Vulvar Cancer

What is the Vulva?

The vulva is the external female genital organ composed of 3 portions- the “Labia Majora,” “Labia Minora,” and “Clitoris.” The skin between the vagina and anus (“Perineum”) is considered an extension of the vulva. The urethral opening for urination is close to the clitoris, in the upper vulvarregion. Thus, the vulva is the vaginal “lips” and surrounding area. A cancer of the vulva is not considered “vaginal” cancer, since it arises outside of the vagina proper and behaves differently, tending to spread to different areas. There is a system of draining channels (“lymph channels”) to drain blood serum from the vulva. The drained blood serum is purified by glands (“lymph nodes”), which are normally pea-sized but swell (“lymphadenopathy”) when invaded by infection or cancer. Specifically, the vulva usually drains first to lymph nodes in either upper thigh (“inguinal nodes”), it afterward may drain to lymph nodes in the pelvis proper (“pelvic nodes”). These pelvic nodes then interconnect (via lymph channels) to those in the abdomen (“paraaortic nodes”); the filtered blood serum is finally rejoins the bloodstream above the level of the heart (via the “left thoracic duct”). The lymph glands, which are normally filled with White Blood Cells, are important as they can act as a conduit for the spread of infections or cancer . Initially, at least, disease of the right half or the vulva spreads to the right inguinal nodes, and that of the left vulva to left inguinal nodes. A cancer is the midportion of the vulva spreads equally to both right and left inguinal nodes. There is some “interconnection” between the right and left lymph nodes, so this rule is not steadfast, but it is useful “clinically” to doctors. Furthermore, the vulva has a rich blood supply and venous drainage, which can also promote spread of disease to anywhere in the body. However, this distant spread tends to occur only long after the areas(“regional”) lymph nodes are involved. The vulva can be removed (“vulvectomy”) which interferes with sexual function, but it is not considered a “vital organ” (necessary to live).

What is Vulvar Cancer?

The vulva is composed of various “cells,” which are intricately combined together into “tissues” which form the “organ” . The vulva contains fat, muscle and skin cells. These cells divide to produce new ones, and grow very rapidly during womb life, early childhood and puberty. In adulthood, new cells are produced only to replace those that die of old age, injury or disease. Normally, division of cells is under very tight control. This control is exerted by the “genes” inside each cell, which are housed in long clumps forming“chromosomes,” which are visible under a light microscope. The genes themselves are made up ofDNA, the master genetic code material. If the genes are damaged, say by chemicals or radiation, the control over cell division may be lost in one particular cell. Ultimately, cancer is considered a disease of the DNA. Vulvar cancer starts in a single cell . That cell starts dividing haphazardly, making millions and billions of copies of itself. It takes up the nourishment needed by other cells, depriving them so the cancer can continue to grow. Quickly growing cells can clump up to form a “tumor” . A tumor simply means a swelling, it can be caused by inflammation or infection. A “benign” tumor only grows in it’s local area (although it may get quite large)– it cannot spread and is not cancer. By contrast, a tumor which can spread to other body areas is called “malignant” and this is cancer . The process of cancer spread to other areas is called “metastasis,” so only malignant tumors (i.e. cancer) can metastasize. Theoretically, cancer can spread to any area of the body, and it often grows better in it’s area of spread than in it’s area of origin (“primary site”) . It is this capacity for spread that makes cancer so dangerous. If not treated successfully, vulvar cancer ultimately kills by urinary blockage, debility, anemia, infection, and damage to distant organs like the liver and brain.

How Does Vulvar Cancer Spread?

The most common area for vulvar cancer to start is on the Labia Majora, it is three times more common here than on the Labia Minora. Only about 10% of cases initially involve the Clitoris. When a cell turns pre-cancerous, it may start dividing but stay localized for many years, or even many decades. This is called“Vulvar Intraepithelial Neoplasia (“VIN”) . A portion of “VIN” cases progress to“Carcinoma in Situ” (“CIS”), which is technically the first stage of actual cancer. Only about 5% of VIN cases progress all the way to“Invasive Cancer,” but we don’t know in advance which ones will or won’t. Once Invasive Cancer manifests, it tends to grow for month to years in it’s local area. It then spreads to the Inguinal lymph nodes(“lymphogenously”), usually to one side first. Then it spreads to deep pelvic lymph nodes. Only 4% of patients have pelvic lymph nodes involved(“positive”) in the absence of Inguinal lymph node involvement. The cancer may then track up through the lymph channels to invade Paraaortic lymph nodes in the abdomen. It continue to grow locally to invade the skin, urethra, bladder, perineum, rectum, and pelvic bone . It tends to spread through the bloodstream (“hematogenously”) only late in the disease, mostly to the liver, lung, bone and brain.

How Common is Vulvar Cancer?

There are about 3200 new cases of invasive vulvar cancer each year in the U.S.A., it accounts for1200 deaths annually. Vulvar cancer represents 4% of the total cancers involving the female genital tract (“gynecologic malignancies”). Overall, gynecologic malignancies account for 13% of new cancer cases in American women. Patients with the precancerous VIN condition have are an average of 44 years old. Frank cancer (but not VIN) is rare before age 50, and the average patient is 61 years old. The overall incidence (number of new cases annually) of vulvar cancer is steady. Over 90% of cases are “Squamous Cell Carcinoma,” which originates from the “epithelial” (skin and lining cells) of the vulva (the ones overlying the fat and muscle). About 7% of cases are“Melanoma,” arising from“melanocyte” pigment cells; this has more predilection for spread in the bloodstream.“Paget’s Disease of the Vulva” is a pre-cancerous condition showing on the vulvar skin a red and velvety area, it has an underlying “invasive cancer” in about 2% of cases. Paget’s is a “marker” for the development of another gynecological malignancy (e.g. cervix or uterine cancer) which eventually occurs in 25% of patients with it. “Bartholin’s Gland Cancer” is an “adenocarcinoma” (arises from gland cells) seen exclusively in post-menopausal women (over about age 50) it makes up less than 1% of vulvar cancer cases. Other rare possibilities are“Sarcoma” (from the underlying muscle or fat), “Lymphoma” (from the immune cells in the vulva) or“Basal Carcinoma” (a skin cancer) The treatment for these follows that in other body areas where they are more common.

What Causes, or Increases the Risk for Vulvar Cancer?

As for any cancer, the exact reason why one woman gets vulvar cancer and another does not remains unknown. However, these “risk factors” are often present:

1) Vulvar Intraepithelial Neoplasia (VIN) or Carcinoma in Situ (CIS) may exist for several decades before manifesting as Invasive Cancer, which they only go on to less in less than 5% of patients.
2) Viruses of the Genital Tract — Specifically Human Papillomavirus (“HPV”) which causes genital warts (“condyloma acuminatum”). Around 5% of patients with vulvar cancer have genital warts. Also Herpes Simplex II is also associated with VIN and later Invasive Cancer. These viruses are sexually transmitted(“STD’s”) .
3) Nulliparity means never having had children, about 25% of American women are “nulliparous.” This is a risk factor for breast, uterus, and ovarian cancer too. Menopause at an early age (i.e. the mid 40′s) has a higher risk also.
4) Lower Socioeconomic Status means being poor, especially as a member of a generally poorer group (Blacks, Hispanics, Native Americans). Some feel that the higher incidence is explained by more promiscuity in these groups (more chance of getting a Sexually Transmitted Disease from multiple male partners). It is probable that the poor don’t get medical attention until the disease is further advanced, and have other medical problems associated with this cancer.
5) Other Medical Problems associated with vulvar cancer include being obese, having high blood pressure, heart disease, diabetes, and kidney problems. All of these conditions are related to each other. 
6) Occupation and Environmental Exposure
— Women who worked in the Laundry or Custodian (cleaning) Industries have a greater risk, reason unknown.

What are the Symptoms of Vulvar Cancer?

Very early vulvar cancer has no symptoms, as it is too small to cause problems.
1) A Lump or Bump (“Mass”) on the vulva is the most common first sign, it can become a sore(“lesion”) which will not heal and grows slowly over months.
2) Itching (“Pruritis”) and Pain will occur as the cancer grows in it’s local area.
3) Bleeding will occur as the cancer breaks through the skin. It may be scant.
4) Lymph Gland Swelling (“lymphadenopathy”) in the pelvis; this does not prove that the glands are involved since infection will also swell them. On the other hand, non-swollen glands may still be microscopically involved with cancer.
5) Urinary and Bowel Problems can occur as the cancer invades the urethra, bladder and rectum respectively.
6) Signs of Distant Spread include back pain (from spread to para-aortic lymph nodes), bone pain from spread there, or nervous system problems from brain spread. Vulvar cancer tends to grow large in it’s local area prior to spreading.
***20% of patients have no previous symptoms when their disease is detected.

Is Vulvar Cancer Preventable?

There is no sure way of preventing vulvar cancer . Being careful to avoid getting a Sexually Transmitted Disease will lower the risk, as will good vulvar hygiene. If a woman has the risk factors for this disease, she should be especially vigilant about doing vulvar“self-exam” on a monthly basis to look for any new suspicious areas, and bring them to a doctor’s attention without delay.

The vulva is the genital organ area between the vagina and upper thighs in a women. The vulva includes the mound of tissue on pelvic surface (mons), the vaginal lips (labia), and clitoris. Infections of the vulva, including sexually transmitted diseases, are the main problems women have with this area.Rarely, the vulva gets cancer. It is critical to get prompt diagnosis and proper treatment of a vulvar cancer problem; this can literally make the difference between life and death. In the past, radical and mutilating surgery was all that could be done, and it was often unsuccessful. Fortunately, improved treatments are now available which often maintain sexual function and give better survival than ever before. Understanding your options will give you the peace-of-mind of knowing you have done everything possible to ensure a successful outcome for yourself or a loved one.

CancerAnswersÕs material explains, in plain English, the definition, types, risk factors, frequency, evaluation, historical and latest effective treatments for vulvar cancer. We describe surgery, radiation, and chemotherapy along with their side-effects and results. While we don’t promise a cure, we tell you everything you need to know to help you make the right choices today in dealing with a vulvar cancer problem.

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