Penoscrotal Extramammary Paget's Disease: The University of Texas M. D. Anderson Cancer Center Contemporary Experience

Hegarty, Paul K.; Suh, Jane; Fisher, Mark B.; Taylor, Jennifer; Nguyen, Tri H.; Ivan, Doina; Prieto, Víctor G.; Pagliaro, Lance C.; Pettaway, Curtis A.

doi:10.1016/j.juro.2011.02.2685

Cited by 65 publications

(57 citation statements)

References 17 publications

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“…In a recent M.D. Anderson Cancer Centre report, the incidence of a second malignancy was 45% 31 , and in a European study, the overall standardized incidence ratio was 1.39 3 .…”

Section: Discussionmentioning

confidence: 92%

“…The literature also describes prs with 5-fluorouracil plus cisplatin 61 in a low-dose schedule (5-fluorouracil 500 mg daily, 7 days per week, and cisplatin 5 mg daily, 5 days per week), followed by oral tegafur uracil (600 mg daily) 62 . Complete radiologic response to docetaxel and carboplatin, with a disease-free survival of 13 months 31 , was reported for penoscrotal empd. Responses to docetaxel, weekly or every 3 weeks, alone or in combination, have also been reported in Japanese cases 63 , with complete response lasting 2 years 64 and pr for up to 12 months despite prior use of other agents (mitomycin C, epirubicin, vincristine, cisplatin, and 5-fluorouracil) 65 .…”

Section: 24mentioning

confidence: 99%

“…The M.D. Anderson group recommends at least 1-cm to 1.5-cm margins of grossly visible tumour, with re-resection according to frozen section results 31 . Wide local excision may be followed by reconstructive surgical procedures to obtain better cosmesis.…”

Section: 21mentioning

confidence: 99%

“…Dermal or lymph node invasion confers a worse prognosis 31 . In fact, in a multivariate analysis, a significant improvement in outcomes was observed for patients who had surgery compared with those who had no intervention (hazard ratio: 0.44), and the addition of radiotherapy to local surgery did not improve outcomes 6 .…”

Section: 21mentioning

confidence: 99%

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Management of Penoscrotal Extramammary Paget Disease: Case Series and Review of the Literature

et al. 2013

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The medical literature available to guide the management of empd is limited, particularly for metastatic disease. Another challenge is to differentiate metastatic empd from a concurrent invasive cancer. Here, we present our case series of 6 patients with empd of the penoscrotal region, our experience with systemic therapy, and a literature review for this rare malignancy. METHODSWe identified 6 cases of penoscrotal empd treated at The Ottawa Hospital from 2007 to 2012. All cases underwent pathology review. After research ethics board approval, clinical and demographic data, including age at presentation, presence of a second malignancy, management, and follow-up were collected from patient records. The literature was reviewed for clinical and pathologic features, treatment options, and outcomes. RESULTS 3

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“…In a recent M.D. Anderson Cancer Centre report, the incidence of a second malignancy was 45% 31 , and in a European study, the overall standardized incidence ratio was 1.39 3 .…”

Section: Discussionmentioning

confidence: 92%

Section: 24mentioning

confidence: 99%

Section: 21mentioning

confidence: 99%

Section: 21mentioning

confidence: 99%

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Management of Penoscrotal Extramammary Paget Disease: Case Series and Review of the Literature

et al. 2013

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“…Cases of EMPD with microscopic (< 1 mm) dermal invasion are proposed to have a more favourable prognosis than lesions showing deeper invasion or lymphovascular invasion [8]. [10]. The role of radiotherapy is not well defined in literature.…”

Section: Figure 1a: Pre Treatment Evaluationmentioning

confidence: 99%

Perianal Extramammary Paget’s Disease: Diagnosis, Treatment and Prognosis

Felice¹

2014

J Cytol Histol

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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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Penoscrotal Extramammary Paget's Disease: The University of Texas M. D. Anderson Cancer Center Contemporary Experience

Cited by 65 publications

References 17 publications

Management of Penoscrotal Extramammary Paget Disease: Case Series and Review of the Literature

Management of Penoscrotal Extramammary Paget Disease: Case Series and Review of the Literature

Perianal Extramammary Paget’s Disease: Diagnosis, Treatment and Prognosis

Anatomic Subtype Differences in Extramammary Paget Disease

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