Paget's disease of the vulva: Prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision

Vulvar Paget Disease: One Century After First Reported

“…It affects mainly white patients aged between 40 and 80 years. The median age is approximately 70 [10][11][12][13] both for intraepithelial and invasive vulvar Paget disease patients [14]. No element in patients' family, environmental, or social histories suggested an etiology or propensity for developing vulvar Paget disease.…”

“…In Table 2 the studies where a distinction among the above-cited subgroups was feasible are reported [11, [13][14][15]20 …”

“…The development of invasive vulvar Paget disease is reported up to 10 years after initial treatment for intraepithelial vulvar Paget disease [20]. In most invasive vulvar Paget disease an extensive intraepithelial skin involvement (Ն 10 cm in diameter) is reported [13], and invasion does appear to develop in an undifferentiated area of intraepithelial disease [106].…”

Androgen receptors are frequently expressed in mammary and extramammary Paget's disease

“…In contrast, primary vulvar Paget's disease is associated with a high recurrence rate of up to 60% after primary surgery. 7,8 This relatively high rate of recurrence can be explained by either (i) positive resection margins, (ii) limitation of the vulvar surgery due to the anatomy of this region, or (iii) multifocal nature of the disease, even if the examined resection margins are histologically negative.…”

Extramammary Paget Disease: Case Report

“…8 Hastalarda çoğunlukla primer intraepitelyal lezyon olsa da, %4-20 oranında vulva, vajina, serviks, uterus, mesane, over, karaciğer, meme, kolon ve rektum kanseri eşlik edebilir. 9,10 Eritematöz plak olarak başlayabilir, daha sonra büyüyerek tümöre dönüşebilir, en önemli özelliği keskin sınırlı olmasıdır. 6 Asemptomatik veya kaşıntılı olabilir, yanma hissi eşlik edebilir, kanayabilir.…”