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WHO Classification of Tumours
Paget disease, extramammary
Anus and anal canal


Definition

Extramammary Paget disease usually affects sites with a high density of apocrine glands, such as the anogenital region, where it presents as a slowly spreading, erythematous eczematoid plaque that may extend up to the dentate line
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Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM (1997)
Paget's disease of the perianal region--an aggressive disease?
Dis Colon Rectum 40: 1187-94



. Histologically, the basal part or whole thickness of the squamous epithelium is infiltrated by large cells with abundant pale cytoplasm and large nuclei. Occasional cells have the appearance of signet rings. Paget cells invariably react positively for mucin stains and nearly always for keratin 7, but Merkel cells and Toker cells may also be positive for the latter.

Paget disease of the anus appears to represent two entities:
> About half of the cases are associated with a synchronous or metachronous malignancy, most often a colorectal adenocarcinoma. Such cases can be regarded as a pagetoid extension of the tumour. They usually react positively for keratin 20 and negatively for gross cystic-disease fluid protein-15 (GCDFP-15), a marker for apocrine cells.
> This is in contrast to the other half of the cases, which are not associated with internal malignancies but have a high rate of local recurrence and may become invasive

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Marchesa P, Fazio VW, Oliart S, Goldblum JR, Lavery IC, Milsom JW (1997)
Long-term outcome of patients with perianal Paget's disease.
Ann Surg Oncol 4: 475-80



. Only the latter entity can be regarded as a true epidermotrophic apocrine neoplasm
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Armitage NC, Jass JR, Richman PI, Thomson JP, Phillips RK (1989)
Paget's disease of the anus: a clinicopathological study.
Br J Surg 76: 60-3




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Battles OE, Page DL, Johnson JE (1997)
Cytokeratins, CEA, and mucin histochemistry in the diagnosis and characterization of extramammary Paget's disease.
Am J Clin Pathol 108: 6-12




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Goldblum JR, Hart WR (1998)
Perianal Paget's disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma.
Am J Surg Pathol 22: 170-9




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Nowak MA, Guerriere-Kovach P, Pathan A, Campbell TE, Deppisch LM (1998)
Perianal Paget's disease: distinguishing primary and secondary lesions using immunohistochemical studies including gross cystic disease fluid protein-15 and cytokeratin 20 expression.
Arch Pathol Lab Med 122: 1077-81



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Recently, it has been proposed that this disease arises from adnexal stem cells residing in the infundibulo-sebaceous unit of hair follicles and adnexal structures, on the basis of expression of keratins 15 and 19

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Regauer S (2006)
Extramammary Paget's disease--a proliferation of adnexal origin?
Histopathology 48: 723-9



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Topographic definition of the anal canal, anal margin and perianal region