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Perianal Paget’s disease: presentation of six cases and literature review

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International Journal of Colorectal Disease Aims and scope Submit manuscript

Abstract

Purpose

Extramammary Paget’s disease (EMPD) is frequently associated with adnexal or visceral synchronous or metachronous malignancies. Our purpose was to evaluate, retrospectively, the results obtained in six cases of EMPD and to review the literature.

Methods

Six patients with the perianal Paget’s disease had been treated in our division between March 1996 and December 2006. In three cases, the disease was confined in the epidermis; in one case, there was a microinvasion of the dermis, while in another one the dermis was infiltrated. The last case was associated to a low rectal adenocarcinoma. All patients underwent wide perianal excision and reconstruction with skin graft. We performed a transanal resection of the rectal adenocarcinoma. A review of the literature from 1990 to 2008 revealed 193 cases of perianal EMPD, 112 were intraepithelial/intradermal while 81 were associated with malignancies. Anorectal adenocarcinoma was already existing in two cases, synchronous in 48, and subsequent to diagnosis in 11.

Results

In three cases, the disease recurred locally, but no patient developed metastatic spread. Five patients survived and are free of disease. The review of the literature allows a clear identification of the primitive EMPD and the form associated to anorectal adenocarcinoma and little information about cases associated with synchronous adnexal adenocarcinoma. The Paget’s disease can relapse after radical surgery and has a capacity of metastatic spread.

Conclusions

Up to now, no clear guidelines have been established for the diagnosis of EMPD. The association with synchronous or metachronous carcinomas imposes a long-term follow-up with frequent clinical, radiological, and endoscopical controls.

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Authors and Affiliations

  1. II Scuola di Specializzazione in Chirurgia Generale, I Divisione Clinicizzata di Chirurgia Generale, Ospedale Civile Maggiore, Università degli Studi di Verona, P.le A. Stefani, 1, 37126, Verona, Italy

    Annamaria Minicozzi, Giuseppe Borzellino, Rostand Momo, Francesca Steccanella, Federica Pitoni & Giovanni de Manzoni

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  1. Annamaria Minicozzi
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  2. Giuseppe Borzellino
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  3. Rostand Momo
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Correspondence to Annamaria Minicozzi.

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Minicozzi, A., Borzellino, G., Momo, R. et al. Perianal Paget’s disease: presentation of six cases and literature review. Int J Colorectal Dis 25, 1–7 (2010). https://doi.org/10.1007/s00384-009-0797-9

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