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Vulvar cancer



 
 
Vulvar cancer, a malignant invasive growth in the vulva
Vulva

The vulva refers to the external sex organ of the female. In colloquial speech, the term vagina is often used to refer to the female genitals generally, although, strictly speaking, the vagina is a specific internal structure, whereas the vulva is the whole exterior genitalia....
, accounts for about 4 % of all gynecological cancers and typically affects women in later life. It is estimated that in the United States in 2006 about 3,740 new cases will be diagnosed and about 880 women will die as a result of vulvar cancer. Vulvar carcinoma is separated from vulvar intraepithelial neoplasia
Vulvar intraepithelial neoplasia

Vulvar intraepithelial neoplasia denotes a squamous intraepithelial lesion of the vulva that shows dysplasia with varying degrees of atypia. The epithelial basement membrane is intact and the lesion is thus not invasive but has invasive potential....
 (VIN), a non-invasive lesion of the epithelium
Epithelium

In biology and medicine, epithelium is a Biological tissue composed of cell s that line the cavities and surfaces of structures throughout the body....
 that can progress via carcinoma-in-situ to squamous cell cancer, and from Paget disease of the vulva.

vast majority of vulvar cancer is caused by squamous cell carcinoma
Squamous cell carcinoma

In medicine, squamous cell carcinoma is a form of cancer of the carcinoma type that may occur in many different organs, including the skin, lips, mouth, esophagus, urinary bladder, prostate, lungs, vagina, and cervix....
 originating from the epidermis
Epidermis (skin)

The epidermis is the outermost layer of the skin, composed of terminally differentiated stratified squamous epithelium, acting as the body's major barrier against an inhospitable environment....
 of the vulva tissue.






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Vulvar cancer, a malignant invasive growth in the vulva
Vulva

The vulva refers to the external sex organ of the female. In colloquial speech, the term vagina is often used to refer to the female genitals generally, although, strictly speaking, the vagina is a specific internal structure, whereas the vulva is the whole exterior genitalia....
, accounts for about 4 % of all gynecological cancers and typically affects women in later life. It is estimated that in the United States in 2006 about 3,740 new cases will be diagnosed and about 880 women will die as a result of vulvar cancer. Vulvar carcinoma is separated from vulvar intraepithelial neoplasia
Vulvar intraepithelial neoplasia

Vulvar intraepithelial neoplasia denotes a squamous intraepithelial lesion of the vulva that shows dysplasia with varying degrees of atypia. The epithelial basement membrane is intact and the lesion is thus not invasive but has invasive potential....
 (VIN), a non-invasive lesion of the epithelium
Epithelium

In biology and medicine, epithelium is a Biological tissue composed of cell s that line the cavities and surfaces of structures throughout the body....
 that can progress via carcinoma-in-situ to squamous cell cancer, and from Paget disease of the vulva.

Types


Squamous cell carcinoma

The vast majority of vulvar cancer is caused by squamous cell carcinoma
Squamous cell carcinoma

In medicine, squamous cell carcinoma is a form of cancer of the carcinoma type that may occur in many different organs, including the skin, lips, mouth, esophagus, urinary bladder, prostate, lungs, vagina, and cervix....
 originating from the epidermis
Epidermis (skin)

The epidermis is the outermost layer of the skin, composed of terminally differentiated stratified squamous epithelium, acting as the body's major barrier against an inhospitable environment....
 of the vulva tissue. Carcinoma-in-situ is a precursor stage of squamous cell cancer prior to invading through the basement membrane
Basement membrane

The basement membrane is a sheet of cells and fibers that covers two other kinds of cells -- the epithelium, which lines the cavities and surfaces of organs, and the endothelium, which lines the interior surface of blood vessels....
. Most lesions originate in the labia, primarily the labia majora
Labia majora

The labia majora are two prominent longitudinal cutaneous folds which extend downward and backward from the mons pubis to the perineum and form the lateral boundaries of the cleft of venus, which contains the labia minora, interlabial sulci, clitoral hood, clitoral glans, frenulum clitoridis, the Hart's Line, and the vulval vestibule, which...
. Other areas affected are the clitoris
Clitoris

The clitoris is a sex organ that is present only in female mammals. In humans, the visible button-like portion is located near the anterior junction of the labia minora, above the opening of the urethra and vagina....
, and fourchette
Fourchette

Fourchette can have different meanings:* The French language word for fork...
, and the local glands. While the lesion is more common with advancing age, younger women who have risk factors (v.i.) may also be affected. In the elderly treatment may be complicated by the interference of other medical conditions.

Squamous lesions tend to be unifocal, growing with local extension, and spreading via the local lymph system. The lymphatic drainage of the labia proceeds to the upper vulva and mons, then to the inguinal and femoral nodes with both superficial and deep lymph nodes. The last deep femoral node is called the Cloquet’s node; spread beyond this node affects the lymph nodes of the pelvis. The tumor may also invade adjacent organs such as the vagina
Vagina

The vagina is a fibromuscular cylinder tract leading from the uterus to the exterior of the body in female placental mammals and marsupials, or to the cloaca in female birds, monotremes, and some reptiles....
, urethra
Urethra

In anatomy, the urethra is a tube which connects the urinary bladder to the outside of the body. The urethra has an excretory function in both sexes to pass urine to the outside, and also a reproductive function in the male, as a passage for semen....
, and rectum
Rectum

The rectum is the final straight portion of the large intestine in some mammals, and the Gastrointestinal tract in others, terminating in the anus....
 and spread via their lymphatics.

A verrucous carcinoma of the vulva is a subtype of the squamous cell cancer and tend to appear as a slowly growing wart
Wart

A wart is generally a small, rough tumor, typically on hands and feet but often other locations, that can resemble a cauliflower or a solid blister....
.

Melanoma

About 5% of vulvar malignancy is caused by melanoma
Melanoma

Melanoma is a malignant tumor of melanocytes which are found predominantly in skin but also in the bowel and the eye . It is one of the rarer types of skin cancer but causes the majority of skin cancer related deaths....
 of the vulva. Such melanoma behaves like melanoma in other locations and may affect a much younger population. Contrary to squamous carcinoma, melanoma has a high risk of metastasis
Metastasis

Metastasis , or Metastatic disease, sometimes abbreviated mets, is the spread of a disease from one Organ or part to another non-adjacent organ or part....
.

Basal cell carcinoma

Basal cell carcinoma
Basal cell carcinoma

Basal cell carcinoma is the most common of all types of skin cancer. Statistically speaking, approximately 3 out of 10 caucasians develop a basal cell cancer within their lifetime....
 affects about 1-2% of vulvar cancer is a slowly growing lesion and affects the elderly. Its behavior is similar to basal cell carcinoma in other locations that is it tends to grow locally with a low potential of deep invasion or metastasis.

Other lesions

Vulvar cancer can be caused by other lesions such as adenocarcinoma
Adenocarcinoma

Adenocarcinoma is a cancer that originates in glandular tissue. This tissue is also part of a larger tissue category known as epithelial tissue....
 or sarcoma
Sarcoma

A sarcoma is a cancer of the connective tissue resulting in mesoderm proliferation.This is in contrast to Carcinoma, which are of Epithelium origin ....
.

Signs and Symptoms

Typically a lesion is present in form of a lump or ulceration, often associated with itching, irritation, sometimes local bleeding and discharge. Also dysuria
Dysuria

In medicine, specifically urology, dysuria refers to painful urination. This is typically described to be a burning or stinging sensation. It is most often a result of a urinary tract infection....
, dyspareunia
Dyspareunia

Dyspareunia is painful sexual intercourse, due to medical or psychological causes. The symptom is reported almost exclusively by women, although the problem can also occur in men....
 and pain may be noted. Because of modesty
Modesty

Standards of modesty are aspects of the culture of a country or people, at a given point in time, and is a measure against which an individual in society may be judged....
 or embarrassment, symptoms may not be heeded in a timely fashion. Melanomas tend to display the typical dark discoloration. Adenocarcinoma can arise from the Bartholin gland and results in a lump that may be quite painful.

Diagnosis

Examination of the vulva is part of the gynecologic evaluation and may reveal ulceration, a lump, or a mass. A suspicious lesion needs to undergo a biopsy
Biopsy

A biopsy is a medical test involving the removal of Cell_s or Biological tissues for examination. It is the removal of tissue from a living subject to determine the presence or extent of a disease....
 that generally can be performed in an office setting under local anesthesia
Local anesthesia

Local anesthesia is any technique to render part of the body insensitive to pain without affecting consciousness. It allows patients to undergo surgical and dentistry procedures with reduced pain and distress....
. Small lesion can be excised under local anesthesia. Examination of the vulva should include a thorough inspection of the perineal area, including areas around the clitoris and urethra. Palpation of the Bartholin's glands should be performed as well. Supplemental evaluation may include a chest X-ray
Chest X-ray

A chest X-ray, commonly Abbreviation CXR, is a projection radiograph , taken by a radiographer, of the thorax which is used to diagnose problems with that area....
, an IVP
IVP

IVP may stand for:* International Viewpoint, the monthly magazine of the reunified Fourth International* International Visitor Leadership Program , a professional exchange program funded by the U.S....
, cystoscopy
Cystoscopy

Endoscopy of the urinary bladder via the urethra is called cystoscopy.Diagnostic cystoscopy is usually carried out with local anaesthesia. General anaesthesia is sometimes used for operative cystoscopic procedures....
 and proctoscopy
Proctoscopy

Proctoscopy is a common medical procedure in which an instrument called a proctoscope is used to examine the anal cavity, rectum or sigmoid colon....
, as well as blood counts and metabolic assessment.

Differential diagnosis

Other neoplastic lesions that need to be considered in the differential diagnosis are Paget disease of the vulva and VIN. Non-neoplastic vulvar disease includes lichen sclerosus
Lichen sclerosus

Lichen sclerosus is an uncommon disease of unknown cause that results in white patches on the skin, which may cause scarring on and around genital skin....
, squamous cell hyperplasia
Hyperplasia

Hyperplasia is a general term referring to the proliferation of cells within an organ or tissue beyond that which is ordinarily seen . Hyperplasia may result in the gross enlargement of an organ, the formation of a benign tumor, or may be visible only histology....
, and vulvar vestibulitis. Infectious disease lesions can be caused by a number of diseases including herpes genitalis
Herpes genitalis

Herpes genitalis refers to a genital infection by herpes simplex virus.Following the classification HSV into two distinct categories of HSV-1 and HSV-2 in the 60s, it was established that "HSV-2 was below the waist, HSV-1 was above the waist"....
, human papillomavirus
Human papillomavirus

A human papillomavirus is a papillomavirus that infects the skin and mucous membranes of humans. Approximately 130 HPV types have been identified....
, syphilis
Syphilis

Syphilis is a sexually transmitted disease caused by the spirochete bacterium Treponema pallidum subspecies pallidum. The route of transmission of syphilis is almost always through sexual contact, although there are examples of congenital syphilis via transmission from mother to child in utero....
, chancroid
Chancroid

Chancroid is a sexually transmitted infection characterized by painful sores on the genitalia. Chancroid is known to be spread from one to another individual through sexual contact....
, granuloma inguinale, and lymphogranuloma venereum
Lymphogranuloma venereum

Lymphogranuloma venereum is a sexually transmitted disease caused by the invasive Biovar L1, L2, or L3 of Chlamydia trachomatis.LGV was first described by Wallace in 1833 and again by Durand, Nicolas, and Favre in 1913....
.

Etiology

The etiology of the cancer is unclear; however, some condition such as condyloma
Condyloma

Condyloma refers to an infection of the genitals. The two subtypes are:* "condylomata acuminata", another term for genital warts - caused by human papilloma virus, types 6 and 11 and others....
 or squamous dysplasia
Dysplasia

Dysplasia is a term used in pathology to refer to an abnormality in maturation of cell within a tissue . This generally consists of an expansion of immature cells, with a corresponding decrease in the number and location of mature cells....
s may have preceded the cancer. Human papillomavirus
Human papillomavirus

A human papillomavirus is a papillomavirus that infects the skin and mucous membranes of humans. Approximately 130 HPV types have been identified....
 (HPV) is suspected to be a possible risk factor in the etiology of vulvar cancer. Patients infected with HIV
HIV

Human immunodeficiency virus is a lentivirus that can lead to AIDS , a condition in humans in which the immune system begins to fail, leading to life-threatening opportunistic infections....
 tend to be more susceptible to vulvar malignancy. Also, smokers tend to be at higher risk.

Staging

Preclinical staging has been supplemented by surgical staging since 1988. FIGO
International Federation of Gynecology and Obstetrics

The International Federation of Gynecology and Obstetrics, usually just FIGO as the acronym of its French name F?d?ration Internationale de Gyn?cologie et d'Obst?trique, founded in 1954 is a worldwide NGO organisation representing obstetricians and gynecologists in over one hundred territories with the aim to "promote the well-be...
’s revised staging TNM classification system uses criteria of tumor size (T), involvement of lymph nodes (N), and metastasis (M). Stage I describes the early stage of the cancer that still appears to be confined to the site of origin, stage II and III define less or more extensive extensions to neighboring tissue and lymph nodes, while stage IV indicates metastatic disease.

Treatment

Staging and treatment are generally handled by an oncologist familiar with gynecologic cancer. The extent of the surgery is dictated by the surgical staging. Surgery
Surgery

Surgery is a medical specialty that uses operative manual and instrumental techniques on a patient to investigate and/or treat a pathological condition such as disease or injury, to help improve bodily function or appearance, or sometimes for some other reason....
 is a mainstay of therapy and usually accomplished by use of a radical vulvectomy
Vulvectomy

Vulvectomy refers to a gynecology procedure in which the vulva is partly or completely removed. Usually this is performed as a last resort in certain cases of cancer, vulvar dysplasia or Human Papilloma Virus ....
, removal of vulvar tissue as well as the removal of lymph nodes from the inguinal and femoral areas. Complications of such surgery include wound infection, sexual dysfunction, edema and thrombosis. Surgery is significantly more extensive when vulvar cancer has spread to adjacent organs such as urethra, vagina, and rectum. In cases of early vulvar carcinoma the surgery may be less radical and disfiguring and consist of wide excision or a simple vulvectomy.

Radiation therapy
Radiation therapy

Radiation therapy is the medicine use of ionizing radiation as part of cancer oncology to control malignant cell s . Radiotherapy may be used for curative or Adjuvant chemotherapy cancer treatment....
 and chemotherapy
Chemotherapy

Chemotherapy, in its most general sense, refers to treatment of disease by chemicals that kill cells, specifically those of micro-organisms or cancer....
 are usually not a primary choice of therapy but may be used in selected cases of advanced vulvar cancer.

Prognosis

The prognosis of vulvar cancer shows overall about a 75% five year survival rate, but, of course, individually affected by many factors, notably stage and type of the lesion and age and general medical health. Five-year survival is down to about 20% when pelvic lymph nodes are involved but better than 90% for patients with stage I lesions. Thus early diagnosis is imperative.

External links

  • Am Fam Physician 2002;66:1269-74,1276. Review article for general practitioners.