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  • Presentation

Overview of Vulvar Malignancies: Squamous Cell Carcinoma, Melanoma, and Extramammary Paget Disease

Description

The talk reviewed the main vulvar malignancies—squamous cell carcinoma (SCC) and its precursors, melanoma, and extramammary Paget disease (EMPD)—with emphasis on diagnosis, pathology, treatment, and the importance of surveillance. For vulvar SCC precursors, the speaker explained the confusing evolution of terminology and the key distinction between HPV-dependent HSIL/UVIN and HPV-independent differentiated VIN (DIVIN), noting their different p16/p53 profiles, age distribution, and prognoses. HPV vaccination was highlighted as effective prevention, though uptake remains limited. Many HSIL cases can be treated with destructive or topical therapy, while invasive SCC generally requires surgery and sometimes lymph node evaluation; the speaker stressed that lichen sclerosus is a major precursor to SCC, especially when poorly controlled, and lifelong follow-up is essential. Vulvar melanoma was presented as the second most common vulvar malignancy, often occurring in older white women, with poor outcomes and frequent delayed diagnosis because lesions can mimic benign pigmentation; stability, color, and biopsy of suspicious lesions are critical. Management depends on whether the lesion is vulvar versus vulvovaginal and may include surgery, Mohs in selected cases, and sentinel node biopsy for invasive disease. EMPD was described as often subtle at first, later becoming an erythematous, eroded “strawberries and cream” plaque, with frequent subclinical spread and delayed diagnosis. Workup should include appropriate screening for secondary malignancy, and treatment may involve Mohs surgery, topical imiquimod, other topical approaches, or combined multidisciplinary care. Across all entities, the speaker emphasized a low threshold to biopsy, the need for close long-term surveillance, and attention to the major psychosocial and sexual-function impacts of vulvar cancer.

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Conclusions

  • Vulvar neoplasms are uncommon overall, but squamous cell carcinoma accounts for most vulvar cancers, so clinicians need a low threshold to biopsy suspicious lesions.
  • For squamous precursor lesions, distinguishing HPV-related HSIL from HPV-independent dVIN matters because their biology, age distribution, treatment, and prognosis differ.
  • HPV-related vulvar lesions are typically p16-positive and p53-negative, occur in younger women, and are more often suitable for nonsurgical or destructive treatment when in situ.
  • HPV-independent dVIN and lichen sclerosus–associated disease occur in older women, progress more quickly, and are better managed with surgery and close surveillance.
  • Effective HPV vaccination provides strong prevention against HPV-related vulvar disease, but uptake remains suboptimal and the vaccine does not treat established infection.
  • Long-term control of lichen sclerosus with appropriate steroid therapy is essential because poor disease control increases the risk of progression to invasive squamous cell carcinoma.
  • Invasive vulvar squamous cell carcinoma requires surgery, and larger or deeper lesions need lymph node evaluation as well.
  • Mohs surgery appears to be a useful option for selected vulvar cancers, especially dVIN and stage IA squamous tumors, with potentially lower recurrence and better tissue preservation.
  • Vulvar melanoma is rare but aggressive, often presents late, and has worse outcomes than cutaneous melanoma, making careful vulvar examination and biopsy of changing pigmented lesions critical.
  • Pigmented vulvar lesions should be monitored for stability, because melanosis is usually stable while melanoma is more likely to change in color, pattern, or extent.
  • Vulvar melanoma may be treated surgically in situ, but invasive or vulvovaginal disease often requires sentinel node evaluation and still carries poor prognosis.
  • Extramammary Paget disease is frequently diagnosed late because it can resemble benign dermatitis, so biopsy and appropriate workup are essential for chronic vulvar rashes or plaques.
  • EMPD often has substantial subclinical spread, so scouting biopsies and margin-directed approaches are helpful to define true disease extent.
  • When complete surgical clearance is difficult or morbidity would be high, topical treatments such as imiquimod can be effective for some EMPD cases.
  • Because vulvar cancers can significantly affect sexual function and psychosocial well-being, distress screening and multidisciplinary follow-up should be routine.
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