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Penis and scrotum
Dysplasia / carcinoma in situ
Paget’s Disease of Penis
Editor: Antonio Cubilla, M.D. and Alcides Chaux, M.D. (see Author/Reviewers page)
Revised: 26 May 2010, last major update May 2010
Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.
See also Paget’s disease of scrotum
Definition
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● A form of primary or secondary intraepithelial adenocarcinoma
● ICD-O: 8542/3
Terminology
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● Extramammary Paget’s disease (EMPD): terminology used to distinguish from mammary Paget’s 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
Epidemiology
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● Ages 50’s and 60’s most common
Sites
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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
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● Flat, scaly, eczematous, red macular or plaque-like lesion
Prognostic factors
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● Presence of stromal invasion in primary EMPD is associated with more aggressive behavior
Case reports
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● 59 year old man with ulcer at base of shaft (Dermatology Online Journal 2008;14(10):15 )
● 87 year old man with plaque on shaft of penis (J Med Case Reports 2009 Jan 6;3:4)
Treatment
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● Wide local excision
Clinical images
Micro description (Histopathology)
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● 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
Micro images
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Note the typical, basally located, atypical cells with abundant vacuolated cytoplasm
Positive stains
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Primary EMPD
● CK7, MUC1, MUC5AC, CAM 5.2, EMA, CEA, GCDFP-15
Secondary EMPD
● MUC2
Negative stains
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Primary EMPD
● MUC2, MUC6, CK20
Secondary EMPD
● GCDFP-15
Differential Diagnosis
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● 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, 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
Additional references
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End of Penis and scrotum > Dysplasia / carcinoma in situ > Paget’s Disease
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