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

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International Journal of Colorectal Disease Aims and scope Submit manuscript

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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Acknowledgments

The study was supported by the Ed and Joey Story Endowed Chair in Colorectal Surgery. Preliminary results were presented to the Annual Meeting of the American Society of Colon and Rectal Surgeons, Hollywood, Florida, USA, May 2014.

Conflict of interest

The authors declare no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Colorectal Surgery, Digestive Disease Institute, Cleveland Clinic, 9500 Euclid Ave., Cleveland, OH, 44195, USA

    Ozgen Isik, Erman Aytac, Michael A. Valente, Maher A. Abbas & Emre Gorgun

  2. Department of Anatomic Pathology, Cleveland Clinic, Cleveland, OH, USA

    Jennifer Brainard

  3. Department of Colorectal Surgery, Digestive Disease Institute, Cleveland Clinic, Al Maryah Island, Abu Dhabi, United Arab Emirates

    Maher A. Abbas

Authors
  1. Ozgen Isik
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  2. Erman Aytac
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  3. Jennifer Brainard
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  4. Michael A. Valente
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  5. Maher A. Abbas
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  6. Emre Gorgun
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Corresponding author

Correspondence to Emre Gorgun.

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Isik, O., Aytac, E., Brainard, J. et al. Perianal Paget’s disease: three decades experience of a single institution. Int J Colorectal Dis 31, 29–34 (2016). https://doi.org/10.1007/s00384-015-2342-3

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  • DOI: https://doi.org/10.1007/s00384-015-2342-3

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