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

Moretto, Patrícia; Nair, Vimoj; Malone, Shawn; Belanger, Eric C.; Morash, Chris; Canil, Christina

doi:10.3747/co.20.1353

Cited by 34 publications

(29 citation statements)

References 67 publications

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“…Other treatment options such as carbon dioxide Laser vaporisation and photodynamic therapy can only be used after invasive disease is excluded 6 10. Radiotherapy treatment for penoscrotal EMPD lacks experience as it has only been used for vulvar disease 6 10.…”

Section: Discussionmentioning

confidence: 99%

A case of metastatic adenocarcinoma on a background of penoscrotal extramammary Paget's disease

et al. 2015

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A 77-year-old man presented with a chronic lesion located in the left penoscrotal area. Apart from pruritus, bleeding and an occasional discharge from this area, he also reported reduced appetite and weight loss. Examination revealed an ulcerated skin lesion attached to a firm subcutaneous mass. Wide local excision of the lesion revealed invasive adenocarcinoma on a background of extramammary Paget's disease. Staging studies showed disseminated metastatic disease within the lymph nodes, and liver and bone metastases. He was treated with carboplatin and paclitaxel chemotherapy initially, but then continued only on Carboplatin chemotherapy due to side effects from Paclitaxel. Eleven weeks after the start of his chemotherapy, his restaging imaging showed reduced lymphadenopathy, unchanged liver metastasis and sclerosis of bone metastasis. With completion of chemotherapy, repeat imaging showed stable disease. The patient is currently on follow-up.

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Section: Discussionmentioning

confidence: 99%

A case of metastatic adenocarcinoma on a background of penoscrotal extramammary Paget's disease

et al. 2015

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“…For this reason, adequate workup should include screening for metastatic malignancy after histologic diagnosis was established [6]. A computed tomographic scan of the abdomen and pelvis is recommended to detect any associated or metastatic abdominal or genitourinary cancers [3]. If lymph node metastases are detected clinically or histopathologically, complete ilioinguinal lymph node dissection is indicated [8].…”

Section: Answermentioning

confidence: 99%

“…Disease can present as carcinoma in situ or as invasive disease with metastasis to the lymph nodes and/or distant organs [3]. For this reason, adequate workup should include screening for metastatic malignancy after histologic diagnosis was established [6].…”

Section: Answermentioning

confidence: 99%

“…Screening with chest X-ray, colonoscopy, cystoscopy, and serum prostate-specific antigen levels is also performed by some physicians. Poor prognostic indicators include perianal disease, dermal invasion, and lymph node metastasis [3, 6]. …”

Section: Answermentioning

confidence: 99%

“…It is considered an adenocarcinoma of the apocrine glands of the axilla, vulva, perianal region, scrotum, and/or penis [3]. The pathogenesis of EMPD is highly debated; however, current theory suggests that primary EMPD arises as a primary neoplasm from the intraepidermal cells of the apocrine gland ducts or from pluripotent keratinocyte stem cells, while secondary EMPD represents the spread from an underlying visceral neoplasm of the bladder, urethra, prostate, and/or rectum to a dermal adnexal gland or a local organ with contiguous epithelium [4, 5].…”

Section: Answermentioning

confidence: 99%

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Enlarging Bump on the Scrotum

Cervantes

Rosen

Cho

2018

Skin Appendage Disord

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Extramammary Paget disease is a rare, slow-growing intraepithelial neoplasm of the skin or its underlying appendages. It more commonly affects the apocrine glands of the axilla, vulva, perianal region, scrotum, and/or penis. It often presents as a well-demarcated, thickened, erythematous, or gray-white scaly plaque with indolent growth that may become ulcerated, crusted, papillomatous, or eczematous over time. Pruritus, pain, and burning sensations are common symptoms; however, they are not appreciated by all patients. Many patients are erroneously treated for eczema for months to years before a proper diagnosis is reached. Patients presenting with chronic genital or perineal dermatitis or skin lesions that have been unresponsive to topical therapy should be biopsied for definitive diagnosis.

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

Cited by 34 publications

References 67 publications

A case of metastatic adenocarcinoma on a background of penoscrotal extramammary Paget's disease

A case of metastatic adenocarcinoma on a background of penoscrotal extramammary Paget's disease

Enlarging Bump on the Scrotum

Anatomic Subtype Differences in Extramammary Paget Disease

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