Vulvar cancer
Encyclopedia
Vulvar cancer, a malignant invasive growth in the vulva
, 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
(VIN), a non-invasive lesion of the epithelium
that can progress via carcinoma-in-situ to squamous cell cancer, and from Paget disease of the vulva.
, which originates from the epidermis of the vulvar tissue. Carcinoma-in-situ is a precursor stage of squamous cell cancer prior to invading through the basement membrane
. Most lesions originate in the labia, primarily the labia majora
. Other areas affected are the clitoris
, and fourchette
, 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
, urethra
, and rectum
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
.
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
.
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
or sarcoma
.
, dyspareunia
and pain may be noted. Because of modesty
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.
that generally can be performed in an office setting under local anesthesia
. 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
, an IVP
, cystoscopy
and proctoscopy
, as well as blood counts and metabolic assessment.
, squamous cell hyperplasia
, and vulvar vestibulitis
. Infectious disease lesions can be caused by a number of diseases including herpes genitalis
, human papillomavirus
, syphilis
, chancroid
, granuloma inguinale, and lymphogranuloma venereum
.
or squamous dysplasia
s may have preceded the cancer. Human papillomavirus
(HPV) is suspected to be a possible risk factor in the etiology of vulvar cancer. Patients infected with HIV
tend to be more susceptible to vulvar malignancy. Also, smokers tend to be at higher risk.
’s revised staging TNM classification system uses criteria of tumor size (T), involvement of lymph nodes (N), and metastasis (M). Stage I and stage II describe the early stage of the cancer that still appears to be confined to the site of origin, stage III defines less or more extensive extensions to neighboring tissue and unilateral inguinal lymph nodes, while stage IV indicates metastatic disease to inguinal nodes bilaterally or distant metastases.
is a mainstay of therapy and usually accomplished by use of a radical vulvectomy
, 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
. Sentinel lymph node
(SLN) dissection may eventually replace routine groin node dissection.
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
and chemotherapy
are usually not a primary choice of therapy but may be used in selected cases of advanced vulvar cancer.
Vulva
The vulva consists of the external genital organs of the female mammal. This article deals with the vulva of the human being, although the structures are similar for other mammals....
, 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
The term Vulvar intraepithelial neoplasia refers to particular changes that can occur in the skin that covers the vulva. VIN is not cancer, and in some women it disappears without treatment...
(VIN), a non-invasive lesion of the epithelium
Epithelium
Epithelium is one of the four basic types of animal tissue, along with connective tissue, muscle tissue and nervous tissue. Epithelial tissues line the cavities and surfaces of structures throughout the body, and also form many glands. Functions of epithelial cells include secretion, selective...
that can progress via carcinoma-in-situ to squamous cell cancer, and from Paget disease of the vulva.
Squamous cell carcinoma
The vast majority of vulvar cancer (approximately 90%) is squamous cell carcinomaSquamous cell carcinoma
Squamous cell carcinoma , occasionally rendered as "squamous-cell carcinoma", is a histologically distinct form of cancer. It arises from the uncontrolled multiplication of malignant cells deriving from epithelium, or showing particular cytological or tissue architectural characteristics of...
, which originates from the epidermis of the vulvar 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 thin sheet of fibers that underlies the epithelium, which lines the cavities and surfaces of organs including skin, or the endothelium, which lines the interior surface of blood vessels.- Composition :...
. Most lesions originate in the labia, primarily the labia majora
Labia majora
The labia majora are two prominent longitudinal cutaneous folds that extend downward and backward from the mons pubis to the perineum and form the lateral boundaries of the pudendal cleft, which contains the labia minora, interlabial sulci, clitoral hood, clitoral glans, frenulum clitoridis, the...
. Other areas affected are the clitoris
Clitoris
The clitoris is a sexual 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. Unlike the penis, which is homologous to the clitoris, the clitoris does not...
, and fourchette
Fourchette
Fourchette can have different meanings:* The French word for fork .* The strip or shaped piece used for the sides of the fingers of a glove.* In English, it is a technical term for a type of dessert 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 tubular 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. Female insects and other invertebrates also have a vagina, which is the terminal part of the...
, urethra
Urethra
In anatomy, the urethra is a tube that connects the urinary bladder to the genitals for the removal of fluids out of the body. In males, the urethra travels through the penis, and carries semen as well as urine...
, and rectum
Rectum
The rectum is the final straight portion of the large intestine in some mammals, and the gut in others, terminating in the anus. The human rectum is about 12 cm long...
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 growth, typically on a human’s hands or feet but often other locations, that can resemble a cauliflower or a solid blister. They are caused by a viral infection, specifically by human papillomavirus 2 and 7. There are as many as 10 varieties of warts, the most...
.
Melanoma
About 5% of vulvar malignancy is caused by melanomaMelanoma
Melanoma is a malignant tumor of melanocytes. Melanocytes are cells that produce the dark pigment, melanin, which is responsible for the color of skin. They predominantly occur in skin, but are also found in other parts of the body, including the bowel and the eye...
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 , is the spread of a disease from one organ or part to another non-adjacent organ or part. It was previously thought that only malignant tumor cells and infections have the capacity to metastasize; however, this is being reconsidered due to new research...
.
Basal cell carcinoma
Basal cell carcinomaBasal cell carcinoma
Basal-cell carcinoma is the most common type of skin cancer. It rarely metastasizes or kills. However, because it can cause significant destruction and disfigurement by invading surrounding tissues, it is still considered malignant. Statistically, approximately 3 out of 10 Caucasians may develop a...
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 adenocarcinomaAdenocarcinoma
Adenocarcinoma is a cancer of an epithelium that originates in glandular tissue. Epithelial tissue includes, but is not limited to, the surface layer of skin, glands and a variety of other tissue that lines the cavities and organs of the body. Epithelium can be derived embryologically from...
or sarcoma
Sarcoma
A sarcoma is a cancer that arises from transformed cells in one of a number of tissues that develop from embryonic mesoderm. Thus, sarcomas include tumors of bone, cartilage, fat, muscle, vascular, and hematopoietic tissues...
.
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 dysuriaDysuria
In medicine, specifically urology, dysuria refers to painful urination.Difficult urination is also sometimes described as dysuria.It is one of a constellation of irritative bladder symptoms, which includes urinary frequency and haematuria....
, 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. The causes are often reversible, even when long-standing, but self-perpetuating pain is a factor after the original...
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 biopsyBiopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
that generally can be performed in an office setting under local anesthesia
Local anesthesia
Local anesthesia is any technique to induce the absence of sensation in part of the body, generally for the aim of inducing local analgesia, that is, local insensitivity to pain, although other local senses may be affected as well. It allows patients to undergo surgical and dental procedures with...
. 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
In medicine, a chest radiograph, commonly called a chest X-ray , is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures...
, an IVP
IVP
IVP may stand for:* Institutional Venture Partners, one of the premier later-stage venture capital and growth equity firms* International Viewpoint, the monthly magazine of the reunified Fourth International...
, cystoscopy
Cystoscopy
Cystoscopy is endoscopy of the urinary bladder via the urethra. It is carried out with a cystoscope.Diagnostic cystoscopy is usually carried out with local anaesthesia...
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. A proctoscope is a short, straight, rigid, hollow metal tube, and usually has a small light bulb mounted at the end...
, 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 sclerosusLichen 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 means increase in number of cells/proliferation of cells. It may result in the gross enlargement of an organ and the term is sometimes mixed with benign neoplasia/ benign tumor....
, and vulvar vestibulitis
Vulvar vestibulitis
Vulvar Vestibulitis Syndrome , vestibulodynia, or simply vulvar vestibulitis is vulvodynia localized to the vulvar region. It tends to be associated with a highly localized “burning” or “cutting” type of pain....
. 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 1960s, it was established that "HSV-2 was below the waist, HSV-1 was above the waist"...
, human papillomavirus
Human papillomavirus
Human papillomavirus is a member of the papillomavirus family of viruses that is capable of infecting humans. Like all papillomaviruses, HPVs establish productive infections only in keratinocytes of the skin or mucous membranes...
, syphilis
Syphilis
Syphilis is a sexually transmitted infection caused by the spirochete bacterium Treponema pallidum subspecies pallidum. The primary route of transmission is through sexual contact; however, it may also be transmitted from mother to fetus during pregnancy or at birth, resulting in congenital syphilis...
, chancroid
Chancroid
Chancroid is a sexually transmitted infection characterized by painful sores on the genitalia...
, granuloma inguinale, and lymphogranuloma venereum
Lymphogranuloma venereum
Lymphogranuloma venereum is a sexually transmitted disease caused by the invasive serovars L1, L2, L2a or L3 of Chlamydia trachomatis....
.
Etiology
The etiology of the cancer is unclear; however, some condition such as condylomaCondyloma
Condyloma refers to an infection of the genitals. The two subtypes are:* Condyloma acuminata, or genital warts, caused by human papilloma virus subtypes 6, 11, and others...
or squamous dysplasia
Dysplasia
Dysplasia , is a term used in pathology to refer to an abnormality of development. This generally consists of an expansion of immature cells, with a corresponding decrease in the number and location of mature cells. Dysplasia is often indicative of an early neoplastic process...
s may have preceded the cancer. Human papillomavirus
Human papillomavirus
Human papillomavirus is a member of the papillomavirus family of viruses that is capable of infecting humans. Like all papillomaviruses, HPVs establish productive infections only in keratinocytes of the skin or mucous membranes...
(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 causes acquired immunodeficiency syndrome , a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thrive...
tend to be more susceptible to vulvar malignancy. Also, smokers tend to be at higher risk.
Staging
Preclinical staging has been supplemented by anatomical staging since 1988. FIGOInternational Federation of Gynecology and Obstetrics
The International Federation of Gynaecology and Obstetrics, usually just FIGO as the acronym of its French name Fédération Internationale de Gynécologie et d'Obstétrique, is a worldwide NGO organisation representing obstetricians and gynaecologists in over one hundred territories...
’s revised staging TNM classification system uses criteria of tumor size (T), involvement of lymph nodes (N), and metastasis (M). Stage I and stage II describe the early stage of the cancer that still appears to be confined to the site of origin, stage III defines less or more extensive extensions to neighboring tissue and unilateral inguinal lymph nodes, while stage IV indicates metastatic disease to inguinal nodes bilaterally or distant metastases.
Treatment
Staging and treatment are generally handled by an oncologist familiar with gynecologic cancer. The extent of the surgery is dictated by the anatomical staging. SurgerySurgery
Surgery is an ancient 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, or to help improve bodily function or appearance.An act of performing surgery may be called a surgical...
is a mainstay of therapy and usually accomplished by use of a radical vulvectomy
Vulvectomy
Vulvectomy refers to a gynecological procedure in which the vulva is partly or completely removed. Usually performed as a last resort in certain cases of cancer, vulvar dysplasia, Human Papilloma Virus or FGM/C...
, 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
Edema
Edema or oedema ; both words from the Greek , oídēma "swelling"), formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin or in one or more cavities of the body that produces swelling...
and thrombosis
Thrombosis
Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot to prevent blood loss...
. Sentinel lymph node
Sentinel lymph node
The sentinel lymph node is the hypothetical first lymph node or group of nodes reached by metastasizing cancer cells from a primary tumor.-Physiology:...
(SLN) dissection may eventually replace routine groin node dissection.
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 , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
and chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
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
- http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?dt=45Guide by the American Cancer SocietyAmerican Cancer SocietyThe American Cancer Society is the "nationwide community-based voluntary health organization" dedicated, in their own words, "to eliminating cancer as a major health problem by preventing cancer, saving lives, and diminishing suffering from cancer, through research, education, advocacy, and...
] - http://www.cancer.gov/cancertopics/pdq/treatment/vulvar/healthprofessionalInformation from the National Cancer InstituteNational Cancer InstituteThe National Cancer Institute is part of the National Institutes of Health , which is one of 11 agencies that are part of the U.S. Department of Health and Human Services. The NCI coordinates the U.S...
] - Vulvar Cancer. Timothy P. Canavan and Donna Cohen. Am Fam Physician 2002;66:1269-74,1276. Review article for general practitioners.
- Cancerbackup site
- Anne’s Site about Vulvar Cancer
- Vulva Awareness Campaign Organisation
- Women Conquering Cancer