Skin nonmelanocytic tumor
Adnexal tumors - eccrine sweat glands
Extramammary Paget disease


Topic Completed: 1 June 2012

Revised: 10 April 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Paget disease [title] extramammary
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Cite this page: Hale CS. Extramammary Paget disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumornonmelanocyticpagets.html. Accessed April 25th, 2019.
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
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.

Breast

Vulva

Extramammary



Extramammary

Dermal invasion



GCDFP15

PAS



Images hosted on other servers:

Site unspecified

Paget cells

GCDFP-15, CK7, CK20

Positive stains
Electron microscopy description
  • Cells with glandular differentiation, not melanocytes or keratinocytes
Differential diagnosis
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