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Skin - Nonmelanocytic tumors

Premalignant or noninvasive

Pagetís disease (extramammary)


Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 4 July 2012, last major update June 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● May originate from intraepidermal portion of sweat glands or primitive basal cells with ability to differentiation towards glandular elements
● Labia majora, scrotum and perineum are most common sites
● Due to underlying carcinoma in 10-20% of cases of vulvar disease

Treatment:
● Complete surgical excision

Case reports
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● 66 year old woman with vulvar Paget disease (BMC Cancer 2010;10:405)

Clinical images
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Various images

Gross description
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● Erythematous, eczematous or ring-shaped
● Often multicentric, extensive, pigmented

Micro description
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● Malignant cells in epidermis with differentiation towards local glandular structures
● Almost always simultaneous involvement of eccrine glands or hair follicles
● Dermal invasion is rare in vulva, more common in perianal region)
● Single, clusters or glandular formations of large cells with pale, vacuolated cytoplasm, usually just above basal layer of epidermis
● May have cleft-like spaces between Pagetís cells and neighboring keratinocytes
● No intercellular bridges, no dyskeratosis

Micro images
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Various images

Positive stains
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● Mucin, EMA, CEA, PAS, low molecular weight cytokeratin (CAM 5.2, CK7)
Vulva - GCDFP-15 and androgen receptors
Perianal - CK20

Electron microscopy description
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● Cells with glandular differentiation, not melanocytes or keratinocytes

Differential diagnosis
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● Pagetoid Bowenís disease
● Pagetoid actinic keratosis
● Melanocytic lesions
● Metastatic epidermotropic carcinomas
● Clear cell papulosis

End of Skin - Nonmelanocytic tumors > Premalignant or noninvasive > Pagetís disease (extramammary)


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