Skin nonmelanocytic tumor
Adnexal tumors
Sweat gland derived (apocrine & eccrine glands)
Extramammary Paget disease


Topic Completed: 1 June 2012

Minor changes: 15 October 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Paget disease [title] extramammary

Christopher S. Hale, M.D.
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Cite this page: Hale CS. Extramammary Paget disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticpagets.html. Accessed October 26th, 2020.
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

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

Breast

Vulva

Vulva

Extramammary Extramammary Extramammary Extramammary

Extramammary

Extramammary Extramammary Extramammary

Extramammary

Dermal invasion Dermal invasion

Dermal invasion

GCDFP15

GCDFP15


PAS PAS

PAS



Images hosted on other servers:
Site unspecified

Site unspecified

Paget cells

Paget cells

GCDFP-15, CK7, CK20

GCDFP-15, CK7, CK20

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