8540/3 Paget disease of breast

Related terms

Paget disease, mammary


ICD-O-3 topography code: C50
ICD10: C50

A breast cancer characterized by the presence of malignant glandular epithelial cells (Paget cells) within the squamous epithelium of the nipple that may extend into the areola and adjacent skin. It is usually associated with underlying carcinoma, which is mostly high-grade invasive carcinoma of no special type (53?60%) or ductal carcinoma in situ (DCIS) (24?43%) that can be central, peripheral or multicentric. Paget disease of the nipple (PD) without underlying carcinoma is rare, with a reported incidence of 1.4?13% of all PD cases 1
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Caliskan M, Gatti G, Sosnovskikh I, Rotmensz N, Botteri E, Musmeci S, Rosali dos Santos G, Viale G, Luini A (2008)
Paget's disease of the breast: the experience of the European Institute of Oncology and review of the literature.
Breast Cancer Res Treat 112: 513-21

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Chen CY, Sun LM, Anderson BO (2006)
Paget disease of the breast: changing patterns of incidence, clinical presentation, and treatment in the U.S.
Cancer 107: 1448-58

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Kothari AS, Beechey-Newman N, Hamed H, Fentiman IS, D'Arrigo C, Hanby AM, Ryder K (2002)
Paget disease of the nipple: a multifocal manifestation of higher-risk disease.
Cancer 95: 1-7

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Siponen E, Hukkinen K, Heikkilä P, Joensuu H, Leidenius M (2010)
Surgical treatment in Paget's disease of the breast.
Am J Surg 200: 241-6


ICD-O-3 topography code: C44
ICD10: C44

Mammary Paget disease (MPD) is an intraepidermal adenocarcinoma that is almost always associated with an in situ or invasive ductal carcinoma of the breast and represents the retrograde extension of the underlying carcinoma into the epidermis. Most cases of MPD are unilateral. Patients initially present with erythema of the nipple and areola. The lesion progresses to a scaly, crusted plaque and ultimately develops areas of erosion and ulceration. Advanced lesions may extend to the adjacent skin. In approximately 50% of cases, a breast mass is palpable; in these cases, >90% of the underlying carcinoma will be invasive. Advanced stages of lesions with a large underlying carcinoma may be associated with nipple retraction and serosanguinous discharge. In a relatively large percentage (10-28%) of patients the lesions do not display clinical features and are are detected only on histologic examination
Rosen P.P.
Paget's disease of the nipple. In: Rosen's Breast Pathology
2nd edition
Lippincott Williams & Wilkins: Philadelphia 2001

Histologically, the tumour is characterized by large, atypical neoplastic cells (Paget cells) featuring large nuclei with prominent nucleoli and abundant pale cytoplasm. They are scattered singly or in small clusters throughout the epidermis. The epidermis is often hyperkeratotic and acanthotic; the epidermal changes are accompanied by a dermal perivascular lymphohistiocytic infiltrate.
Mammary Paget disease affects almost exclusively women. The prognosis depends on the size and characteristics of the underlying carcinoma. Patients without a clinically detectable breast mass have a far better prognosis
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005