Dataset Information

Treatment of Extramammary Paget Disease and the Role of Reflectance Confocal Microscopy: A Prospective Study.

ABSTRACT:

Background

Extramammary Paget disease (EMPD) poses treatment challenges. Invasive and noninvasive treatment modalities exist with variable success reported. Reflectance confocal microscopy (RCM) is emerging as an adjuvant diagnostic tool.

Objective

To evaluate the treatment of EMPD patients and the role of RCM.

Methods

Prospective study. Demographic and tumor characteristics were recorded. Handheld-RCM was performed and correlated with histology. Treatment, clearance, pathology, and follow-up were all recorded.

Results

Thirty-six EMPD lesions in 33 patients were included. Mean age was 71.7 years, and 23 were men. Mean number of surgical stages needed to clear margins was 1.9 (SD, 0.9; 1.0-3.0 stages), and mean margin needed to clear was 1.8 cm. Reflectance confocal microscopy correlated well with scouting punch biopsies (kappa, 0.93; p < .001). Disruption of the dermoepidermal junction was associated with invasive EMPD versus in situ (83.3% vs 25.9%) on histology (p = .01).

Limitations

Relatively small sample size.

Conclusion

Extramammary Paget disease is challenging, and lesion demarcation is of the utmost importance. Using a staged surgical excision approach, the mean margins needed were 1.8 cm, less than previously reported. Nonsurgical modalities, including radiation therapy, imiquimod, or photodynamic therapy can be considered if surgery is not pursued. Reflectance confocal microscopy is a valuable noninvasive imaging modality for the management of EMPD.

SUBMITTER: Navarrete-Dechent C

PROVIDER: S-EPMC10599152 | biostudies-literature | 2021 Apr

REPOSITORIES: biostudies-literature

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Publications

Treatment of Extramammary Paget Disease and the Role of Reflectance Confocal Microscopy: A Prospective Study.

Navarrete-Dechent Cristian C Aleissa Saud S Cordova Miguel M Hibler Brian P BP Erlendsson Andres M AM Polansky Max M Cordova Frank F Lee Erica H EH Busam Klaus J KJ Hollmann Travis T Lezcano Cecilia C Moy Andrea A Pulitzer Melissa M Leitao Mario M MM Rossi Anthony M AM

Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] 20210401 4

<h4>Background</h4>Extramammary Paget disease (EMPD) poses treatment challenges. Invasive and noninvasive treatment modalities exist with variable success reported. Reflectance confocal microscopy (RCM) is emerging as an adjuvant diagnostic tool.<h4>Objective</h4>To evaluate the treatment of EMPD patients and the role of RCM.<h4>Methods</h4>Prospective study. Demographic and tumor characteristics were recorded. Handheld-RCM was performed and correlated with histology. Treatment, clearance, patho ...[more]

PMID: 33625139

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Project description: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.

Project description:IntroductionThe treatment of genital lichen sclerosus (GLS) remains challenging. Baricitinib has been introduced in the treatment of GLS, but there's no imaging evaluation for GLS patients treated with it. No comparison of dermoscopy and reflectance confocal microscopy (RCM) assessments in GLS has been conducted. We performed this study to evaluate the efficacy and safety of baricitinib for GLS and to compare the value of dermoscopy and RCM assessments in GLS.MethodsParticipants were treated with baricitinib for 6 months and assessed at week 0, 2, 4, 6, 8, and every 4 weeks for the next 16 weeks. All patients were evaluated for clinical, dermoscopic, and RCM variables, with numeric scores assigned to each parameter.ResultsTwenty-six GLS patients were included in this study. All patients achieved Investigator's Global Assessment score ≤ 1 (with ≥ 2-grade improvement) at week 20. The scores of pruritus and pain decreased since week 2 (both P < 0.05). The DLQI and VQLI scores significantly decreased since week 4 (both P < 0.0001). White structureless areas improved at week 2 and white shiny streaks and follicular plugs improved at week 4 under dermoscopic examination. Vessels (P < 0.001) and brown structureless areas (P = 0.003) increased at week 8. In RCM, inflammatory cells count significantly decreased at week 2 (100.03 ± 33.24, P < 0.0001), with substantial regression at week 8 (16.98 ± 5.54, P < 0.0001). Epidermal thickness increased at week 12 (157.44 ± 37.87 μm versus 134.13 ± 36.60 μm, P = 0.0284). Irregular papillae, spongiosis, and fiber structures improved at week 20, week 4, and week 6 (all P < 0.01). Transient hypercholesterolemia (11.54%), thrombocytosis (7.69%), and elevated alanine aminotransferase (7.69%) occurred during treatment.ConclusionBoth dermoscopy and RCM can be useful and non-invasive adjuvant tools for the evaluation and therapeutic monitoring of GLS. We recommended white structureless areas under dermoscopy and inflammatory cells count under RCM as variables for dermatologic imaging evaluation for GLS. Baricitinib is effective and safe for GLS, while randomized controlled trials are warranted.

Dataset Information

Treatment of Extramammary Paget Disease and the Role of Reflectance Confocal Microscopy: A Prospective Study.

Background

Objective

Methods

Results

Limitations

Conclusion

Publications

Treatment of Extramammary Paget Disease and the Role of Reflectance Confocal Microscopy: A Prospective Study.

Similar Datasets

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