Paget's disease of the vulva:

Tsai, Cheng-Wei; Lin, Ho‐Hsiung; Chang, Daw-Yuan; Huang, Su‐Cheng

doi:10.3109/00016349609055033

Cited by 3 publications

(2 citation statements)

References 9 publications

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“…Portanto, não há evidências na literatura de que a avaliação das margens cirúrgi-cas com biópsia de congelação reduza a recidiva ou melhore a sobrevida das pacientes com DPV 1 . A recorrência pode ocorrer em um terço dos casos, independente das margens cirúrgicas 4,10,16 , como também em enxertos de pele retirados de outra parte do corpo 17 e em até quinze anos após o tratamento 8,9 , resultante da retrodisseminação de células de Paget, através dos vasos linfáticos de sítio metastático oculto prévio 18. A lesão que se manifesta na recidiva é quase sempre in situ 19 . Quando acomete a região perianal tem taxa de 17 .…”

Section: Discussionunclassified

Doença de Paget invasiva da vulva e região perianal: relato de caso

Trindade¹,

Polcheira²,

Basílio³

et al. 2004

Rev. Bras. Ginecol. Obstet.

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Section: Discussionunclassified

Doença de Paget invasiva da vulva e região perianal: relato de caso

Trindade¹,

Polcheira²,

Basílio³

et al. 2004

Rev. Bras. Ginecol. Obstet.

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“…; eFigure in Supplement 1), and 135 articles were retained when (1) cases were limited to a single anatomic subtype (ie, perianal, vulvar, or penoscrotal) or (2) case-level data allowed distinction among these 3 anatomic subtypes (eTable in Supplement 1). Among the included articles, 113 (83%) included 10 or more cases of EMPD, suggesting reasonable cohort sizes considering the rarity of the disease. Cases that were metastatic on presentation were excluded.…”

Section: Methodsmentioning

confidence: 99%

Anatomic Subtype Differences in Extramammary Paget Disease

Kibbi,

Owen,

Worley

et al. 2024

JAMA Dermatol

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ImportanceExtramammary Paget disease (EMPD) is a rare, highly recurrent cutaneous malignant neoplasm of unclear origin. EMPD arises most commonly on the vulvar and penoscrotal skin. It is not presently known how anatomic subtype of EMPD affects disease presentation and management.ObjectiveTo compare demographic and tumor characteristics and treatment approaches for different EMPD subtypes. Recommendations for diagnosis and treatment are presented.Data SourcesMEDLINE, Embase, Web of Science Core Collection, and Cochrane Reviews CENTRAL from December 1, 1990, to October 24, 2022.Study SelectionArticles were excluded if they were not in English, reported fewer than 3 patients, did not specify information by anatomic subtype, or contained no case-level data. Metastatic cases on presentation were also excluded.Data Extraction and SynthesisAbstracts of 1295 eligible articles were independently reviewed by 5 coauthors, and 135 articles retained. Reporting was in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guidelines. The analysis was cunducted in August 2019 and updated in November 2022.FindingsMost vulvar EMPD cases were asymptomatic, and diagnosis was relatively delayed (mean, 25.1 months). Although most vulvar EMPD cases were intraepidermal (1247/1773 [70.3%]), radical surgeries were still performed in almost one-third of cases. Despite this aggressive surgical approach, 481 of 1423 (34%) recurred, commonly confined to the skin and mucosa (177/198 [89.4%]). By contrast, 152 of 1101 penoscrotal EMPD cases (14%) recurred, but more than one-third of these recurrences were regional or associated with distant metastases (54 of 152 [35.5%]). Perianal EMPD cases recurred in one-third of cases (74/218 [33.9%]), with one-third of these recurrences being regional or associated with distant metastasis (20 of 74 [27.0%]). Perianal EMPD also had the highest rate of invasive disease (50% of cases).Conclusions and RelevanceThe diagnosis and treatment of EMPD should differ based on anatomic subtypes. Considerations for updated practice may include less morbid treatments for vulvar EMPD, which is primarily epidermal, and close surveillance for local recurrence in vulvar EMPD and metastatic recurrence in perianal EMPD. Recurrences in penoscrotal subtype were less common, and selective surveillance in this subtype may be considered. Limitations of this study include the lack of replication cohorts and the exclusion of studies that did not stratify outcomes by anatomic subtype.

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