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Penis and scrotum
Neoplastic lesions of scrotum
Paget’s disease of scrotum
Editors: 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.
Definition
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● Adenocarcinoma in situ of scrotal skin
● ICD-O: 8542/3
Terminology
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● Primary extramammary Paget’s 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)
Epidemiology
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● Mean age 71 years (range 55-84 years)
Clinical features
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● 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
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● With prior squamous cell carcinoma in situ of scrotum (Archives 2004;128:84)
Treatment
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● Wide surgical excision; possibly radiation or chemotherapy
● Possibly photodynamic therapy for lesions < 4 cm (Acta Derm Venereol 2010;90:159)
Clinical images
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Ill-defined eczematous scaly lesion Red scaling skin
involves a large portion of the scrotal
skin. AFIP image courtesy of Dr. D.
Bell, Halifax, Nova Scotia, Canada
Gross description (Macroscopy)
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● Erythematous plaque or raised lesion on scrotal or penile skin
Micro description (Histopathology)
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● 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 2009 Jul 14 [Epub ahead of print])
Micro images
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Low power High power With Bowen’s disease and stains
Intraepidermal nests of large vacuolated cells are predominantly in basal epidermis (A).
Alcian Blue stain (B) and CEA (C) are positive.
CEA PAS EMA (site unspecified)
Positive stains
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Primary EMPD
● CK7, CAM 5.2, EMA, CEA
● Also PAS, MUC1, MUC5AC, mucicarmine, Alcian Blue, GCDFP-15 (Am J Surg Pathol 2001;25:1469)
Negative stains
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Primary EMPD
● 34betaE12 (high molecular weight keratin), MUC2, HMB45, MelanA/Mart1, S100
Differential Diagnosis
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● Superficial spreading melanoma: positive for melanocytic markers (HMB-45, Melan-A, S-100)
● Pagetoid Bowen’s disease: positive for 34betaE12, p63, negative for mucicarmine, CEA, CK7 and CK20
End of Penis and scrotum > Neoplastic lesions of scrotum > Paget’s disease of scrotum
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