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01.12.2018 | Extramammäre Paget Erkrankung | Case report | Ausgabe 1/2018 Open Access

BMC Cancer 1/2018

Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy

Zeitschrift:
BMC Cancer > Ausgabe 1/2018
Autoren:
Jéssica Silva dos Santos, Gabriel Alves Bonafé, José Aires Pereira, Danilo Toshio Kanno, Carlos Augusto Real Martinez, Manoela Marques Ortega

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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