Rare perianal extramammary Paget disease successfully treated using topical Imiquimod therapy.

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.

• Authors: Jéssica Silva Dos Santos, Gabriel Alves Bonafé, José Aires Pereira, Danilo Toshio Kanno, Carlos Augusto Real Martinez, Manoela Marques Ortega
• Journal: BMC cancer (BMC Cancer) Vol. 18 Issue 1 Pg. 921 (Sep 25 2018) ISSN: 1471-2407 [Electronic] England
• PMID: 30253739 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antineoplastic Agents
• Biomarkers, Tumor
• Imiquimod

Topics

• Administration, Topical
• Aged
• Antineoplastic Agents (administration & dosage)
• Anus Neoplasms (diagnosis, drug therapy)
• Biomarkers, Tumor
• Biopsy
• Humans
• Imiquimod (administration & dosage)
• Immunohistochemistry
• Male
• Paget Disease, Extramammary (diagnosis, drug therapy)
• Treatment Outcome