Version May 2024
INTRODUCTION — Vulvar cancer is less common than other gynecologic malignancies, including uterine corpus, ovarian, and cervical cancer; in the United States, vulvar cancer is also less common than vaginal cancer [1]. Squamous cell carcinoma is the most common histologic type of vulvar cancer, comprising at least 75 percent of cases [2,3]. Other histologies include melanoma, basal cell carcinoma, Bartholin gland adenocarcinoma, sarcoma, and Paget disease (table 1).
Human papillomavirus (HPV) 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.
The epidemiology, clinical presentation, diagnosis, and histology of patients with vulvar cancer will be reviewed here. General treatment recommendations are introduced in this topic and discussed in detail elsewhere. Staging, treatment, and prognosis of squamous cell vulvar carcinoma are discussed separately. (See "Squamous cell carcinoma of the vulva: Staging and surgical treatment" and "Squamous cell carcinoma of the vulva: Medical therapy and prognosis".)
EPIDEMIOLOGY — In the United States, there are approximately 6900 new cases of and 1630 deaths from vulvar cancer each year [1].
Incidence rates by race or ethnicity in the United States from 2010 to 2014 were: Non-Hispanic White (2.7 per 100,000 persons), non-Hispanic Black (1.8), Asian American/Pacific Islander (0.9), Hispanic American (1.8), and non-Hispanic American (2.6) [4]. In the United States, some data suggest that Black patients present with vulvar cancer at a younger age and have an increased probability of distant spread, which is a consistent finding across the different tumor models [5,6].
Females in the United States have a 0.3 percent lifetime risk of being diagnosed with vulvar cancer [4]. The average age of diagnosis of vulvar cancer in the United States is 68 years. The age distribution of cases of vulvar cancer from 2010 to 2014 was:
●<20 years – 0.1 percent
●20 to 34 years – 1.8 percent
●35 to 44 – 5.5 percent
●45 to 54 – 14.6 percent
●55 to 64 – 20.4 percent
●65 to 74 – 21.3 percent
●75 to 84 – 21.4 percent
●85 or older – 15 percent
Most patients with vulvar cancer are diagnosed at an early stage; the distribution of stage at diagnosis is: confined to primary site (59 percent), spread to regional organs and lymph nodes (30 percent), and distant metastases (6 percent) [4]. In the United States, survival at five years after diagnosis is 72.1 percent; median age at death is 78 years.
Squamous cell carcinoma is the most common histologic type of vulvar cancer. Most of the data regarding incidence and prevalence are for vulvar cancer in general, not just squamous cell carcinoma. (See 'Histologic types' below.)
RISK FACTORS AND ETIOLOGY — Risk factors for vulvar cancer include vulvar or cervical intraepithelial neoplasia, a prior history of cervical cancer, cigarette smoking, vulvar lichen sclerosus, immunodeficiency syndromes, and northern European ancestry [7,8].
There are two proposed and independent pathways for the development of vulvar squamous cell carcinoma. The first is related to mucosal human papillomavirus (HPV) infection, and the second is related to chronic inflammatory (vulvar dystrophy) or autoimmune processes [9-12]. In a meta-analysis including 92 studies, the prevalence of HPV in vulvar cancer was 39 percent; the most common genotypes were HPV 16 (78 percent) followed by HPV 33 (7.5 percent) [13]. Among those with HPV-positive vulvar cancer, p16INK4a, a surrogate marker for HPV positivity, was also positive in the majority (73 percent) of cases.
For patients with lichen sclerosus, differentiated VIN has been proposed as a precursor lesion to vulvar carcinoma [14,15]. Differentiated VIN is often found in lesions, previously diagnosed as lichen sclerosus, which have progressed to vulvar squamous cell carcinoma. (See "Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia)", section on 'Risk factors and prevention'.)
CLINICAL PRESENTATION — Patients with vulvar cancer typically present with a vulvar lesion, noted by the patient or a clinician. The signs and symptoms of all histologic types of vulvar malignancy are similar. Many patients are asymptomatic at the time of diagnosis, but some patients present with vulvar pruritus or bleeding.
Vulvar lesion — Most patients present with a unifocal vulvar plaque, ulcer, or mass (fleshy, nodular, or warty) on the labia majora; the labia minora, perineum, clitoris, and mons are less frequently involved (picture 1A and picture 1B and picture 1C). In 10 percent of cases, the lesion is too extensive to determine the actual site of origin (picture 1D and picture 2) [16].
Lesions are multifocal in 5 percent of cases; thus, all vulvar and perianal skin surfaces, as well as the cervix and vagina, should be evaluated. A synchronous second malignancy, most commonly cervical neoplasia, is found in up to 22 percent of patients with a vulvar malignancy [17].
Vulvar pruritus — Pruritus is a common complaint associated with many vulvar disorders; it is especially prevalent when there is an underlying vulvar dermatosis (eg, lichen sclerosus or lichen planus) (table 2). (See "Vulvar lichen sclerosus: Clinical manifestations and diagnosis".)
Other presentations — 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 and are suggestive of advanced disease.
DIAGNOSTIC EVALUATION — The goal of the diagnostic evaluation is to detect vulvar lesions and determine whether biopsy is indicated. The diagnostic evaluation for vulvar cancer includes:
●A history of risk factors or symptoms associated with vulvar cancer and of factors that may impact treatment.
●A complete pelvic examination, with attention to inspection and palpation of the vulva and groin for lesions, color changes, masses, or ulceration.
●Biopsy of grossly visible lesions that raise suspicion of vulvar cancer (picture 1A-D and picture 3). The appearance may vary, and lesions may be single or multiple. Typically, squamous cell carcinoma lesions are firm, white, red, or skin-colored papules, nodules, or plaques. Varying degrees of erosion or ulceration may occur. The surface is often friable. (See "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers" and "Vulvar lesions: Diagnostic evaluation", section on 'Procedure'.)
●Colposcopy of the vulva, if necessary, to identify subclinical lesions not appreciated on gross visual examination. (See "Colposcopy", section on 'Vulvar colposcopy'.)
In general, the diagnostic evaluation is similar to that for a patient suspected of vulvar intraepithelial neoplasia. This evaluation is discussed in detail separately. (See "Vulvar squamous intraepithelial lesions (vulvar intraepithelial neoplasia)", section on 'Diagnostic evaluation'.)
DIAGNOSIS — Vulvar cancer is a histologic diagnosis made based on a vulvar biopsy. The diagnosis should not be made based on gross or colposcopic appearance only.
DIFFERENTIAL DIAGNOSIS — The differential diagnosis for vulvar cancer includes other vulvar lesions.
Vulvar cancer must be differentiated from vulvar intraepithelial neoplasia, which may appear similar or identical to squamous cell vulvar carcinoma and can be differentiated only with biopsy.
Vulvar melanoma is rare but is one of the more common nonsquamous histologies of vulvar cancer. There are many etiologies of pigmented lesions of the vulva; common lesions include a melanocytic nevus or angiokeratoma (table 3). Pigmented lesions of the vulva should be examined and evaluated to determine if a biopsy is indicated. The morphology of vulvar melanoma is discussed in detail separately. (See "Vulvar lesions: Differential diagnosis of pigmented (black, brown, blue) lesions".)
Vulvar dermatoses that appear as white patches or plaques may also appear similar to vulvar carcinoma. These include lichen sclerosus or lichen planus. (See "Vulvar lichen sclerosus: Clinical manifestations and diagnosis" and "Vulvar lichen planus".)
Other lesions that appear similar to vulvar carcinoma include epidermal inclusion cysts, condyloma acuminata (genital warts), disorders of Bartholin gland, acrochordons, seborrheic keratoses, and hidradenomas. If one of these disorders is initially suspected but does not respond to appropriate treatment, biopsy should be performed.
The differential diagnosis of vulvar lesions is discussed in detail separately. (See "Vulvar lesions: Differential diagnosis of vesicles, bullae, erosions, and ulcers".)
HISTOLOGIC TYPES — Squamous cell carcinoma is the most common histologic type of vulvar cancer, comprising at least 75 percent or more of cases [2,3]. Other histologies include melanoma, basal cell, Bartholin gland adenocarcinoma, sarcoma, and Paget disease (table 1). The typical distribution of histologies was illustrated in a Dutch cancer registry study from 1989 to 2010 including 5680 patients with vulvar cancer: squamous cell carcinoma (81 percent), basal cell carcinoma (8 percent), melanoma (6 percent), and other histologic subtypes (5 percent) [2].
Squamous cell carcinoma — Seventy-five percent or more of vulvar malignancies are squamous cell carcinomas [2,3]. There are two subtypes, both of which usually occur on the labia or vestibule:
●The keratinizing, differentiated, or simplex type is more common, occurs in older patients, is not related to human papillomavirus (HPV) infection, but is associated with vulvar dystrophies such as lichen sclerosus and, in resource-limited countries, chronic venereal granulomatous disease (picture 4).
●The classic, warty, or Bowenoid type is predominantly associated with HPV 16, 18, and 33, and found in younger patients [18,19]. Risk factors associated with HPV infection include early age at first intercourse, multiple sexual partners, HIV infection, and cigarette smoking. These patients tend to present with early-stage disease [20], although several cases of stage III/IV disease in HIV-infected patients have been reported [21].
Cervical cancer is also strongly linked to persistent HPV infections [22]. Moreover, there is evidence that some high-grade vulvar and vaginal intraepithelial neoplasias are monoclonal lesions derived from high-grade or malignant cervical disease [23]. Presence of the cervix does not appear to be necessary for oncogenic HPV to infect the genital tract. The prevalence of oncogenic HPV subtypes in the vagina is similar in patients who have and have not undergone hysterectomy [24]. (See "Virology of human papillomavirus infections and the link to cancer".)
Verrucous carcinoma — Verrucous vulvar carcinoma is a variant of squamous cell carcinoma that has distinctive features. Although 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 — Basal cell carcinoma is a squamous histology, but is distinct from squamous cell vulvar carcinoma (table 1). Approximately 2 to 8 percent of vulvar cancers are basal cell cancers, and 2 percent of basal cell cancers occur on the vulva [2,25].
Basal cell vulvar carcinoma usually affects postmenopausal White patients and may be locally invasive, although it is usually nonmetastasizing [26,27]. 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.
Basal cell carcinomas are associated with a high incidence of antecedent or concomitant malignancy elsewhere in the body [27]. Thus, a thorough search for other primary malignancies should be performed. (See "Basal cell carcinoma: Epidemiology, pathogenesis, clinical features, and diagnosis".)
Melanoma — Melanoma is the second most common vulvar cancer histology, accounting for approximately 2 to 10 percent of primary vulvar neoplasms [26,28-30].
Melanoma of the vulva occurs predominantly in postmenopausal, non-Hispanic White patients, at a median age of 68 years (range, 10 to 99 years) [29]. By contrast, cutaneous melanomas presenting at other sites often develop before age 45. (See "Melanoma: Epidemiology and risk factors", section on 'Epidemiology'.)
In a United States national cancer database from 1985 to 1994, female genital tract lesions comprised 0.02 percent of all melanomas and 18 percent of mucosal melanomas [31].
Vulvar melanoma is usually a pigmented lesion, but amelanotic lesions also occur. Most arise de novo on the clitoris or labia minora, but can also develop within preexisting junctional or compound nevi [32]. (See "Vulvar lesions: Differential diagnosis of pigmented (black, brown, blue) lesions", section on 'Melanomas'.)
Diagnosis and treatment of vulvar melanoma is discussed in detail elsewhere. (See "Surgical management of primary cutaneous melanoma or melanoma at other unusual sites".)
Sarcoma — Soft tissue sarcomas (including leiomyosarcomas, rhabdomyosarcomas, liposarcomas, angiosarcomas, neurofibrosarcomas, epithelioid sarcomas, and undifferentiated/unclassified soft tissue sarcomas) constitute 1 to 2 percent of vulvar malignancies [33]. The prognosis is generally poor [34,35].
As with soft tissue sarcomas located elsewhere on the extremities and trunk, high-grade lesions that are larger than 5 cm in diameter, with infiltrating margins and a high mitotic rate, are those most likely to recur. (See "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma", section on 'Histopathology'.)
Paget disease of the vulva — Extramammary Paget disease, an intraepithelial adenocarcinoma, accounts for less than 1 percent of all vulvar malignancies [36]. Most patients are in their 60s and 70s and are from a White population.
Pruritus is the most common symptom, present in 70 percent of patients. Vulvar Paget disease is similar in appearance to Paget disease of the breast (see "Paget disease of the breast (PDB)"). The lesion has an eczematoid appearance; it is well demarcated and has slightly raised edges and a red background, often dotted with small, pale islands (picture 5). It is usually multifocal and may occur anywhere on the vulva, mons, perineum/perianal area, or inner thigh. Extension into the vagina has also been reported [37].
Diagnosis is based on characteristic histopathology (picture 6A-B). Vulvar biopsy should be performed in patients with suspicious lesions, including those with persistent pruritic eczematous lesions that fail to resolve within six weeks of appropriate antieczema therapy.
Invasive adenocarcinomas may be present within or beneath the surface lesion in up to 25 percent of patients in small series (picture 7A-B) [36,38-41]. Patients with Paget disease of the vulva should also be evaluated for the possibility of synchronous neoplasms, as approximately 20 to 30 percent of these patients have a noncontiguous carcinoma (eg, involving breast, rectum, bladder, urethra, cervix, or ovary) [42]. Evaluation may include mammography, colonoscopy, urine cytology, and/or transvaginal ultrasound [43].
Bartholin gland carcinoma — Bartholin gland carcinoma comprises approximately 0.1 to 5 percent of all vulvar carcinomas and 0.001 percent of all female malignancies [44]. The incidence of Bartholin gland carcinoma in one series was 0.023 per 100,000 person-years in premenopausal patients and 0.114 per 100,000 person-years in postmenopausal patients [45]. The incidence of Bartholin gland carcinoma is highest among patients in their 60s. Most affected patients do not have a past history of benign Bartholin gland disorders.
Cancers arising in the Bartholin gland are most often adenocarcinomas or squamous cell carcinomas, but transitional cell carcinomas, adenosquamous, and adenoid cystic carcinomas may also develop [46,47]. Most primary adenocarcinomas of the vulva occur in the Bartholin gland. Only the squamous cell carcinomas of the Bartholin gland are related to HPV infection [46].
Metastatic disease is common in cancers of the Bartholin gland because of the rich vascular and lymphatic network in this area. In one series of 11 patients with Bartholin gland cancer, 55 percent developed recurrent disease, and 67 percent were alive at five years [48]. The only two patients with isolated vulvar recurrence were alive without disease at 8 and 180 months, respectively.
Differentiating Bartholin gland carcinoma from benign Bartholin gland masses is discussed separately.
TREATMENT
Squamous cell carcinoma — The treatment of vulvar squamous cell carcinoma depends on both the extent of the vulvar lesion and the lymph node evaluation and is described in detail elsewhere. (See "Squamous cell carcinoma of the vulva: Staging and surgical treatment" and "Squamous cell carcinoma of the vulva: Medical therapy and prognosis".)
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. (See "Treatment and prognosis of basal cell carcinoma at low risk of recurrence".)
Melanoma — The treatment of vulvar melanoma is discussed elsewhere. (See "Locoregional mucosal melanoma: Epidemiology, clinical diagnosis, and treatment", section on 'Vulvovaginal melanoma'.)
Sarcoma — Among the more common histologic types are angiomyxoma [49] and rhabdomyosarcoma; the latter is typically diagnosed in children [50]. (See "Rhabdomyosarcoma in childhood, adolescence, and adulthood: Treatment", section on 'Role of surgery'.)
Wide local excision is the standard approach to treatment of most vulvar sarcomas. Lymphatic metastases are uncommon [49,51] (see "Rhabdomyosarcoma in childhood, adolescence, and adulthood: Treatment", section on 'Role of surgery'). Surgical treatment is often accompanied by preoperative or postoperative radiation in analogy to the treatment of extremity and truncal soft tissue sarcomas at anatomic sites where wide surgical margins cannot be obtained absent major, debilitating sequelae such as limb amputation.
Paget disease of the vulva — The vulva is one of the most common sites for extramammary Paget disease. Treatment typically consists of wide local excision or vulvectomy, depending on the extent of disease, though more conservative surgery has been described in selected patients (eg, older patients, poor performance status) [52]. Radical excision is not required, but a 2 cm margin is preferred. Greater depth of invasion and lymphovascular involvement are poor prognostic markers [51]. Some have suggested that inguinal lymphadenectomy be performed in cases with invasive disease or an underlying adenocarcinoma [53].
Twelve to 58 percent of patients experience a local recurrence, which may occur despite negative surgical margins, presumably because of multicentricity and microscopic extension of disease beyond the clinically visible margins [38,39,54]. It may require a margin of 5 cm of normal-appearing skin from the visible tumor margin to obtain microscopically clear margins [55]. Use of fluorescein mapping [56] or Mohs micrographic surgery (ie, microscopically controlled systematic excision of cancerous tissue) may be associated with lower recurrence rates, particularly for recurrent tumors [51,55,57,58]. A full description of the technique of Mohs micrographic surgery is found separately. (See "Mohs surgery".)
There are no data addressing the benefit of adjuvant RT. The roles of definitive RT, cytotoxic chemotherapy, and topical imiquimod in the treatment of Paget disease of the vulva are also not well defined, but may be an option for some patients [51,59-66]. Overexpression of the human epidermal growth factor receptor 2 (HER2) has been reported [67,68].
Long-term follow-up is indicated because of the high risk of recurrence and the increased risk of noncontiguous carcinoma. Paget disease is associated with underlying invasive adenocarcinomas in up to 25 percent of patients [36,38-41], and another 20 to 30 percent will have or will develop an adenocarcinoma at another nonvulvar location [36,38]. In one series, 8 percent of patients with noninvasive vulvar Paget disease progressed to invasive vulvar disease after a median of five years, but some or all of these cases may represent a de novo invasive lesion rather than progression of noninvasive disease [39]. The vulva should be inspected annually with a low threshold for biopsy. Screening and surveillance for tumors at other sites (breast, lung, colorectum, gastric, pancreas, and ovary) should be considered. (See "Screening for breast cancer: Strategies and recommendations" and "Screening for colorectal cancer: Strategies in patients at average risk".)
Bartholin gland carcinoma — Primary carcinoma of the Bartholin gland is rare, comprising approximately 0.1 to 5 percent of all vulvar carcinomas and 0.001 percent of all female malignancies [44]. The incidence of Bartholin gland carcinoma in one series was 0.023 per 100,000 person-years in premenopausal patients and 0.114 per 100,000 person-years in postmenopausal patients [45]. The incidence is highest among those in their 60s. Most affected patients do not have a past history of benign Bartholin gland disorders.
Approximately 50 percent of Bartholin gland carcinomas are of squamous histology and are thought to originate in the Bartholin duct rather than the gland. The remainder includes a variety of rare adenocarcinomas including adenoid/cystic carcinomas whose indolent natural history, proclivity for perineural invasion, and infrequent spread to lymph nodes mimic the behavior of adenoid/cystic carcinomas of the salivary glands [69,70].
Bartholin gland carcinoma is typically deep within the vulva (figure 1), and diagnosis is often delayed since findings on gross examination appear late in the course of the disease. The most common presentation is as a painless vulvar mass, and a mass in the Bartholin complex is often misdiagnosed as an abscess or cyst. The mass may be solid, cystic, or abscessed, or a solid area may be palpated within a Bartholin cyst. Fixation to underlying tissue is suspicious for malignancy. Extension to the overlying skin is a late manifestation, and extension through the Bartholin duct to the vagina with subsequent bleeding usually occurs only in the setting of bulky disease.
A complete excision of disease often requires an extensive and deep dissection. The traditional approach to therapy is radical vulvectomy with bilateral groin and pelvic lymphadenectomy. Less radical excisions, such as radical local excision or partial vulvectomy with ipsilateral inguinal lymphadenectomy, also appear to be effective [47,71]. Surgical margins are frequently microscopically positive because of proximity of the primary to the anorectum and pubic arch and require postoperative RT to reduce the incidence of local recurrence [47].
Primary chemoradiotherapy or brachytherapy are therapeutic options that may allow sparing of rectal function or obviate the need for surgery entirely in patients with primary carcinoma of the Bartholin gland [72-75]. Chemoradiation may be particularly effective in cancers with squamous histology. If ipsilateral groin nodes are involved, pelvic and bilateral groin radiation may decrease the frequency of regional recurrence. For advanced disease, single case reports describe activity for pegylated liposomal doxorubicin [61] and paclitaxel [76].
Metastatic disease is common in cancers of the Bartholin gland because of the rich vascular and lymphatic network of the vulva. Nevertheless, a study summarizing 30 years' clinical experience and involving 36 patients with Bartholin carcinoma reported a five-year survival rate of 85 percent [47].
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Vulvar cancer and vaginal cancer".)
SUMMARY AND RECOMMENDATIONS
●Epidemiology – Vulvar cancer is the fourth most common gynecologic malignancy in the United States (after uterine, ovarian, and cervical). There are approximately 6000 cases and 1000 deaths from the disease each year. (See 'Epidemiology' above.)
Females in the United States have an 0.3 percent lifetime risk of being diagnosed with vulvar cancer. The average age of diagnosis of vulvar cancer is 68 years. Most patients with vulvar cancer are diagnosed at an early stage (approximately 60 percent are confined to primary site). Survival at five years after diagnosis is 72.1 percent; median age at death is 78 years. (See 'Epidemiology' above.)
●Histologic types – Squamous cell carcinoma is the most common histologic type of vulvar cancer, comprising at least 75 percent or more of cases [2,3]. Other histologies include melanoma, basal cell carcinoma, Bartholin gland adenocarcinoma, sarcoma, and Paget disease (table 1). (See 'Epidemiology' above and 'Histologic types' above.)
●Risk factors – Risk factors for vulvar cancer include vulvar or cervical intraepithelial neoplasia, a prior history of cervical cancer, cigarette smoking, vulvar lichen sclerosus, and immunodeficiency syndromes. Squamous cell carcinoma of the vulva has two proposed pathogenic pathways: human papillomavirus (HPV) infection and chronic inflammatory or autoimmune processes. (See 'Risk factors and etiology' above.)
●Clinical presentation – Patients with vulvar cancer typically present with a vulvar lesion, noted by the patient or a clinician. Many patients are asymptomatic at the time of diagnosis, but some patients present with vulvar pruritus or bleeding. A complaint of a groin mass or urinary or lower gastrointestinal tract symptoms are associated with advanced disease. (See 'Clinical presentation' above.)
●Diagnosis – Vulvar cancer is a histologic diagnosis and is made based on biopsy of a vulvar lesion. The goal of the diagnostic evaluation is to detect vulvar lesions and determine whether biopsy is indicated. The evaluation includes eliciting a history of vulvar cancer symptoms and risk factors, pelvic examination with careful examination to detect and characterize lesions, possible colposcopy, and biopsy. (See 'Diagnosis' above and 'Diagnostic evaluation' above.)
●Differential diagnosis – The differential diagnosis includes other vulvar lesions with a similar appearance. The appearance of vulvar cancers varies. Typically, squamous cell lesions are firm, white, red, or skin-colored papules, nodules, or plaques. Varying degrees of erosion or ulceration may occur. The surface is often friable. Melanoma is a pigmented lesion. The differential diagnosis includes vulvar intraepithelial neoplasia, melanocytic nevus, angiokeratoma, lichen sclerosus, and condyloma acuminata (genital warts). (See 'Differential diagnosis' above.)
●Management – General treatment principles for vulvar cancer are introduced in this topic and discussed in detail elsewhere. (See 'Treatment' above.)
ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges John C Elkas, MD, JD, who contributed to previous versions of this topic review.
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