What is Vulva Cancer?
Vulvar cancer, based on data from the Surveillance, Epidemiology, and End Results (SEER) Program, makes up 0.3% of all new cancer cases each year in the United States, affecting 2.6 out of every 100,000 women.
It’s most often diagnosed in women in their sixties through eighties and is usually found at an early stage of the disease. The most common type of vulvar cancer is Squamous cell carcinoma (SCC). Some less common types include basal cell carcinoma (BCC), extramammary Paget disease, and vulvar melanoma.
Treatment primarily includes surgery, but medical treatments and radiation therapy are becoming increasingly important in preventing the cancer from coming back and improving patient outcomes.
What Causes Vulva Cancer?
The chances of developing vulvar cancer can increase due to several factors. One of those factors could be getting older. Having an infection with a virus called human papillomavirus, or HPV, is another risk factor. Smoking also increases the risk, as do inflammatory conditions that affect the vulva, which is the outer part of a woman’s genitals. If you’ve had radiation therapy in your pelvic area, this could also increase your risk. Lastly, if your immune system isn’t working as well as it should, which is known as immunodeficiency, you have a higher chance of developing vulvar cancer.
Risk Factors and Frequency for Vulva Cancer
Squamous cell carcinoma is the most usual kind of vulvar cancer. It’s typically found in women aged between 65 and 74, with most cases being diagnosed at around the age of 69. According to research, most of these cancers are detected at a local stage, leading to an 85% chance of survival over five years. The second most frequent type of vulvar cancer is melanoma, which accounts for about 5% of all cases. This cancer often impacts white women aged 50 and 70. The average age of diagnosis for vulvar melanoma is similar to squamous cell carcinoma, which is 68 years. However, about 8.4% of these cases are caught at an advanced stage, meaning the survival rate is lower.
- Squamous cell carcinoma is the most common type of vulvar cancer.
- The cancer is typically diagnosed in women aged 65 to 74, with the average age being 69.
- Most cases (60%) are caught early and have a five-year survival rate of 85%.
- Vulvar melanoma is the second most common type, making up about 5% of all cases.
- This type affects white women between the ages of 50 and 70 more frequently.
- The average age of diagnosis for vulvar melanoma is 68, similar to squamous cell carcinoma.
- About 8.4% of vulvar melanoma cases are advanced and have a lower survival rate.
Signs and Symptoms of Vulva Cancer
The vulva is composed of several parts including the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and the greater vestibular glands. The blood supply to the vulvar comes mostly from the internal and external pudendal arteries. The areas are connected by the ilioinguinal, genitofemoral, and pudendal nerves. The vulvar is connected to the rest of the body through the lymphatic system via the inguinal lymph nodes. It goes in this order: the superficial inguinal nodes, the deeper inguinal nodes, the external iliac nodes, and finally the paraaortic nodes. Diseases related to the vulvar can lead to itching, irritation, or pain but sometimes, there might not be any symptoms at all.
For instance, most patients with vulvar melanoma only discover their condition when it’s in the advanced stages which present signs such as bleeding, a growing mass, and an open sore. It’s important to note that about 25% of vulvar melanomas are amelanotic, making diagnosis difficult for many patients. Another vulvar condition, Paget disease, is also difficult to diagnose due to its nonspecific presentation, leading to delayed diagnosis often by around two years, usually after other treatments like topical steroids or antifungals have proven ineffective. Bartholin’s gland cancer is also not easy to identify as it often appears as a painless visible tumor and is commonly misdiagnosed and wrongly treated as an abscess or cyst before the correct diagnosis is made.
A physical exam could reveal a reddened lesion, a scaly patch, plaques, an open sore, or an ill-defined mass. Lesions of verrucous carcinoma often look like a head of cauliflower. Any suspicious lesions require further investigation, including a pelvic exam, speculum exam, colposcopy of the vulva and vagina, and biopsy. In cases of vulvar melanoma, doctors often use the ABCDE rule, which looks for asymmetry, border irregularity, color, diameter, and evolution to help with the diagnosis.
Testing for Vulva Cancer
The best way to determine if someone has vulvar cancer (a cancer of the outer part of the female genitals) is through a biopsy, which involves taking a small sample of tissue for lab testing. This method, however, is not complete without considering the person’s symptoms and medical history. If a doctor spots a suspicious area on the vulva (the outer part of a woman’s genitals) that could potentially be cancerous, they will likely take a biopsy.
The doctor will pay close attention to where exactly this lesion is located, especially in relation to the midline (the imaginary line that vertically splits the body into right and left halves) and how far it is from the vaginal opening. This information is crucial because, if surgery is necessary, it helps doctors plan for it. Additionally, imaging studies like MRI or CT scans may be performed to see if the cancer has spread.
If the doctor suspects the cancer might have spread to the bladder or rectum, they may perform a cystoscopy (a scope examination of the bladder) or a proctoscopy (an examination of the rectum). This enables them to closely look at these areas for any signs of cancer.
In the case of a specific type of vulvar cancer, known as Paget’s disease, doctors also recommend screening for other types of cancer. This is because sometimes vulvar Paget’s disease can be a secondary condition caused by other types of cancer, primarily within the urinary or digestive tracts, or breast cancer.
Treatment Options for Vulva Cancer
The standard treatment for vulvar cancer is usually an operation to remove the cancerous tissue. However, additional treatments such as radiation or chemotherapy may be recommended based on the specific characteristics and progression of the cancer.
What else can Vulva Cancer be?
Vulvar cancer can sometimes be hard to diagnose because its symptoms can often resemble those of other diseases. Some of these diseases include:
- Skin cancer (cutaneous SCC or BCC, cutaneous melanoma)
- Skin conditions (atopic dermatitis, psoriasis, lichen sclerosus, lichen planus, contact dermatitis)
- Chronic itching or irritation (lichen chronicus simplex)
- Yeast infection (candidiasis)
- Skin disorders (pemphigus vegetans, mycosis fungoides)
Note: The description “Kraurosis vulvae, lichen sclerosus, Chronic inflammatory dermatosis, white plaque, vulva, precancerous, squamous cell carcinoma, Urogenital, Genitalia, Vulvar Diseases” is a detailed medical term related to vulvar conditions and isn’t easily understandable for a non-specialist audience.
Surgical Treatment of Vulva Cancer
Surgery is often the main form of treatment for diseases detected at an early stage. The chances of the disease reoccurring are linked with the size of the tumor, if there are lymph nodes involved, and if there are “positive margins” (where cancer cells are found at the edge of the tissue removed during surgery).
If you have Squamous Case Carcinoma (SCC, a type of skin cancer) the depth of the invasion (how deep the cancer is) can determine the course of treatment. If the depth of the invasion is ≤1mm (less or equal to 1mm), then a surgical process called wide-local excision (removal of the cancer and some healthy tissue around it) without lymphadenectomy (surgery to remove one or more lymph nodes) is typically done. This removal is done ensuring 1 to 2 cm of “surgical margin” or healthy tissue around the tumor is also taken off. However, if the tumor depth is over 1mm or if it’s wider than 2 cm, more radical surgery may be needed extending the excision to the perineal fascia (fibrous tissue enclosing the pelvic floor muscles) and inguinal lymph node (located in the groin) should be done. This approach is needed because once the tumor exceeds such parameters, there’s a higher risk of the cancer cells spreading to the lymph nodes nearby and it poses a more significant threat.
Vulvar melanoma is another form of skin cancer that requires a similar method for treatment: wide local excision. When treating vulvar melanoma, excessively radical surgery doesn’t improve survival and actually increases the potential for complications. Vulvar Paget’s disease (a rare type of cancer that affects the skin in the vulvar region) typically requires local excision. However, because it’s often found in multiple areas, there can be a higher rate of positive margins and recurrences. Surgeons will consider removing lymph nodes in the groin if the invasion is more than 1mm. Mohs surgery (procedure to remove skin cancer layer by layer while leaving the healthy surrounding tissue untouched) might be beneficial in completely removing Pagetoid lesions. In vulvar sarcoma (a type of cancer originating in the bones or soft tissues), the most effective treatment is radical local excision, and not having totally clear margins greatly increases the chances of the disease reoccurring.
The decision to undertake a more detailed surgery (staging lymphadenectomy, a procedure to remove one or more groups of lymph nodes to assess the spread of cancer) is a delicate one, based on the risk of hidden (occult) disease and the patient’s overall health. Unfortunately, for patients with vulvar cancer, there’s no successful treatment if cancer comes back to the groin area. Plus, after a groin dissection (surgery involving the removal of lymph nodes in the groin), anywhere from 14 to 48% of patients will experience swelling in the lower limbs (known as lymphedema). An evaluation of the lymph nodes is recommended for vulvar cancers with a depth of invasion (DOI) over 1mm.
The type of node assessment also affects patients’ health as those who undergo complete lymph node removal face a significantly higher risk of lymphedema than those who go for sentinel lymph node (SNL) biopsy (a procedure where only the first few lymph nodes into which a tumor drains are removed and tested for cancer). This SNL concept has been shown to be safe and very applicable for other cancers like breast cancer, melanoma, and some gynecologic cancers, including vulvar cancer. Studies suggest SNL mapping for early-stage vulvar cancer is safe and helps detect cancer at a high rate.
For vulvar SCC, a surgical procedure known as an ipsilateral inguinal lymphadenectomy can be performed if the primary lesion (the original area where the cancer started) is more than 1cm from the centerline of the vulva. This is because the risk of the cancer spreading to the lymph nodes on the other side is less than one percent. The likelihood of not detecting vulvar cancer is only 2% if the original tumors are smaller than 4 cm, which increases to 7.4% if the tumors are larger than 4 cm. Although this process may include the use of technetium-99 (radioactive tracer) combined with blue dye, it produces a near-perfect rate of sentinel lymph node detection.
Other types of vulvar cancers like BCC, verrucous carcinoma, and vulvar sarcoma each have their specific treatment protocols depending on their type and features. Nonetheless, there are similarities in the recommended treatments with those for vulvar SCC.
What to expect with Vulva Cancer
In the case of vulvar cancer, the disease typically spreads first to the superficial lymph nodes on the same side of the body, and then to the deeper lymph nodes in the groin. The condition of the lymph nodes can significantly impact a patient’s chances of survival. Survival rates are much lower for patients where the cancer has spread to the lymph nodes; with survival rates of 65% at 10 years, 52.5% at 5 years and 56.2% at 3 years, comparing to 91%, 87.5%, and 90.2% respectively in patients without lymph node metastasis.
However, patients who have cancer in their lymph nodes but receive additional radiation therapy have improved chances for a cancer-free recovery. That said, the recurrence rate, meaning the cancer returns, is generally high with 37% of patients experiencing a return within five years.
Patients whose cancer has spread to other areas of the body outside the vulva tend to have a poorer prognosis. For vulvar melanoma (skin cancer), the outlook isn’t great, with an estimated 5-year survival rate of between just 10% to 63%. Especially, patients with melanoma located centrally in the vulva have a lower survival rate, with the cancer likely to return sooner.
Vulvar Paget’s disease, another type of vulvar cancer, tends to have a better prognosis although it frequently comes back. If the patient also has adenocarcinoma (a type of cancer arising from glandular cells), the outlook depends on the type and success of the treatment for it. Recurrence is common in cases of vulvar sarcoma (soft tissue cancer), particularly if the entire tumor is not removed during surgery, or if the tumor is larger than 5mm, involves a deep margin, and has a high cell growth rate.
The prognosis for Bartholin gland carcinoma (a rare type of vulvar cancer) is dependent on the stage at diagnosis, similar to the skin cancer (SCC) of the vulvar. The patient’s HPV status also plays a role in prognosis, with HPV-positive vulvar cancers generally having a better outlook. This information could aid in planning more conservative treatment approaches.
For recurrent disease that is confined to the vulva, surgical removal can be a successful treatment. However, if the returning cancer has spread to other areas or aggressively invaded local tissues, the overall 2-year survival rate is only 57% even after extensive surgery.
Possible Complications When Diagnosed with Vulva Cancer
The complications caused by vulvar cancer can result from the progression of the disease itself or from its treatment. As the cancer spreads, it may result in increasing pain and irritation and a decline in the patient’s overall health and functional ability.
Surgical treatment of vulvar cancer can also lead to various complications. These include the risk of infection, significant loss of blood, lymphedema (swelling caused by a blockage in the lymphatic system), and chronic pain. If additional procedures are required to remove the disease effectively, complications may involve other organs.
Chemotherapy and radiation treatments can bring their own set of complications. These include increased risk of infections, a range of uncomfortable stomach and gut reactions, as well as tissue hardening (fibrosis) and lymphedema.
Typical Complications:
- Increasing pain and irritation
- Decline in health and functional ability
- Infections from surgery
- Significant blood loss
- Swelling caused by a blockage in the lymphatic system (lymphedema)
- Persistent pain
- Complications related to other organs due to additional procedures
- Susceptibility to infections from chemotherapy and radiation
- Multiply gastrointestinal issues caused by chemotherapy and radiation
- Tissue hardening or fibrosis
- Lymphedema caused by chemotherapy and radiation
Recovery from Vulva Cancer
Given the high chance of the condition coming back, it’s very important for patients to remain closely monitored after treatment. Regular check-ups should be scheduled as follows: every three months for the first two years, every six months for the next three years, and then once a year after that.
Preventing Vulva Cancer
It’s important for patients to understand their illness. Knowing what it entails and why regular check-ups are crucial can help manage the condition better. Patients should also be well-informed about the various treatments that are available, including the risks associated with each one. Once the treatment has concluded, it is essential for patients to understand the importance of regular monitoring and maintaining general health with follow-up visits to their doctor.
