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Anus and perianal area


Pagetís disease

Reviewer: Nat Pernick, M.D. (see Reviewers page)
Revised: 16 April 2013, last major update April 2005
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.


● Intraepithelial tumor of apocrine glands
● Variable percentage (15-50%) of perianal Pagetís disease is associated with an underlying local malignancy
● May extend into anal canal to dentate line
● Tumors that are GCDFP15+, CK20- are associated with primary cutaneous intraepithelial malignancy with sweat gland differentiation
● Tumors that are MUC2+, CK20+ or GCDFP15- may have an underlying rectal adenocarcinoma (Am J Surg Pathol 2001;25:1469, Arch Pathol Lab Med 2001;125:1074, Arch Pathol Lab Med 1998;122:1077)
● Associated with squamous hyperplasia, fibroepithelioma-like hyperplasia and papillomatous hyperplasia (Am J Surg Pathol 2000;24:543)

Gross description

● Erythematous, ulcerated or eczematous

Micro description

● Usually single, occasionally nests or gland like formations of large, pale-staining to clear intraepidermal carcinoma cells with abundant mucin

Positive stains

● Mucin (mucicarmine, PAS), CK7, CEA, EMA; variable CK20

End of Anus and perianal area > Carcinoma > Pagetís disease

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