Home > Skin nonmelanocytic tumor > Extramammary Paget disease

Skin nonmelanocytic tumor

Adnexal tumors

Sweat gland derived (apocrine & eccrine glands)

Extramammary Paget disease

Author: Christopher S. Hale, M.D.

Last author update: 1 June 2012

Last staff update: 15 April 2024 (update in progress)

Copyright: 2002-2024, PathologyOutlines.com, Inc.

PubMed Search: Paget disease [title] extramammary

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Table of Contents

Cite this page: Hale CS. Extramammary Paget disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticpagets.html. Accessed April 16th, 2024.

Definition / general

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

Case reports

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

Treatment

Complete surgical excision

Clinical images

Contributed by Mark R. Wick, M.D.

Images hosted on other servers:

Red firm mass

Gross description

Erythematous, eczematous or ring shaped
Often multicentric, extensive, pigmented

Microscopic (histologic) description

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 Paget cells and neighboring keratinocytes
No intercellular bridges, no dyskeratosis

Microscopic (histologic) images

Contributed by Mark R. Wick, M.D.