Invasive Vulvar Paget's Disease

s Disease (VPD).Vulvar Paget's Disease (VPD) is an intraepithelial adenocarcinoma, with or without underlying invasive adenocarcinoma.It can be primary (cutaneous and/or adnexal) or secondary, originating from the recto-colic or urothelial epithelium.First described by Dubreuilh in 1901, VPD is not always a purely intraepithelial proliferation; it may present as an invasive form.The reported frequency of invasion varies between 10% and 47%, based on small case series.Three levels of invasion have been described: (1) in situ within the epidermis, (2) microinvasion of the papillary dermis, and (3) deep invasion into the reticular dermis and hypodermis.VPD is a rare cutaneous malignancy, predominantly observed in postmenopausal Caucasian women.The underlying etiology remains poorly understood.VPD is rarely associated with an underlying urogenital, gastrointestinal, or vulvar carcinoma.In approximately 25% of cases, VPD is invasive, which is associated with a worse prognosis compared to non-invasive cases.The recurrence rate of invasive VPD is high, reaching 33% in cases with clear surgical margins and even higher when margins are not clear, regardless of invasion status.Historically, surgical excision has been the treatment of choice.In this article, we report the case of a 67-year-old postmenopausal woman diagnosed with invasive extramammary vulvar Paget's disease, with deep invasion into the underlying dermis and hypodermis, as well as ipsilateral inguinal lymph node involvement.Through this case report and a review of the literature, we discuss the clinical and histopathological diagnosis, treatment, and prognosis of this rare malignancy.