- Vulvar cancer
Name = PAGENAME
DiseasesDB = 14013
ICD10 = ICD10|C|51|.9|c|51
ICD9 = ICD9|184.4
MedlinePlus = 000902
eMedicineSubj = med
eMedicineTopic = 3296
eMedicine_mult = eMedicine2|med|3328
MeshID = D014846
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. [cite web | url = http://www.cancer.org/downloads/STT/CAFF2006PWSecured.pdf | author = American Cancer Society | year = 2006 | title = Cancer facts and Figures 2006 | accessdate = 2006-10-13] Vulvar carcinoma is separated from vulvar intraepithelial neoplasia(VIN), a non-invasive lesion of the epitheliumthat can progress via carcinoma-in-situto squamous cell cancer, and from Paget disease of the vulva.
Squamous cell carcinoma
The vast majority of vulvar cancer is caused by
squamous cell carcinomaoriginating from the epidermis of the vulva 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 rectumand 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
About 5% of vulvar malignancy is caused by
melanomaof 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.
Basal cell carcinoma
Basal cell carcinomaaffects 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.
Vulvar cancer can be caused by other lesions such as
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, dyspareuniaand pain may be noted. Because of modestyor embarrassment, symptoms may not be heeded in a timely fashion. Melanomas tend to display the typical dark discoloration.Adenocarcinoma can arise from the Bartholin glandand results in a lump that may be quite painful.
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
biopsythat 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. cite web | title =Vulvar Cancer | work =Gynecologic Neoplasms | url=http://www.health.am/cr/vulvar-cancer/ | year = 2005 | publsiher=Armenian Health Network, Health.am | accessdate=2007-11-08] Supplemental evaluation may include a chest X-ray, an IVP, cystoscopyand proctoscopy, as well as blood counts and metabolic assessment.
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, 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.
The etiology of the cancer is unclear; however, some condition such as
condylomaor squamous dysplasias 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 HIVtend to be more susceptible to vulvar malignancy. Also, smokers tend to be at higher risk.
Preclinical staging has been supplemented by surgical staging since 1988. FIGO’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.cite web | url = http://www.igcs.org/guidelines/guideline_staging-booklet.pdf| author = International Federation of Gynecologists and Obstetricians (FIGO)| title = Staging classification and clinical practice guidelines of gynaecologic cancers | year = 2000 |accessdate = 2006-10-13]
Staging and treatment are generally handled by an
oncologistfamiliar with gynecologic cancer. The extent of the surgery is dictated by the surgical staging. Surgeryis 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.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 therapyand chemotherapyare usually not a primary choice of therapy but may be used in selected cases of advanced vulvar cancer.
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.
* [http://www.cancer.org/docroot/CRI/CRI_2_3x.asp?dt=45 Guide by the
American Cancer Society]
* [http://www.cancer.gov/cancertopics/pdq/treatment/vulvar/healthprofessional Information from the
National Cancer Institute]
* [http://www.aafp.org/afp/20021001/1269.html Vulvar cancer review article]
* [http://www.cancerbackup.org.uk/Cancertype/Vulva Cancerbackup site]
* [http://annescancer.tripod.com/index.html Anne’s Site about Vulvar Cancer]
* [http://vaco.co.uk/ Vulva Awareness Campaign Organisation]
* [http://www.geocities.com/womenconqueringcancer/ Women Conquering Cancer]
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