Fig. 8: Schematic representation of HPV-independent, p53-wild-type verruciform acanthotic Vulvar Intraepithelial Neoplasia. | Modern Pathology

Fig. 8: Schematic representation of HPV-independent, p53-wild-type verruciform acanthotic Vulvar Intraepithelial Neoplasia.

From: HPV-independent, p53-wild-type vulvar intraepithelial neoplasia: a review of nomenclature and the journey to characterize verruciform and acanthotic precursor lesions of the vulva

Fig. 8

HPVi(p53wt) vaVIN, as defined, includes lesions formerly described as differentiated exophytic vulvar intraepithelial lesion (DEVIL). DEVIL, in turn, was characterized on cohorts formerly classified as vulvar acanthosis with altered differentiation (VAAD) and verruciform lichen simplex chronicus (vLSC). It also includes other lesions with verruciform atypia, which along with VAAD and vLSC harbor recurrent PIK3CA and HRAS mutations. All these lesions have a risk for concurrent or subsequent invasive squamous cell carcinoma. The so-called hypertrophic lichen sclerosus has overlapping features with HPVi(p53wt) vaVIN, but it remains to be documented whether it represents a distinct precursor or belongs to the HPVi(p53wt) vaVIN spectrum. The term vulvar aberrant maturation (VAM) is recommended by the International Society of the Study of Vulvovaginal Diseases. This term could be considered, but only when faced with a lesion that does not fit in the clinical and pathologic definition of HPVi(p53wt) vaVIN and after exclusion of HSIL/uVIN and HPVi(p53mutant) dVIN, with an explanatory note. Lastly, this category may include lesions that are not acanthotic/verruciform, which requires further study.

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