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  • Two independent pathways for development: HPV or chronic inflammation.

  • History of prolonged vulvar irritation, with pruritus, local discomfort, or slight bloody discharge.

  • Early lesions may suggest or include non-neoplastic epithelial disorders.

  • Late lesions appear as a mass, an exophytic growth, or a firm, ulcerated area in the vulva (eFigure 18–21).

  • Biopsy is necessary for diagnosis.

eFigure 18–21.

Vulvar high-grade intraepithelial lesion (HSIL) with extensive perineal and perianal extension. (Reproduced, with permission, from Hoffman BL, Schorge JO, Halvorson LM, Hamid CA, Corton MM, Schaffer JI. Williams Gynecology, 4th edition. McGraw-Hill, 2020.)


Most cancers of the vulva are squamous lesions that classically occur in women over 50. Vulvar low-grade squamous intraepithelial lesions (LSIL) are benign and do not require intervention. Vulvar high-grade squamous intraepithelial lesions (HSIL) and differentiated vulvar intraepithelial neoplasia (dVIN) are premalignant conditions. Vulvar HSIL (VIN usual type) is associated with HPV, while dVIN is associated with vulvar dermatoses, eg, lichens sclerosus. About 70–90% of premalignant lesions are vulvar HSIL, but HSIL is the precursor for only 20% of vulvar cancers, while dVIN is the precursor for approximately 80% of vulvar cancers. Given that high percentages of HSIL and vulvar cancers are HPV-related, immunization with the HPV vaccine is beneficial to reduce the risk of HPV-related vulvar disease.


Other vulvar lesions must be considered. Vulvar intraepithelial neoplasia may resemble vulvar cancer and must be distinguished by histology. Benign vulvar disorders that must be excluded in the diagnosis of carcinoma of the vulva include inflammatory vulvar dermatoses (psoriasis, lichen sclerosus, lichen planus), chronic granulomatous lesions (eg, lymphogranuloma venereum, syphilis), condylomas, epidermal inclusion cysts, hidradenomas, or neurofibromas (eFigure 18–22). Lichen sclerosus and other associated leukoplakic changes in the skin should be biopsied. The likelihood that a superimposed vulvar cancer will develop in a woman with a non-neoplastic epithelial disorder is low (1–5%).

eFigure 18–22.

Lymphogranuloma venereum (LGV), an infection of the lymphatic and soft tissues of the perineum caused by one of the aggressive L serotypes (L1, L2, or L3) of Chlamydia trachomatis. Note the large ovoid bubo, an inflammatory enlargement of the inguinal lymph nodes characteristic of LGV. (Reproduced, with permission, from DeCherney AH, Nathan L, Laufer N, Roman S. Current Diagnosis & Treatment: Obstetrics & Gynecology, 12th edition. McGraw-Hill, 2019.)


Biopsy is essential for the diagnosis of VIN and vulvar cancer and should be performed with any localized atypical vulvar lesion, including white patches and hyperpigmented lesions. Multiple skin-punch specimens can be taken in the office under local anesthesia, with care to include tissue from the edges of each lesion sampled. Colposcopy of vulva, ...

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