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BMC Cancer
Published in: BMC Cancer 1/2018

Open Access 01-12-2018 | Case report

Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy

Authors: Jéssica Silva dos Santos, Gabriel Alves Bonafé, José Aires Pereira, Danilo Toshio Kanno, Carlos Augusto Real Martinez, Manoela Marques Ortega

Published in: BMC Cancer | Issue 1/2018

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Abstract

Background

Perianal Paget’s disease (PPD) is a rare intraepithelial adenocarcinoma of the anal margin. Primary PPD likely represents intra-epithelial neoplasm from an apocrine source, whereas secondary disease may represent “pagetoid” spread from an anorectal malignancy.

Case presentation

Histologic CDX-2 and CK20 are hallmark markers for colorectal-derived Paget’s cells. Interestingly, our primary PPD patient presented both positive and no internal malignancy was identified. In addition, a negative CK7 marker was observed in our case in contrast with previously reported. Surgical excision is the standard treatment; however, previous studies have demonstrated good response with Imiquimod 5% cream in patients with vulval extramammary Paget disease (EMPD). The efficiency of Imiquimod treatment for PPD has not been well described. Our PPD patient was successfully treated using Imiquimod 5% cream.

Conclusions

This study describes a primary cutaneous PPD patient CDX-2+/CK20+/CK7- without invasion of the dermis and no associated colorectal carcinoma effectively treated using topical Imiquimod therapy, suggesting that Imiquimod might potentially be considered as a first-line treatment for PPD.
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Metadata
Title
Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy
Authors
Jéssica Silva dos Santos
Gabriel Alves Bonafé
José Aires Pereira
Danilo Toshio Kanno
Carlos Augusto Real Martinez
Manoela Marques Ortega
Publication date
01-12-2018
Publisher
BioMed Central
Published in
BMC Cancer / Issue 1/2018
Electronic ISSN: 1471-2407
DOI
https://doi.org/10.1186/s12885-018-4815-6

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