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Vulvar cancer (DIAGNOSIS (HISTOLOGIC TYPES (Squamous cell carcinoma (two…
Vulvar cancer
DIAGNOSIS
- goal of the diagnostic evaluation is to detect vulvar lesions and determine whether biopsy is indicated. includes:
A history of risk factors or symptoms associated with vulvar cancer
A complete pelvic examination
Biopsy of grossly visible lesions that raise suspicion of vulvar cancer
Colposcopy of the vulva, if necessary,
- based upon a vulvar biopsy
- should not be made based upon gross or colposcopic appearance only.
DIFFERENTIAL DIAGNOSIS
- vulvar intraepithelial neoplasia
- lichen sclerosus or lichen planus.
HISTOLOGIC TYPES
Squamous cell carcinoma
- two subtypes, both of which usually occur on the labia or vestibule:
-The keratinizing, differentiated, or simplex type is more common, occurs in older women, is not related to human papillomavirus (HPV) infection, but is associated with vulvar dystrophies such as lichen sclerosus and, in developing countries, chronic venereal granulomatous disease.
-The classic, warty, or Bowenoid type is predominantly associated with HPV 16, 18, and 33, and found in younger women]. Risk factors associated with HPV infection include early age at first intercourse, multiple sexual partners, human immunodeficiency virus (HIV) infection, and cigarette smoking.
Verrucous carcinoma
- cauliflower-like in appearance, it is differentiated from squamous cell carcinoma with a verrucous configuration by biopsy of the lesion base, which shows papillary fronds without the central connective tissue core typical of condylomata acuminata. The lesion grows slowly and rarely metastasizes to lymph nodes, but it may be locally destructive.
Basal cell carcinoma
- usually affects postmenopausal white women and may be locally invasive, although it is usually nonmetastasizing]. The typical appearance is that of a "rodent" ulcer with rolled edges and central ulceration; the lesion may be pigmented or pearly and gray. It is often asymptomatic, but pruritus, bleeding, or pain may occur.
Melanoma
- second most common vulvar cancer histology, accounting for approximately 2 to 10 percent of primary vulvar neoplasms .
- occurs predominantly in postmenopausal, white, non-Hispanic women, at a median age of 68 years
Sarcoma
- The prognosis is generally poor .-
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VIN
- Vulvar LSIL (not a premalignant lesion and equivalent to flat condylomata acuminata, or human papillomavirus [HPV] changes) and vulvar HSIL include HPV-associated disease, whereas differentiated VIN is not HPV-associated.
- prevalence of VIN is higher in premenopausal women than postmenopausal women.
- In premenopausal women, VIN is often associated with human papillomavirus (HPV) infection, immunosuppression, or cigarette smoking, and is multifocal. Postmenopausal women are more likely to have non-HPV associated VIN and unifocal lesions.
- There are no screening strategies for VIN. Pruritus is the most common symptom of VIN.
- Other presentations include a visible lesion, a palpable abnormality, pain, or dysuria, but many women are asymptomatic.
- Squamous intraepithelial neoplasia in more than one location (vulva, vagina, cervix, or perianal area) is relatively common.
- Tissue biopsy is necessary for a definitive diagnosis.
- VIN can be difficult to distinguish clinically from lichen sclerosus or lichen planus, especially when they occur concurrently.
- The goals of treatment are to prevent development of invasive vulvar carcinoma and relieve symptoms, while preserving normal vulvar anatomy and function.
- For women with vulvar HSIL who have a lesion (eg, a lesion that is raised, ulcerative, and/or has irregular borders) and those with clinically significant risk factors for invasive disease (previous VIN or vulvar carcinoma, immunosuppression, tobacco use, age ≥45 years, lichen sclerosus), surgical excision is required
- For women with differentiated VIN, Surgical excision rather than ablation or pharmacologic therapy
TREATMENT
-Verrucous carcinoma — Radical local excision is usually adequate as verrucous carcinoma is locally invasive, but rarely metastasizes.
Suspicious lymph nodes should be biopsied; if positive, then inguinofemoral lymphadenectomy is indicated. Radiation therapy (RT) is thought to be contraindicated because it is thought to induce anaplastic transformation and increase the likelihood of metastases, but the evidence for this is sparse. Recurrences are usually treated surgically.
- Basal cell carcinoma — Basal cell carcinomas are locally aggressive, but rarely metastasize. Therefore, radical local excision without lymphadenectomy is adequate.
- Melanoma — The treatment of vulvar melanoma is discussed elsewhere.
- Sarcoma: Wide local excision is the standard approach to treatment of most vulvar sarcomas.
- Paget disease of the vulva: wide local excision or vulvectomy, depending upon the extent of disease. Radical excision is not required, but a 2 cm margin is preferred.
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RISK FACTORS
- vulvar or cervical intraepithelial neoplasia
- history of cervical cancer,
- cigarette smoking,
- vulvar lichen sclerosus,
- immunodeficiency syndromes,
- northern European ancestry
- HPV
- chronic inflammatory (vulvar dystrophy)
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CLINICAL PRESENTATION
- Vulvar pruritus — Pruritus is a common complaint associated with many vulvar disorders
- Vulvar bleeding or pain may occur in some patients. Dysuria, dyschezia, rectal bleeding, an enlarged lymph node in the groin, or lower-extremity edema are less frequently encountered symptoms
- Vulvar cancer is the fourth most common gynecologic malignancy (after uterine, ovarian, and cervical)
- Squamous cell carcinoma is the most common histologic type of vulvar cancer, comprising at least 75 percent or more of cases
- Other histologies include melanoma, basal cell carcinoma, Bartholin gland adenocarcinoma, sarcoma, and Paget disease.
- Human papillomavirus infection is associated with the majority of vulvar squamous cell carcinomas. In addition, vulvar lichen sclerosus is associated with an increased risk of vulvar cancers