Penis and scrotum
Neoplastic lesions of scrotum
Extramammary Paget disease

Topic Completed: 1 May 2010

Revised: 19 December 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Paget disease[TIAB] scrotum

See Also: Paget disease of penis

Alcides Chaux, M.D.
Antonio L. Cubilla, M.D.
Page views in 2019: 810
Page views in 2020 to date: 122
Cite this page: Chaux A, Cubilla AL. Extramammary Paget disease of scrotum. website. Accessed February 27th, 2020.
Definition / general
  • Adenocarcinoma in situ of scrotal skin
  • ICD-O: 8542 / 3
  • Primary extramammary Paget disease (EMPD): originates from apocrine ducts or sweat gland carcinoma (Ai Zheng 2009;28:879)
  • Secondary EMPD: intraepithelial spread from an underlying malignancy (colon, rectum, prostate)
  • Mean age 71 years (range 55 - 84 years)
Clinical features
  • Scrotal cases are usually not associated with underlying malignancy
  • Less frequent in scrotum than in vulva or perianal region
  • Metastatic disease is rare, associated with dermal and angiolymphatic invasion and high serum CEA (Br J Dermatol 2009;161:577)
Case reports
Clinical images

AFIP images

Ill defined eczematous scaly lesion

Images hosted on other servers:

Before (a), 6 months after photodynamic therapy (b)

Gross description
  • Erythematous plaque or raised lesion on scrotal or penile skin
Microscopic (histologic) description
  • Intraepidermal clusters of large cells with abundant pale granular or vacuolated cytoplasm and large vesicular nuclei with prominent nucleoli, predominantly located in basal layer
  • Single cells, nests and occasional glands may be present
  • May be associated with hyperkeratosis and parakeratosis
  • Frequent invasion of acrosyringeal adnexal structures
  • Invasion (focal or frank) into dermis is rare but may be present
  • May rarely mimic acantholytic squamous cell carcinoma in situ (J Cutan Pathol 2010;37:683)
Microscopic (histologic) images

AFIP images

Intraepidermal nests of large vacuolated cells are predominantly in basal epidermis (left); Alcian blue stain (middle) and CEA (right) are positive

Images hosted on other servers:

Low power

High power

With Bowen disease and stains



Decreased melanocytes,
slight fibrosis and
dilated capillary vessels
(c); after treatment (d)

Positive stains
Differential diagnosis
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