Penis and scrotum
Dysplasia / carcinoma in situ
Extramammary Paget disease of penis

Topic Completed: 1 May 2010

Revised: 13 December 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Paget disease of penis

See Also: Paget disease of scrotum

Alcides Chaux, M.D.
Antonio L. Cubilla, M.D.
Page views in 2019: 931
Page views in 2020 to date: 60
Cite this page: Chaux A, Cubilla AL. Extramammary Paget disease of penis. website. Accessed January 26th, 2020.
Definition / general
  • Extramammary Paget disease (EMPD): terminology used to distinguish from mammary Paget disease, which has distinctive clinicopathologic features
  • Primary EMPD (90% of all cases): intraepithelial mucin secreting adenocarcinoma originates from intraepithelial extension of a sweat gland carcinoma or from intraepithelial sweat gland ducts
  • Secondary EMPD (10% of all cases): pagetoid intraepithelial spread from an underlying malignancy from prostate, bladder (J Cutan Pathol 2004;31:341), testes, ureter or kidney
  • Ages 50s and 60s most common
  • Primary EMPD: usually affects the scrotum and only rarely is restricted to the penis; frequently affects the skin of the shaft as part of a more disseminated lesion
  • Secondary EMPD: affects glans and perimeatal region
Clinical features
  • Flat, scaly, eczematous, red macular or plaque-like lesion
Prognostic factors
  • Presence of stromal invasion in primary EMPD is associated with more aggressive behavior
Case reports
  • Wide local excision
Clinical images

Images hosted on other servers:

Penile lesion

Microscopic (histologic) description
  • Intraepithelial proliferation of large, round, atypical cells with abundant pale mucin rich cytoplasm (Paget cells)
  • Vesicular nuclei with prominent nucleoli
  • Paget cells found throughout the entire epithelium, although predominate in basal layers
  • Paget cells frequently compress surrounding keratinocytes
  • Gland-like structures occasionally seen
  • Epithelium may be hyperplastic or atrophic
  • Mitoses may be numerous
  • Intracytoplasmic melanin occasionally found in Paget cells
Microscopic (histologic) images


Note the typical, basally
located, atypical cells
with abundant
vacuolated cytoplasm

Images hosted on other servers:

Note the typical, basally located, atypical cells with abundant vacuolated cytoplasm

Occasional cells near the margin

Prominent intraepidermal pagetoid spread

Positive stains
Negative stains
Differential diagnosis
  • Clear cell papulosis: pubic area of Asian individuals, multiple small white papules along the milk line and lower abdomen, basally located clear cells with bland cytology
  • Clear cell urothelial carcinoma in situ: glans / perimeatal area, concurrent / previous history of urothelial carcinoma elsewhere, more typical areas of urothelial carcinoma in situ elsewhere and positive for urothelial markers (uroplakin III, thrombomodulin)
  • Melanoma: positive for melanocytic markers (HMB45, MelanA, S100)
  • Mucinous metaplasia: inner foreskin mucosa of elderly patients, associated with inflammatory conditions, goblet-like cells in upper layers, positive for PAS, CAM 5.2, CEA and EMA
  • Pagetoid dyskeratosis: inner foreskin of patients with phimosis, clear cells with bland cytology, upper layer location, intercellular bridges between clear cells and keratinocytes, low mitotic rate, negative for PAS, EMA, CK7 and CEA
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