Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy

BMC Cancer

卷:18

Carlos Augusto Real Martinez¹  José Aires Pereira¹  Danilo Toshio Kanno¹  Jéssica Silva dos Santos²  Gabriel Alves Bonafé²  Manoela Marques Ortega² 

[1] Department of Surgery and Proctology, São Francisco University (USF);

[2] Laboratory of Cell and Molecular Tumor Biology and Bioactive Compounds, Department of Postgraduate Program in Health Science, São Francisco University (USF);

关键词: Perianal Paget’s disease;    Histological markers;    Differential diagnosis;    Topical Imiquimod therapy;   

DOI  :  10.1186/s12885-018-4815-6

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown