Women

,

Incidence and Survival of Extramammary Paget’s Disease from the Surveillance, Epidemiology, and End Results (SEER) Database

,
Incidence and Survival of Extramammary Paget’s Disease from the Surveillance, Epidemiology, and End Results (SEER) Database

Extramammary Paget’s Disease (EMPD) is a rare intraepithelial neoplasm predominantly affecting the anogenital regions of older, female, and Asian patients. It often progresses insidiously, leading to diagnostic delays spanning years. This study, conducted through a retrospective review of EMPD patients from 22 SEER registries between 2004 and 2020, aimed to analyze contemporary trends in incidence and survival across sexes. The data included various demographics and clinical factors, and age-adjusted incidence rates were calculated over a 20-year period. Survival estimates were assessed using Kaplan-Meier curves, while Cox proportional hazards models were utilized to identify factors associated with all-cause mortality. The study included 3608 patients, with a higher incidence in Asian patients, who had a rate double that of white patients. Notably, the incidence increase was significant only among white patients. Females had a better ten-year survival rate (63.0%) compared to males (53.4%). Multivariable analysis revealed that older age, advanced disease stage, and treatment delays were linked to poorer overall survival, while primary surgical treatment significantly reduced mortality risk compared to no cancer-directed treatment. The study highlights the rising incidence of EMPD and emphasizes the importance of early, complete surgical resection in improving long-term survival outcomes.

Doença de Paget vulvar: uma série de casos no sul do Brasil

Doença de Paget vulvar: uma série de casos no sul do Brasil

A doença de Paget vulvar é rara e manifesta-se clinicamente como lesão cutânea eritematosa pruriginosa com áreas de hiperqueratose. O presente relatório descreve os dados de diagnóstico, manejo e resultados de uma série de casos de mulheres diagnosticadas com doença de Paget vulvar em um hospital terciário no sul do Brasil. A doença de Paget vulvar tem uma morbidade significativa e os dados disponíveis são limitados, especialmente no Brasil. Devido à raridade da doença, não há ensaios clínicos randomizados disponíveis na literatura e, portanto, é difícil comparar os resultados do tratamento cirúrgico com outras modalidades terapêuticas.

Mayo Clinic: Prospective Registry of Gynecologic Patients With Extramammary Paget's Disease Study

Mayo Clinic: Prospective Registry of Gynecologic Patients With Extramammary Paget's Disease Study

The Mayo Clinic (Rochester, MN, USA) is currently recruiting women with vulvar Extramammary Paget's Disease for an EMPD study. This includes EMPD of the vulva, vagina or perianal EMPD. The 25-year study will allow physicians to better understand EMPD. The researchers also want to learn more about the microorganisms (microbiome) that live on or near the areas of skin affected by EMPD, in order to better understand this condition. Tumor tissues will be obtained in order to perform tumor molecular profiling to identify targetable somatic mutations in EMPD. This EMPD study is for women only. More information on the Prospective Registry of Gynecologic Patients With Extramammary Paget's Disease study can be found here. 

Extramammary Paget disease of the vulva: immunohistochemical analysis of neoangiogenesis and epithelial-mesenchymal transition markers expression

Extramammary Paget disease of the vulva: immunohistochemical analysis of neoangiogenesis and epithelial-mesenchymal transition markers expression

Extra-mammary Paget’s disease of the vulva (EMPDV) is an infrequent chronic disease that often recurs. The aim of the study was to assess the presence of neoangiogenesis and the expression of epithelial-mesenchymal transition (EMT) markers in EMPDV, and their potential correlation with stromal invasion. All the women consecutively treated for EMPDV at our Institute, between January 2011 and December 2014, were studied for neoangiogenesis, analysed by microvessel density (MVD) using antibodies against CD31 and CD34. Immunohistochemical expression of E- / N-cadherin, β-catenin and SLUG was also evaluated. In each slide, three fields with the highest number of capillaries and small venules were identified at low power. In these three fields, the highest vessel density (HVD) and the average vessel density (AVD) at 200× and 400× magnification were counted. Immunohistochemical reactions for non-vascular markers were semiquantitatively scored by two pathologists, using a three-tier scale. Seventeen cases of EMPDV (including 10 cases of invasive disease) were included. The AVD at 200× and 400× and the HVD at 400× magnification were significantly associated with invasive EMPDV (p = 0.02, 0.03, 0.03 respectively). No significant correlation between MVD, EMT-markers expression and risk of recurrence was detected. These results indicate that MVD, as a measure of neoangiogenesis, may be associated with histological progression of EMPDV. EMT could also be linked to an invasive potential of EMPDV but larger series are required to confirm this hypothesis.

Interventions for the treatment of Paget’s disease of the vulva

Interventions for the treatment of Paget’s disease of the vulva

We found no reliable evidence to inform decisions about different interventions for women with Paget’s disease of the vulva. Ideally, a multicentre RCT of reasonable size is needed. In particular, evidence regarding the increasing use of imiquimod would be helpful to women and clinicians alike. Well-designed non-randomised studies, that use multivariate analysis to adjust for baseline imbalances, as well as other key methodological strengths, are also lacking.

© myEMPD.com • Helping those with extramammary Paget’s disease (EMPD).