It is most likely to appear in the outer vaginal lips. Typical symptoms include a lump, itching, and bleeding.
Vulvar cancer accounts for around 0.6 percent of all cancers in women. The American Cancer Society predict that in 2017 there will be about 6,020 diagnoses of vulvar cancers in the United States, and that 1,150 women will die of vulvar cancer.
Here are some key points about the vulvar cancer. More detail is in the main article.
- Vulvar cancer affects the external genital organs of a woman, most commonly the outer lips of the vagina.
- Symptoms include a lump, itching, and bleeding, and with some types discoloration of the skin and pain.
- Early diagnosis increases the chance of successful treatment dramatically, but without treatment, cancer can spread to other parts of the body.
- Regularly attending smear tests and checking for changes to the vaginal lips can help diagnose vulvar cancer in the early stages.
- Avoiding smoking and unprotected sex can reduce the risk.
Symptoms of vulvar cancer include itching and pain.
The vulva includes the labia majora, mons pubis, labia minora, clitoris, the vestibule of the vagina, the bulb of the vestibule, greater and lesser vestibular glands, and vaginal orifice.
Vulvar cancer most commonly affects the outer lips of the vagina.
Cancer that originates in the vulva is called primary vulvar cancer. If it starts in another part of the body and then spreads to the vulva, it is called secondary vulvar cancer.
There are several types of vulvar cancer.
Squamous cell carcinoma affects the flat, outer layers of skin. In medicine, the word squamous refers to flat cells that look like fish scales. About 90 percent of all vulvar cancers are squamous cell carcinomas. It takes several years for noticeable symptoms to develop.
Vulvar melanoma accounts for about 5 percent of all vulvar cancers. A melanoma presents as a dark patch of discoloration. There is a high risk of this type of cancer spreading to other parts of the body, a process known as metastasis. It may affect younger women.
Adenocarcinoma originates in glandular tissue, and in this case, the cells line the glands in the vulva. It accounts for a very small proportion of vulvar cancers.
Sarcoma originates in the connective tissue. Most cancers of this type are malignant. It is rare.
Verrucous carcinoma is a subtype of the squamous cell cancer, and it tends to appear as a slowly growing wart.
The outlook is normally good if diagnosis happens in the early stages, before the cancer spreads, and if the woman receives prompt and appropriate treatment.
Signs and symptoms
The first sign is usually a lump or ulceration, possibly with itching, irritation, or bleeding.
Sometimes, a woman may not seek medical help at once due to embarrassment, but an early diagnosis will improve the outlook.
Most typical symptoms include:
- painful sexual intercourse
- pain and burning
- dark discoloration in cases of melanoma
- painful urination
- persistent itching
- rawness and sensitivity
- wart-like growths
- thickened skin
Different types of vulvar cancer may have different symptoms, and in some cases, there may be no noticeable symptoms. Any changes that take place should be checked with a doctor.
Cancer happens when cell growth is out of control.
Most cancers harm the body when damaged cells divide uncontrollably to form lumps or masses of tissue, or tumors. Tumors can grow and affect body function. A benign tumor stays in one place and does not spread, but a malignant tumor spreads and causes further damage.
Malignancy occurs when two things happen:
- a cancerous cell manages to move throughout the body using the blood or lymph systems, destroying healthy tissue via a process called invasion.
- the cell divides and grows through a process called angiogenesis, making new blood vessels to feed itself.
Without treatment, cancer can grow and spread to other parts of the body. This is called metastasis. If it enters the lymphatic system, it can reach other parts of the body, including vital organs.
Experts do not know exactly why cells start to grow too fast, but certain risk factors increase the chance of developing the disease.
Age: Over half of all cases are in women aged over 70 years, and fewer than 1 in 5 occur before the age of 50 years.
Human papilloma virus (HPV): Women infected with HPV have a higher risk of developing vulval cancer.
Vulvar intraepithelial neoplasia (VIN): This is a general term for a precancerous state, in which certain cells within the vulvar epithelium have a range of low-grade carcinoma. Women with VIN have a significantly higher risk of developing vulvar cancer.
Lichen sclerosus et atrophicus (LSA): This causes the skin to become thick and itchy, and it may increase the susceptibility to vulvar cancer slightly.
Melanoma: A personal or family history of melanoma in other parts of the body increases the risk of vulvar cancer.
Sexually transmitted infections (STIs): Women with a higher level of antibodies to the herpes simplex virus type 2 appear to have a higher risk of vulvar cancer.
Smoking: studies suggest that women who smoke regularly have a three to six times increased risk of vulvar cancer. If the regular smoker also has the HPV infection, the risk is higher still.
Kidney transplant: a kidney transplant appears to increase the chances of developing vulvar cancer. This may be due to the use of immunosuppressant drugs. These drugs are used for the rest of a patient's life after a transplant to keep the body from rejecting the organ.
Human immunodeficiency virus (HIV): people with HIV or AIDS are more susceptible to HPV infection.
Other risk factors include having systemic lupus erythematosus, also known as SLE or lupus, having psoriasis, or having radiotherapy for womb cancer.
Diagnosis and staging
The doctor will carry out a gynecological evaluation, which includes checking the vulva.
If there is an ulceration, lump, or a mass that looks suspicious, a biopsy is required.
The examination should include the perineal area, including the areas around the clitoris and urethra. The doctor should also palpate the Bartholin's glands. Anesthesia may be used.
Depending on the results of the biopsy, there may be further tests:
- Cystoscopy: the bladder is examined to determine whether the cancer has spread to that area.
- Proctoscopy: the rectum is examined to check whether the cancer has spread to the rectal wall.
- Imaging scans: these can help the doctor determine whether the cancer has spread, and if so, where to. An MRI or CT scan may be used. X-rays may be used to determine whether the cancer has reached the lungs.
If a biopsy confirms the presence of vulvar cancer, the doctor will stage it with the help of imaging scans.
There are different ways of staging cancer.
The four-stage system is as follows:
- Stage 0, or carcinoma in situ: The cancer is only on the surface of the skin.
- Stage 1: The cancer is limited to the vulva or perineum and is up to 2 centimeters in size.
- Stage 2: The same as stage 1, but the tumor is at least 2 centimeters in size.
- Stage 3: The cancer has reached nearby tissue, such as the anus or vagina, and it may have reached the lymph nodes.
- Stage 4: The cancer has reached the lymph nodes on both sides of the groin, and it may have reached the bowel, the bladder, or the urethra, the passage through which urine leaves the body.
It is important to seek early diagnosis and treatment, to prevent the spread of cancer.
The types of treatment normally used for vulvar cancer are surgery, chemotherapy, radiation therapy, and biologic therapy.
Surgery is the main way to treat vulvar cancer. Treatment aims to remove the cancer while leaving sexual function intact.
If diagnosis occurs in the early stages of the cancer, limited surgery is required.
At the later stages, and if the cancer has spread to nearby organs, such as the urethra, vagina or rectum, surgery will be more extensive.
Types of surgery include:
- Laser surgery: This uses a laser beam as a knife, to remove lesions.
- Excision: The surgeon attempts to remove all the cancer and some healthy tissue around it.
- Skinning vulvectomy: The surgeon removes the top layer of skin, where the cancer is located. A skin graft from another part of the body can be used to replace what was lost.
- Radical vulvectomy: The surgeon removes the whole vulva, including the clitoris, vaginal lips, the opening to the vagina, and usually the nearby lymph nodes as well
Radiation therapy can shrink deep lesions or tumors before surgery, so they will be easier to remove. It can also treat lymph nodes. It can be used to relieve symptoms and improve quality of life. How it is used depends on which stage the cancer has reached.
Chemotherapy is often used with radiotherapy as part of palliative care. It may be used on the skin, as a cream or lotion, but the method will depend on whether and how far the cancer has spread.
Reconstructive surgery may be possible, depending on how much tissue is removed. Plastic surgery reconstruction can involve skin flaps, and a skin graft is sometimes possible.
Biologic therapy is a kind of immunotherapy. It uses either synthetic or natural substances to help the body defend itself against cancer. Imiquimod is an example. It may be applied topically, as a cream, to treat vulvar cancer.
Up to 24 percent of vulvar cancers will eventually come back. It is important to attend follow-up visits.
Measures that can reduce the risk of developing vulvar cancer include:
- practicing safe sex
- attending scheduled cervical smear tests
- having the HPV vaccination
- not smoking
There is no standard screening for vulvar cancer, but women should carry out checkups as their doctor recommends, and be aware of any changes to their body. The outlook is better with early diagnosis.
If vulvar cancer is diagnosed in the local stage, when it is still in a limited area, the relative chance of living for at least 5 years after diagnosis is 86 percent.
If it has spread to nearby lymph nodes or tissues, the patient has a 54-percent chance of living for at least 5 years. If diagnosis happens when the cancer has already reached more distant organs, the chance of surviving at least 5 more years is 16 percent.
It is important to attend regular pap tests and to look out for any unusual changes, because finding a cancer early increases the chance of a good outcome.
Vulvar cancer is a malignant, invasive growth in the vulva, or the outer portion of the female genitals. The disease accounts for only 0.6% of cancer diagnoses but 5% of gynecologic cancers in the United States. The labia majora are the most common site involved representing about 50% of all cases, followed by the labia minora. The clitoris and Bartholin glands may rarely be involved. Vulvar cancer is separate from vulvar intraepithelial neoplasia (VIN), a superficial lesion of the epithelium that has not invaded the basement membrane—or a pre-cancer. VIN may progress to carcinoma-in-situ and, eventually, squamous cell cancer.
According to the American Cancer Society, in 2014, there were about 4,850 new cases of vulvar cancer and 1,030 deaths from the disease. In the United States, five-year survival rates for vulvar cancer are around 70%.
Squamous cell carcinoma
Most vulvar cancer (approximately 90%) is squamous cell carcinoma, which originates from epidermal squamous cells, the most common type of skin cell. Carcinoma-in-situ is a precursor lesion of squamous cell cancer that does not invade through the basement membrane. While this type of lesion is more common with older age, young women with risk factors may also be affected. In the elderly, complications may occur due to the presence of other medical conditions.
Squamous lesions tend to arise in a single site and occur most commonly in the vestibule. They grow by local extension and spread via the local lymph system. The lymphatics of the labia drain to the upper vulva and mons, then to both superficial and deep inguinal and femoral lymph nodes. The last deep femoral node is called the Cloquet’s node. Spread beyond this node reaches the lymph nodes of the pelvis. The tumor may also invade nearby organs such as the vagina, urethra, and rectum and spread via their lymphatics.
A verrucous carcinoma of the vulva is a rare subtype of squamous cell cancer and tends to appear as a slowly growing wart. Verrucous vulvar cancers tend to have good overall prognoses.
Melanoma is the second most common type of vulvar cancer and causes 8–10% of vulvar cancer cases. These lesions arise from melanocytes, the cells that give skin color and are most common in Caucasian women 50–80 years old. Melanoma of the vulva behaves like melanoma in any other location and may affect a much younger population.
There are three distinct types of vulvar melanoma: superficial spreading, nodular, and acral lentigous melanoma. Vulvar melanomas are unique in that they are microstaged with the Chung, Clark and/or Breslow systems, which specify stage and tumor depth of invasion. In general, they come with a high risk of metastasis and carry a poor overall prognosis.
Basal cell carcinoma
Basal cell carcinoma makes up about 1–2% of vulvar cancer. These tend to be slow-growing lesions on the labia majora but can occur anywhere on the vulva. Their behavior is similar to basal cell cancers in other locations. They often grow locally and have low risk for deep invasion or metastasis.
Treatment involves excision, but these lesions have a tendency to recur if not completely removed.
Bartholin gland carcinoma
Main article: Bartholin gland carcinoma
The Bartholin gland is a rare malignancy and usually occurs in women in their mid-sixties.
Other lesions, such as adenocarcinoma (of the Bartholin glands, for example) or sarcoma, may cause vulvar cancer as well. Erythroplasia of Queyrat, typically found on the penis may affect the vulvae in females.
Although the exact cause of vulvar cancer isn't known, certain factors appear to increase your risk of the disease.
- Increasing age
- Exposure to human papillomavirus
- Being infected with the human immunodeficiency virus (HIV)
- Having a history of precancerous conditions of the vulva
- Having a skin condition involving the vulva
Signs and symptoms
Many malignancies can develop in vulvar structures. The signs and symptoms can include:
- Itching, burn, or bleeding on the vulva that does not go away.
- Changes in the color of the skin of the vulva, so that it looks redder or whiter than is normal.
- Skin changes in the vulva, including what looks like a rash or warts.
- Sores, lumps, or ulcers on the vulva that do not go away.
- Pain in the pelvis, especially during urination or sex.
Typically, a lesion presents in the form of a lump or ulcer on the labia majora and may be associated with itching, irritation, local bleeding or discharge, in addition to pain with urination or pain during sexual intercourse. The labia minora, clitoris, perineum and mons are less commonly involved. Due to modesty or embarrassment, patients may put off seeing a doctor.
Melanomas tend to display the typical asymmetry, uneven borders and dark discoloration as do melanomas in other parts of the body.
Adenocarcinoma can arise from the Bartholin gland and present with a painful lump.
Some conditions such as lichen sclerosus, squamous dysplasia or chronic vulvar itching may precede cancer. In younger women affected with vulvar cancer, risk factors include low socioeconomic status, multiple sexual partners, cigarette use and cervical cancer. Patients that are infected with HIV tend to be more susceptible to vulvar cancer as well. Human papillomavirus (HPV) infection is associated with vulvar cancer.
Examination of the vulva is part of the gynecologic evaluation and should include a thorough inspection of the perineum, including areas around the clitoris and urethra, and palpation of the Bartholin's glands. The exam may reveal an ulceration, lump or mass in the vulvar region. Any suspicious lesions need to be sampled, or biopsied. This can generally be done in an office setting under local anesthesia. Small lesions can be removed under local anesthesia as well. Additional evaluation may include a chest X-ray, an intravenous pyelogram, cystoscopy or proctoscopy, as well as blood counts and metabolic assessment.
Other cancerous lesions in the differential diagnosis include Paget's disease of the vulva and vulvar intraepithelial neoplasia (VIN). Non-cancerous vulvar diseases include lichen sclerosus, squamous cell hyperplasia, and vulvar vestibulitis. A number of diseases cause infectious lesions including herpes genitalis, human papillomavirus, syphilis, chancroid, granuloma inguinale, and lymphogranuloma venereum.
Anatomical staging supplemented preclinical staging starting in 1988. FIGO’s revised TNM classification system uses tumor size (T), lymph node involvement (N) and presence or absence of metastasis (M) as criteria for staging. Stages I and II describe the early stages of vulvar cancer that still appear to be confined to the site of origin. Stage III cancers include greater disease extension to neighboring tissues and inguinal lymph nodes on one side. Stage IV indicates metastatic disease to inguinal nodes on both sides or distant metastases.
- Illustrations showing stages of vulvar cancer'"`UNIQ--ref-0000001B-QINU`"'
Stage 1A and 1B vulvar cancer
Staging and treatment are generally handled by an oncologist familiar with gynecologic cancer. Surgery is a mainstay of therapy depending on anatomical staging and is usually reserved for cancers that have not spread beyond the vulva. Surgery may involve a wide local excision, radical partial vulvectomy, or radical complete vulvectomy with removal of vulvar tissue, inguinal and femoral lymph nodes. In cases of early vulvar cancer, the surgery may be less extensive and consist of wide excision or a simple vulvectomy. Surgery is significantly more extensive when the cancer has spread to nearby organs such as the urethra, vagina, or rectum. Complications of surgery include wound infection, sexual dysfunction, edema and thrombosis, as well as lymphedema secondary to dissected lymph nodes.
Sentinel lymph node (SLN) dissection is the identification of the main lymph node(s) draining the tumor, with the aim of removing as few nodes as possible, decreasing the risk of adverse effects. Location of the sentinel node(s) may require the use of technetium(99m)-labeled nano-colloid, or a combination of technetium and 1% isosulfan blue dye, wherein the combination may reduce the number of women with "'missed"' groin node metastases compared with technetium only.
Radiation therapy may be used in more advanced vulvar cancer cases when disease has spread to the lymph nodes and/or pelvis. It may be performed before or after surgery. Chemotherapy is not usually used as primary treatment but may be used in advanced cases with spread to the bones, liver or lungs. It may also be given at a lower dose together with radiation therapy.
Women with vulvar cancer should have routine follow-up and exams with their oncologist, often every 3 months for the first 2–3 years after treatment. They should not have routine surveillance imaging to monitor the cancer unless new symptoms appear or tumor markers begin rising. Imaging without these indications is discouraged because it is unlikely to detect a recurrence or improve survival and is associated with its own side effects and financial costs.
Overall, five-year survival rates for vulvar cancer are around 78% but may be affected by individual factors including cancer stage, cancer type, patient age and general medical health. Five-year survival is greater than 90% for patients with stage I lesions but decreases to 20% when pelvic lymph nodes are involved. Lymph node involvement is the most important predictor of prognosis. Thus, early diagnosis is important.
Vulvar cancer causes less than 1% of all cancer cases and deaths but around 6% of all gynecologic cancers diagnosed in the UK. Around 1,200 women were diagnosed with the disease in 2011, and 400 women died in 2012. Vulvar cancer causes about 0. 6% of all cancer cases but 5% of gynecologic cancers in the United States. About 4900 cases are diagnosed each year in the United States.
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