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Erschienen in: International Journal of Colorectal Disease 1/2016

01.01.2016 | Original Article

Perianal Paget’s disease: three decades experience of a single institution

verfasst von: Ozgen Isik, Erman Aytac, Jennifer Brainard, Michael A. Valente, Maher A. Abbas, Emre Gorgun

Erschienen in: International Journal of Colorectal Disease | Ausgabe 1/2016

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Abstract

Purpose

Perianal Paget’s disease is a rare intraepithelial adenocarcinoma of the perianal skin and the second most common localization of extramammary Paget’s disease. This study was designed to evaluate long-term outcomes in patients with perianal Paget’s disease.

Methods

We identified patients who were treated for perianal Paget’s disease between 1981 and 2013. Patient demographics, family history, associated malignancies, treatments, histopathological features, need for re-operations, and long-term outcomes were documented.

Results

Our study cohort consisted of 15 male and 10 female patients with a median age of 67 (40–83) years. Four patients had concurrent anorectal adenocarcinoma (two anal canal, two rectal) when perianal Paget’s disease was diagnosed. Index operations performed were wide local excision (14 patients), local excision (five patients), abdominoperineal resection (four patients), and radiotherapy (two patients). Five patients developed invasive carcinoma (three anal canal, one vulvar, one perianal squamous cell carcinoma) during a median follow-up time of 60 (3–299) months. Thirteen patients were re-operated. Fifteen patients had a reconstructive procedure following excision. Overall survival was similar between the patients who were treated with wide local excision and local excision regardless of surgical margin status at index excision (P = 0.75).

Conclusions

Since there is a risk for developing invasive carcinoma in the anal canal and perineum in patients with perianal Paget’s disease, close follow-up is needed after R0 or R1 excision. Physicians should be aware of the risk of cancers associated with perianal Paget’s disease and should rule out them during management.
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Metadaten
Titel
Perianal Paget’s disease: three decades experience of a single institution
verfasst von
Ozgen Isik
Erman Aytac
Jennifer Brainard
Michael A. Valente
Maher A. Abbas
Emre Gorgun
Publikationsdatum
01.01.2016
Verlag
Springer Berlin Heidelberg
Erschienen in
International Journal of Colorectal Disease / Ausgabe 1/2016
Print ISSN: 0179-1958
Elektronische ISSN: 1432-1262
DOI
https://doi.org/10.1007/s00384-015-2342-3

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