The case of an extensive primary extramammary Paget's disease diagnosis and treatment

Sarivalasis, Apostolos; Triboulet, Cécile; Anchisi, Sandro

doi:10.5348/ijcri-201705-cr-10744

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2019

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“…5 The disease has a predilection for postmenopausal white women, and clinical signs include eczematous eruptions in glandular tissue and associated pruritus. 7 The presence of Paget's cells is considered a premalignant condition, progressing to invasive adenocarcinoma within or below the superficial lesion in 4%-17% of cases. As for synchronous cancers, approximately 20%-30% of patients have non-contiguous carcinomas involving the breast, rectum, bladder, urethra, vagina, or ovary.…”

Section: Discussionmentioning

confidence: 99%

Perianal Paget's disease

Cassol

Pastorello

Oliveira

et al. 2019

Journal of Coloproctology

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Paget'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.

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Section: Discussionmentioning

confidence: 99%