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A Journal on Surgery

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Chirurgia 2001 June-August;14(3-4):157-60

language: English

Extramammary Paget's disease of the perianal region complicated with a malignant degeneration of an eccrine poroma. A case report

Guzzetti T., Scevola S., Thione A., Savani A.


Perianal (extramammary) Paget's disease, first described in 1893, is rare and probably corresponds to an intraepithelial adenocarcinoma arising from dermal apocrine sweat glands. Diagnosis is based on the findings of Paget's cells in the bioptic specimen. Its high risk of association with a visceral internal carcinoma demands, besides a local radical treatment, a strict surveillance to early diagnose any further malignancy occurrence. The report about an association between a perianal extramammary Paget's disease and a malignant degeneration of an eccrine poroma is even more interesting. A radical surgery approach is very important, such as an immediate and accurate instrumental investigation, to exclude an overlyng gastrointestinal neoplasm. A long term follow-up includes proctosigmoidoscopy, double contrast barium enema, biopsy once a year and a strict clinical evaluation. Our report is about this rare association between the extramammary Paget's disease of the perianal region and an eccrine poroma.

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