspected, X-rays of the chest can be taken. Even more detailed images of the inside of the body and thus more precise evidence of metastases are provided by computed tomography (CT) and magnetic resonance imaging (MRI).
Classification into disease stages
Based on all examination results, vulvar carcinoma can be assigned to a specific disease stage. This is important for therapy planning.
The stages of vulvar cancer according to the so-called FIGO classification (FIGO = Fédération Internationale de Gynécologie et d’Obstétrique) are:
- Stage I: Vulvar cancer confined to the vulva or vulva and perineum (perineum = area between external genitalia and anus). No involvement of lymph nodes. Depending on the maximum extent of the tumor and depth of penetration into the tissue, a distinction is made between stage Ia and stage IB.
- Stage II: Tumor of any size that has spread to the lower third of the vagina and/or urethra and/or to the anus. No involvement of lymph nodes.
- Stage IV: Tumor of any size that has spread to the upper two-thirds of the vagina and/or urethra and/or to the anus and/or to the mucosa of the urinary bladder or rectum, or that is fixed to the pelvic bone (stage IVA) or that has formed distant metastases (stage IVB).
Vulvar cancer: treatment
How medical professionals treat vulvar cancer depends largely on the type, stage and location of the tumor. They also take into account the patient’s general state of health and her age (relevant with regard to family planning or preservation of sexual function).
In principle, the options available for the treatment of vulvar cancer are surgery, radiotherapy and chemotherapy. They can be used individually or in different combinations – individually adapted to the patient.
Surgery
Surgery is the treatment of choice for vulvar cancer. If possible, the tumor is always excised completely and the vulva is preserved as much as possible. Only in exceptional cases is surgery not performed, for example if a woman cannot be operated on for health reasons or if the tumor has already spread to the anus.
The extent of the operation depends on the stage of the disease:
Larger tumor or multiple tumor sites: Tumors that are already larger, have already spread to neighboring structures (such as the urethra, clitoris, vagina), or occur in multiple locations require more extensive surgery. Then not only the cancerous tissue itself with a fringe of healthy tissue is removed, but also part or all of the vulva (together with the underlying fatty tissue). Vulva removal is called vulvectomy.
During this procedure, the lymph nodes in the groin are always removed as well, because there is a high risk that they are also affected by the cancer. If tissue tests confirm this, the pelvic lymph nodes must also be excised.
Risks of the operation
Especially in the case of small tumors near the clitoris or urethra, surgery is usually performed with the smallest possible margins from the healthy tissue in order to spare the clitoris and urethra. However, if too little healthy tissue is cut out at the edge, the tumor can return.
In the case of complete removal of the vulva, about one in two patients has to deal with wound healing problems afterwards. Other possible consequences of radical surgery include sensory disturbances, scarring, constriction, urinary leakage and recurrent urinary tract infections.
Radiotherapy
If lymph nodes in the groin or pelvis are affected by the cancer, these areas are irradiated. Vulvar cancers themselves generally do not respond very well to radiation therapy. Nonetheless, this treatment method can be helpful in the following cases:
- Adjuvant to surgery: adjuvant radiotherapy is given after surgery, such as when the tumor could not be removed completely or with a large enough margin. Neoadjuvant radiotherapy precedes surgery – it is intended to shrink a tumor that is inoperable because of its size or location (e.g., close to the rectum) to the point that surgical removal is then possible after all.
- Instead of surgery: there are also vulvar carcinomas that are not operable at all and are only irradiated (definitive irradiation).
To make radiation therapy more effective, it can be combined with chemotherapy. Doctors refer to this as radiochemotherapy.
Chemotherapy
Supportive therapy
This includes therapy measures that are intended to prevent or reduce therapy- or tumor-related symptoms. Some examples:
Antiemetic drugs are given to counteract nausea and vomiting – possible side effects of radiation and chemotherapy. Diarrhea resulting from radiation or chemotherapy can also be treated with medication.
Radiation therapy in the urogenital area can trigger acute cystitis. In such cases, antispasmodic and pain-relieving drugs and, if necessary, antibiotics can help.
Cancer patients often suffer from anemia – caused either by the tumor itself or by the tumor therapy. For treatment, the doctor may administer blood transfusions, for example.
In the case of terminal vulvar cancer, a cure is no longer possible. In this case, therapeutic measures such as surgery, (radio)chemotherapy or the administration of pain medication are aimed at alleviating the patient’s symptoms in order to improve her quality of life.
Vulvar carcinoma: prevention
Vaccination is recommended for all girls and boys between the ages of nine and 14, preferably before the first sexual intercourse, because one is infected very quickly during sex. Missed vaccinations should be made up by the age of 18 at the latest. In individual cases, the HPV vaccination can also be useful at a later point in time – interested parties are best advised to discuss this with their doctor (e.g. gynecologist).
The HPV vaccination offers protection against infection with high-risk HPV types – i.e. virus types that are associated with an increased risk of cancer. This primarily concerns cervical cancer, but also, for example, penile cancer, vaginal cancer, anal cancer and, indeed, vulvar cancer.
It is also important to detect and treat (possible) precancerous lesions at an early stage, especially vulvar intraepithelial neoplasia (VIN): These tissue changes in the pubic area have increased in recent decades, especially in women between the ages of 30 and 40. Critical here are the stages VIN II and VIN III: they develop further into vulvar carcinoma in 15 to 22 percent of cases over an average period of three to four years.
