Vulvar cancer is a rare type of gynecological cancer that develops as a result of the uncontrolled proliferation of cells in the vulva, which forms the female external genitalia. The vulva encompasses the outer and inner genital lips (labia majora and labia minora), the clitoris, the vaginal entrance, and the tissues surrounding this region. Although vulvar cancer can occur in any of these structures, it is most commonly seen on the outer genital lips.
Vulvar cancer usually develops slowly. In some cases, precancerous changes may occur in the cells on the vulvar surface before cancer develops. While some of these changes are associated with human papillomavirus (HPV) infection, some vulvar cancers, particularly those seen at an advanced age, may be linked to chronic vulvar skin diseases.
Prolonged itching in the vulva, a non-healing sore, a change in skin color or structure, a palpable mass, a wart-like growth, bleeding, or pain may be among the symptoms of vulvar cancer. However, the presence of these findings does not necessarily mean that he has vulvar cancer. Similar complaints can also occur in infections, dermatological diseases, and benign lesions.
It is important that vulvar changes that persist for several weeks or become progressively more pronounced are evaluated by a gynecology specialist.
What Causes Vulvar Cancer?
Vulvar cancer occurs as a result of cells multiplying in an uncontrolled manner due to changes occurring in the DNA of the cells in the vulvar tissue. However, the reason why cellular changes develop in some individuals cannot always be determined with certainty.
The main factors that may increase the risk of developing vulvar cancer include high-risk HPV infections, vulvar intraepithelial neoplasia (VIN), advanced age, smoking, suppression of the immune system, and certain chronic vulvar skin diseases.
HPV Infection and Vulvar Cancer
Human papillomavirus (HPV) is a common group of viruses that can cause infection in the genital area. There are numerous types of HPV. While some of these are low-risk and associated with genital warts, certain high-risk HPV types can lead to precancerous changes in cells.
The persistence of a high-risk HPV infection for a long time can cause changes in the structure of cells in the vulva and, over time, contribute to the development of vulvar cancer in some individuals.
However, it should be kept in mind that HPV infection is quite common and the vast majority of people with HPV do not develop vulvar cancer. A significant portion of HPV infections are brought under control by the immune system over time.
Vulvar Intraepithelial Neoplasia (VIN)
Vulvar intraepithelial neoplasia (VIN) is the presence of abnormal cellular changes in the surface cells covering the vulva. VIN does not directly mean vulvar cancer, but some types of VIN can transform into invasive vulvar cancer over time.
VIN may be associated particularly with high-risk HPV infections or may develop independently of HPV.
Symptoms such as persistent itching, burning, a change in skin color, and raised or thickened areas in the vulva can be seen in VIN. However, some individuals may not have any symptoms.
Evaluating suspicious areas in the early period and performing a biopsy when necessary are important in terms of detecting precancerous changes.
Age and Other Risk Factors
Although vulvar cancer can be seen at any age, it is more frequently observed especially in the advanced age group.
Other factors associated with increased risk are:
- High-risk HPV infections
- History of vulvar intraepithelial neoplasia
- Smoking
- Weakening of the immune system
- HIV infection
- Use of certain immunosuppressive treatments
- Chronic vulvar skin diseases such lichen sclerosus
- Having a prior history of HPV-related cervical, vaginal, or anal lesions
Having one or more of the risk factors does not mean that he will definitely develop vulvar cancer.
What Are the Types of Vulvar Cancer?
Vulvar cancer is divided into different groups based on the cell type in which the cancer develops. The type, size, location in the vulva, and spread status of the cancer are important in determining the treatment approach.
Squamous Cell Vulvar Cancer
Squamous cell carcinoma is the most common type of vulvar cancer. It develops from the flat epithelial cells covering the surface of the vulva.
While some squamous cell vulvar cancers are associated with HPV infection, some may arise independently of HPV, particularly at advanced ages.
Vulvar Melanoma
Vulvar melanoma is a rare type of vulvar cancer that develops from pigment cells called melanocytes, which give skin its color.
Newly developing dark-colored lesions in the vulva or changes in the shape, color, or size of a pre-existing mole should be evaluated.
Adenocarcinoma
Adenocarcinoma is a rarer type of cancer developing from the glandular tissues in the vulva. It can originate from glands located in the vulvar region, such as Bartholin glands.
A new mass appearing particularly in the Bartholin gland region at advanced ages must be evaluated carefully.
Basal Cell Carcinoma
Basal cell carcinoma is rarely seen in the vulva. It generally develops from basal cells on the skin surface and in many cases may follow a slower course compared to other types of vulvar cancer.
Vulvar Sarcoma
Vulvar sarcoma is one of the rare tumors developing from connective tissue, muscle, or other soft tissues in the vulva.
Since there are different sarcoma subtypes, the treatment approach is planned based on the result of the pathological evaluation.
Paget's Disease
Paget's disease of the vulva is a rare disease originating from glandular cells in the vulvar skin.
Since it may present as a red, scaly, itchy, or eczema-like area, it can initially be confused with dermatological diseases. A biopsy may be required for vulvar skin changes that do not heal for a long time.
What Are the Symptoms of Vulvar Cancer?
Vulvar cancer may not cause prominent complaints in the early stages. When symptoms do appear, various changes can be seen in the appearance, skin structure, and sensation of the vulva.
Itching and Irritation in the Vulva
Prolonged vulvar itching is one of the potential symptoms of vulvar cancer.
Since itching can have numerous different causes such as infection, allergic reaction, and dermatological diseases, a cancer diagnosis cannot be made by looking at itching alone. However, itching that does not resolve despite treatment or that recurs needs to be evaluated.
Non-Healing Sores or Lesions
Sores, ulcers, or crusted lesions that do not heal for a long time can be seen in the vulva.
It is important that vulvar lesions that do not heal within a few weeks, grow, or recur are evaluated with a gynecological examination.
Swelling, Masses, or Wart-Like Growths in the Vulva
Vulvar cancer may be noticed in some individuals in the form of a palpable hardness, swelling, mass, or a wart-like growth.
Every vulvar mass is not cancer. Cysts, infections, and benign skin lesions can also cause a similar appearance.
Color and Skin Changes in the Vulva
The formation of areas lighter, darker, redder, or whiter than normal on the vulvar skin can be observed.
In addition to this:
- Thickening of the skin,
- Scaling,
- Rough appearance,
- Surface changes,
- Changes in the shape or color of moles
symptoms such as these may occur.
Bleeding and Discharge
Genital bleeding or bloody discharge not related to the menstrual period are among the symptoms that can be seen in vulvar cancer.
Any genital bleeding occurring after menopause requires medical evaluation regardless of its cause.
Pain and Pain During Sexual Intercourse
Tenderness, burning, or pain may occur in the vulvar region. Pain during sexual intercourse may develop in some individuals.
Burning or tenderness in the vulvar region while urinating can also be observed.
Is Vulvar Cancer Visible?
Since the vulva is located on the outside of the body, it is possible for certain changes related to vulvar cancer to be seen with the naked eye or noticed by touch.
Vulvar cancer may appear as;
- A non-healing sore,
- A bump or mass,
- A wart-like growth,
- A red or white patch,
- Darkening of the skin,
- A thickened area,
- An ulcer,
- A bleeding lesion
can be seen.
However, vulvar cancer does not always present as a distinct mass. Changes in the early period can be quite small or ambiguous.
Furthermore, a diagnosis of vulvar cancer cannot be made solely by looking at its appearance. Pathological examination of a biopsy taken from the suspicious lesion forms the basis of the definitive diagnosis.
Who is at Risk for Vulvar Cancer?
Although vulvar cancer is rare, the risk may be higher in certain individuals compared to others.
Advanced Age
The incidence of vulvar cancer increases with age. Although it is more frequently seen especially in women in the advanced age group, the disease can also develop in younger individuals.
History of HPV Infection
Infection persisting for a long time with high-risk HPV types is an important risk factor, particularly regarding HPV-associated vulvar cellular changes.
Smoking
Smoking is associated with an increased risk, especially for HPV-associated vulvar cancers.
It is thought that smoking may reduce the capacity of the immune system to clear HPV infection.
Weakening of the Immune System
The weakening of the immune system can increase the likelihood of HPV infections becoming persistent.
The risk may increase in individuals who have an HIV infection or who receive treatments that suppress the immune system for various reasons.
Chronic Vulvar Skin Diseases
Certain chronic vulvar skin diseases, primarily lichen sclerosus, may increase the risk of developing vulvar cancer.
It is important that such diseases are treated appropriately and followed up regularly.
What is the Relationship Between HPV and Vulvar Cancer?
There is a significant relationship between HPV and vulvar cancer. Particularly high-risk HPV types can cause changes in the structure of cells in the vulva over time that can turn into cancer.
The process associated with HPV generally can progress as follows:
HPV infection → infection becoming persistent → cellular changes → development of VIN in some individuals → invasive vulvar cancer in certain cases when left untreated.
However, this process does not apply to everyone with an HPV infection.
HPV is an extremely common infection, and the vast majority of infections are controlled by the immune system before turning into cancer. Therefore, HPV positivity does not directly mean a cancer diagnosis.
How is Vulvar Cancer Diagnosed?
In the diagnosis of vulvar cancer, the individual's complaints, a gynecological examination, and the pathological evaluation of the suspicious tissue are important.
Gynecological Examination
The physician evaluates the vulva, vagina, and, when necessary, surrounding tissues in detail.
During the examination, the lesion's:
- Size,
- Shape,
- Color,
- Location,
- Relationship with surrounding tissues,
- Presence or absence of ulceration or bleeding
can be examined.
Lymph nodes in the groin region can also be evaluated.
Colposcopic Evaluation
A colposcope is an optical device that helps evaluate genital tissues in detail by magnifying them.
It can be used to examine suspicious vulvar areas more closely and to determine from which region the biopsy should be taken.
Vulvar Biopsy
For a definitive diagnosis in case of suspected vulvar cancer, a sample must be taken from the suspicious tissue and examined in a pathology laboratory.
This procedure is called a vulvar biopsy.
As a result of the pathological examination:
- Whether cancer is present or not,
- The cell type of the cancer,
- Invasion characteristics,
- Certain biological features of the tumor
can be evaluated.
Imaging Methods
After a cancer diagnosis is made, imaging methods can be utilized depending on the patient's condition to evaluate the spread of the disease.
Among these:
- Magnetic resonance imaging (MRI),
- Computed tomography (CT),
- PET/CT
can be found.
It is not necessary to apply all imaging methods in every vulvar cancer patient. Which tests will be used are determined based on the characteristics of the disease.
What Are the Stages of Vulvar Cancer?
Staging in vulvar cancer is performed to evaluate how far the disease has spread in the vulva, whether neighboring tissues are affected, whether there is spread to lymph nodes, and whether there are distant organ metastases.
Vulvar cancer is generally classified from Stage 1 to Stage 4. Since there are also substages, an individual's exact stage is determined by evaluating pathology and imaging results together.
Stage 1 Vulvar Cancer
In Stage 1, the cancer is limited to the vulva and/or perineal region, and no cancer has been detected in regional lymph nodes.
Surgical treatment frequently forms the basis of treatment in vulvar cancers detected at an early stage.
Stage 2 Vulvar Cancer
In Stage 2, the tumor may have extended beyond the vulva and spread to nearby perineal structures such as the lower part of the urethra, the lower part of the vagina, or the anus, but there is no regional lymph node metastasis.
Stage 3 Vulvar Cancer
In Stage 3 vulvar cancer, there may be spread to regional groin lymph nodes.
Depending on the characteristics of the disease, surgery, radiotherapy, and chemotherapy can be applied in different combinations.
Stage 4 Vulvar Cancer
In Stage 4 disease, the cancer may have spread to more advanced neighboring structures or distant organs.
Stage 4 is also divided into subgroups within itself. Treatment is planned multidisciplinaryly according to the spread of the tumor, the individual's general condition, and previously administered treatments.
How is Vulvar Cancer Treated?
Vulvar cancer treatment is not the same for every patient.
When planning treatment:
- The type of cancer,
- Its stage,
- The size of the tumor,
- Its location in the vulva,
- The status of the lymph nodes,
- The patient's age,
- General health status,
- Previously received treatments
are taken into consideration.
Surgery, radiotherapy, and chemotherapy can be applied alone or together in treatment. In the early stage, surgical treatment is the primary approach in most cases. In advanced stages, radiotherapy and chemotherapy can be added to the treatment plan.
Vulvar Cancer Surgery
The goal of surgery is to remove the tumor with an adequate surgical margin and to preserve as much healthy vulvar tissue as possible.
While larger surgeries were frequently applied in the past, today more limited and function-preserving surgeries can be preferred in appropriate patients whenever possible.
Radiotherapy
Radiotherapy is a treatment method that aims to destroy cancer cells or prevent them from multiplying by using high-energy rays.
Radiotherapy in vulvar cancer:
- After surgery,
- In patients with lymph node involvement,
- In advanced-stage diseases,
- To shrink the tumor before surgery,
- In certain patients where surgery cannot be performed
can be used.
Chemotherapy
Chemotherapy is a drug treatment aimed at preventing cancer cells from growing and multiplying.
In vulvar cancer, chemotherapy can be applied together with radiotherapy, especially in advanced disease or to increase the effectiveness of radiotherapy.
Since advanced-stage vulvar cancer is rarely seen, the systemic treatment to be applied is individualized according to the patient's condition.
Follow-up After Treatment
The completion of vulvar cancer treatment does not eliminate the need for follow-up.
During check-ups:
- The vulvar region,
- The surgical area,
- Groin lymph nodes,
- Newly developing complaints
can be evaluated.
Imaging or additional tests can be performed when necessary.
The frequency of the follow-up program is determined according to the stage of the disease, the applied treatment, and the individual's risk characteristics. Since recurrence can be seen in vulvar cancer, regular follow-up is important.
How is Vulvar Cancer Surgery Performed?
The scope of vulvar cancer surgery varies depending on the size of the tumor, its location, whether there is more than one focus, and the risk of spreading to lymph nodes.
The goal is not only to remove the cancer but also to preserve the anatomical and functional structure of the vulva as much as possible.
What is Vulvectomy?
Vulvectomy is the surgical removal of a part or a larger portion of the vulva.
The scope of surgery can vary from patient to patient.
While local surgical procedures where the tumor is removed together with healthy tissue around the tumor may be sufficient in small and early-stage tumors, more comprehensive surgeries may be required for larger tumors.
It is not necessary to remove the entire vulva in every vulvar cancer patient.
Evaluation of Lymph Nodes
One of the first regions to which vulvar cancer can spread is the groin lymph nodes. Therefore, evaluating lymph nodes in certain patients constitutes an important part of treatment.
Sentinel lymph node biopsy can be performed in appropriate patients.
The sentinel lymph node is the first lymph node to which cancer cells are expected to reach if they detach from the tumor. This method can help reduce unnecessary extensive lymph node surgery in the appropriate patient group.
In some patients, however, a more extensive removal of groin lymph nodes may be necessary.
Post-Operative Period Following Vulvar Cancer Surgery
The recovery process after surgery varies depending on the extent of the operation.
After surgery:
- Wound care,
- Pain control,
- Monitoring for signs of infection,
- Tracking of urinary and bowel functions,
- Increasing mobility,
- Evaluation of lymphedema symptoms
are important.
Wound healing may take longer in some patients who undergo extensive surgery.
Physical and psychological effects related to sexual life can also be addressed in the post-treatment period.
What Are the Side Effects of Vulvar Cancer Treatment?
The type and severity of side effects related to treatment can vary depending on the method used, the treatment area, and the individual's general health status.
Side Effects of Surgical Treatment
Problems that may be seen after surgery include:
- Pain,
- Delayed wound healing,
- Infection,
- Bleeding,
- Sensation changes in the skin,
- Lymphedema,
- Changes in sexual functions,
- Psychological effects related to body image
can be found.
There may be a risk of developing lymphedema in the legs or genital region in patients whose groin lymph nodes have been removed.
Side Effects of Radiotherapy
Potential side effects of radiotherapy include:
- Redness and tenderness in the skin,
- Fatigue,
- Irritation in the vulvar region,
- Discomfort while urinating,
- Changes in bowel habits,
- Tenderness in vaginal tissues
can be found.
The majority of side effects may decrease over time after the completion of treatment, but some effects may last longer.
Side Effects of Chemotherapy
The side effects of chemotherapy can vary depending on the drug used.
Effects such as nausea, vomiting, loss of appetite, fatigue, susceptibility to infections, and a decrease in blood cells can be observed.
It is important that side effects occurring during treatment are shared with the healthcare team.
Does Vulvar Cancer Recur?
Vulvar cancer can recur after treatment. This is called a vulvar cancer recurrence.
Recurrence:
- In the vulvar region,
- In nearby tissues,
- In lymph nodes,
- More rarely in distant organs
can occur.
The risk of recurrence depends on many factors such as the stage of the initial cancer, lymph node involvement, tumor characteristics, and surgical margins.
Therefore, it is important to adhere to the check-up schedule determined by the doctor even if there are no complaints after treatment.
When individuals who have previously received vulvar cancer treatment experience newly developing sores, itching, bleeding, swelling, or skin changes, they should consult a physician without waiting for the routine check-up date.
Is It Possible to Prevent Vulvar Cancer?
It is not possible to prevent all cases of vulvar cancer. However, there are certain measures that can help reduce the risk of vulvar cancers, particularly those associated with HPV.
HPV Vaccine
The HPV vaccine is an important preventive approach in preventing HPV-associated cancers, including vulvar cancer.
The vaccine has the highest protectiveness when administered especially before encountering HPV. However, the age groups and dose schedule in which the vaccine can be applied may vary according to the individual's age and health status.
Quitting Smoking
Smoking is one of the factors that can increase the risk of vulvar cancer.
Quitting smoking provides significant health benefits not only in terms of vulvar cancer but also for many types of cancer, cardiovascular diseases, and lung diseases.
Regular Gynecological Check-ups
There is no standard screening test applied across the general population for vulvar cancer.
Nevertheless, regular gynecological examinations can help early detection of suspicious changes in the vulva.
It is important that individuals with a history of VIN, lichen sclerosus, or prior HPV-related genital lesions adhere to the follow-up program recommended by their doctor.
Precautions Against HPV
It is not always possible to completely eliminate the risk of contracting HPV.
In addition to the HPV vaccine, using barrier methods can reduce the risk of transmission. However, since condoms do not cover the entire genital region, they do not provide complete protection against HPV.
Quitting smoking and avoiding factors that can negatively affect the immune system can also contribute to reducing the overall risk.