Management of perianal extramammary Paget’s disease involving the dentate line without abdominoperineal resection

Lo, Oswens Siu-Hung; Li, G. K.-H.; Law, Wai Lun

doi:10.1007/s10151-016-1429-3

Cited by 3 publications

(2 citation statements)

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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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Section: Methodsmentioning

confidence: 99%

Anatomic Subtype Differences in Extramammary Paget Disease

Kibbi,

Owen,

Worley

et al. 2024

JAMA Dermatol

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“…20.8). It is estimated that 6% to 20% of all cases of extramammary Paget's disease occur in the perianal region [95][96][97]. It is more common in women with incidence rising starting in the fifth decade [98].…”

Section: Perianal Paget's Disease (Intraepithelial Adenocarcinoma)mentioning

confidence: 99%

Anal Cancer

Sands

Mahmoud

2021

The ASCRS Textbook of Colon and Rectal Surgery

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Tumors of the anal region are divided into anal and perianal cancers with different paths of lymphatic drainage.• Squamous cell carcinoma (SCC) is the most common type of anal cancer. • Chemoradiotherapy is the mainstay for anal (SCCa). The standard chemotherapy consists of 5-FU and mitomycin. The minimum dose of radiation is 45 Gy to the primary tumor. • Inguinal node metastasis can be diagnosed with PET-CT and managed with radiation. • Surgery for anal canal cancer is limited to very small lesions and salvage situations following failed chemoradiotherapy. • Anal adenocarcinoma can arise from an anal gland or chronic fistula tract. They can be difficult to distinguish from distal rectal cancers. These tumors are staged and treated similar to rectal cancers with a lower overall survival. • Verrucous carcinomas are characterized by large size and lack of invasion. Treatment is mainly wide surgical excision. • Anal melanoma is a rare and aggressive tumor. Survival is very poor. Abdominoperineal resection may help to control local disease but does not prolong life expectancy; therefore, local excision is often a first choice when possible. • Perianal Paget's disease -or intraepithelial adenocarcinoma -is frequently associated with other malignancies.Treatment is focused on surgical excision and may require mapping biopsies to plan resection.• Basal cell carcinoma of the perianal region is treated with wide local excision. • Gastrointestinal stromal tumors can be managed with wide local excision or radical excision depending on margin status. Preoperative treatment with tyrosine kinase inhibitors may enhance resectability.

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Pruritus Ani

Davis

2018

Fundamentals of Anorectal Surgery

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Management of perianal extramammary Paget’s disease involving the dentate line without abdominoperineal resection

Cited by 3 publications

References 5 publications

Anatomic Subtype Differences in Extramammary Paget Disease

Anatomic Subtype Differences in Extramammary Paget Disease

Anal Cancer

Pruritus Ani

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