Skin - Nonmelanocytic tumors
Premalignant or noninvasive
Paget's disease (extramammary)

Author: Christopher Hale, M.D. (see Authors page)

Revised: 13 October 2016, last major update June 2012

Copyright: (c) 2002-2016,, Inc.

PubMed Search: Paget's disease [title] extramammary

Cite this page: Paget's disease (extramammary). website. Accessed October 23rd, 2016.
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
  • Complete surgical excision
Clinical Images

Images hosted on PathOut server:

Courtesy of Mark R. Wick, M.D.

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Red firm mass

Gross Description
  • Erythematous, eczematous or ring-shaped
  • Often multicentric, extensive, pigmented
Micro 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 Pagetís cells and neighboring keratinocytes
  • No intercellular bridges, no dyskeratosis
Micro Images

Images hosted on PathOut server:
Images courtesy of Mark R. Wick, M.D.:





Dermal invasion



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Site unspecified

Paget cells

GCDFP-15, CK7, CK20

Positive Stains
Electron Microscopy Description
  • Cells with glandular differentiation, not melanocytes or keratinocytes
Differential Diagnosis