Extramammary Paget's

disease of the scrotum

Deba P Sarma, MD
Omaha
M 30, scrotal biopsy
 Comment
 Extramammary Paget's disease arises in the area rich in
apocrine sweat glands, such as vulva, perianal skin, penis,
scrotum and axilla.
 There is a female predominence.
It represents an in-situ carcinoma most likely derived from the
intraepidermal stem cells of apocrine lineage. In a minority of
cases it may be an intraepidermal spread from an underlying
sweat gland carcinoma, similar to that in Paget's disease of the
breast where the intraepidermal spread is secondary to an
underlying breast carcinoma.

 The epidermis is acanthotic with infiltration by large epithelial

cells with abundant pale blue or red cytoplasm containing large
nuclei. The infiltrating cells are present singly or in clusters
with occasional gland formation in all layers of the epidermis.
Tumor cells are usually positive with PAS-D, Mucicarmine, and
Alcian blue (pH 2.5).
Immunostains: Usually positive for CK7, EMA, GCDFP-15 and
MUC1.
Negative for S-100 protein, HMB-45, MART-1.

 Differential diagnosis: Pagetoid melanoma, Bowen's disease.