Penis & scrotum
Dysplasia/carcinoma in situ
Extramammary Paget disease


Topic Completed: 1 May 2010

Minor changes: 29 March 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Paget disease of penis scrotum

Alcides Chaux, M.D.
Antonio L. Cubilla, M.D.
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Cite this page: Chaux A, Cubilla AL. Extramammary Paget disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/penscrotumpenilepagets.html. Accessed July 5th, 2020.
Definition / general
Terminology
  • 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 (Ai Zheng 2009;28:879)
  • Secondary EMPD (10% of all cases): pagetoid intraepithelial spread from an underlying malignancy from prostate, bladder (J Cutan Pathol 2004;31:341), testes, ureter, kidney, colorectum
Epidemiology
  • Ages 50s and 60s most common
Sites
  • 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
    • Scrotal cases are usually not associated with underlying malignancy
    • Less frequent in scrotum than in vulva or perianal region
  • Secondary EMPD: affects glans and perimeatal region
  • Metastatic disease is rare, associated with dermal and angiolymphatic invasion and high serum CEA (Br J Dermatol 2009;161:577)
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
Treatment
Clinical images
Images hosted on PathOut server:

AFIP: ill defined eczematous scaly lesion



Images hosted on other servers:

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

Penile lesion

Gross description
  • Erythematous plaque or raised lesion on scrotal or penile skin
Microscopic (histologic) description
  • Intraepithelial proliferation of large, round, atypical cells with abundant pale mucin rich, granular or vacuolated cytoplasm (Paget cells)
  • Vesicular nuclei with prominent nucleoli
  • Paget cells found throughout the entire epithelium, although predominate in basal layers
  • Single cells, nests and occasional glands may be present
  • Paget cells frequently compress surrounding keratinocytes
  • Epithelium may be hyperplastic or atrophic
  • Mitoses may be numerous
  • Intracytoplasmic melanin occasionally found in Paget cel
  • 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

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

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
  • Secondary EMPD: MUC2
  • 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
    • Pagetoid Bowen disease: positive for 34betaE12, p63, negative for mucicarmine, CEA, CK7 and CK20
    • Superficial spreading melanoma: positive for melanocytic markers (HMB45, MelanA, S100)
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