Perianal Paget's disease

Author:

Cassol Ornella Sari12ORCID,Pastorello Júlia23,Oliveira Bruna de4,Lenhardt Lucas Henrique4,Montagna Suéllen4,Pietrobelli Vanessa Locatelli4

Affiliation:

1. Hospital da Cidade de Passo Fundo (HCPF), Departamento de Coloproctologia, Passo Fundo, RS, Brazil

2. Instituto Meridional (IMED), Campus Passo Fundo, Passo Fundo, RS, Brazil

3. Hospital da Cidade de Passo Fundo (HCPF), Departamento de Oncologia Clínica, Passo Fundo, RS, Brazil

4. Universidade Federal da Fronteira Sul (UFFS), Faculdade de Medicina, Passo Fundo, RS, Brazil

Abstract

AbstractPaget's disease most commonly affects the breast. Extramammary involvement is rare, and the most commonly affected sites are the vulva, anus, perianal region, and axilla. The disease may progress to invasive adenocarcinoma or synchronous cancers. Due to the lack of distinctive features and nonspecific presenting symptoms, Paget's disease may be misdiagnosed as other conditions, thus delaying the correct diagnosis. We report a case of extramammary Paget's disease in the perianal region that initially presented as an irregular, circumferential, scaling lesion with eczematous eruptions. Immunology and immunohistochemistry confirmed the diagnosis. Although surgery is the standard treatment, the patient opted for pelvic radiotherapy associated with radiosensitizing chemotherapy.

Publisher

Georg Thieme Verlag KG

Subject

Gastroenterology

Reference8 articles.

1. Doença de Paget perianal: a propósito de um caso clínico;Gandra-d’Almeida;Rev Port Cir,2016

2. Extramammary Paget's disease;Mehrtens;N Engl J Med,2017

3. Perineal Paget's disease: a rare disorder and review of literature;Carbotta;Ann Med Surg (Lond),2016

4. Extramammary Paget's disease;Shepherd;BJOG,2005

5. Perianal Paget's disease: three decades experience of a single institution;Isik;Int J Colorectal Dis,2016

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