|
Paget's Disease of the Scrotum |
|
The Key Features |
-
Foreign cells in the epidermis;
-
PAS-D +, mucicarmine +;
-
Intra-epidermal apocrine origin:
GCDFP-15+, CK7+, CEA+, EMA+;
-
Association with underlying adenocarcinoma: especially CK20 or CA19-9+.
|
|
|
CLINICAL FEATURES |
| |
- Eczematoid, red, pruritic, slow-growing;
- Extramammary locations: axilla, labia majora, scrotum,
perineum;
|
|
GROSS FINDINGS |
| |
- Circinate, annular, erythematous, eczematoid plaque
|
|
MICROSCOPIC FINDINGS |
| |
- Foreign epithelioid cells in the epidermis just above the
basal layer; in single, in rows, small nests/clusters, or
gladular;
- Often see a cleft-like separation between the Paget's cells
and the adjacent normal epithelial cells;
- Tumor cells are round or oval with pale, amphophilic
cytoplasm that is commonly vacuolated;
- Nuclei contain dispersed or vesicular chromatin;
- The involved epidermis commonly exhibits hyperkeratosis and
acanthosis that mimics SCC.
|
|
DIFFERENTIAL DIAGNOSES |
| |
- Superficial spreading melanoma: S100+;
- Bowenoid intraepidermal SCC: CK7-.
|
|
IMMUNOHISTOCHEMISTRY AND SPECIAL STAINS |
| |
- Intra-epidermal apocrine origin: GCDFP-15 +, CK7+, CEA+, EMA;
- If CA19-9 or CK20+, should rule out association with
adenocarcinoma in the rectum, urinary bladder, or endocervix;
- Positive for PAS-D, mucicarmine and Alcian blue in 50-60%
cases;
|
|
TREATMENT AND PROGNOSIS |
| |
- Surgical resection but may be very difficult.
|
|
REFERENCES |
| |
- AFIP, nonmelanocytic tumors of the skin, series 4;
- Rosai and Ackerman's Surgical Pathology, 9th edition.
|
