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2008, Number 1

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Med Cutan Iber Lat Am 2008; 36 (1)

Mammary Paget’s disease

Casimiro L, Vilata CJJ, Zaragoza NV

Language: Spanish
References: 95
Page: 3-12
PDF size: 305.23 Kb.


ABSTRACT

Mammary Paget´s disease is an uncommon condition that is associated with an underlying breast carcinoma in up to 100% of the cases. The clinical entity was first described by Velpeau in 1856 but the relationship with an underlying breast cancer was recognized by Sir James Paget in 1874.
Two main hypotheses have tried to explain it´s nature and origin, the epidermotropic theory is the most supported one, and the second theory is the in situ malignat transformation from existing cells.
Paget´s disease presents as a distinct changes in the nipple and areola of the involved breast like eczema, ulceration or retraction. Approximately half of the patients will have a palpable underlying breast mass, and bilateral lesions are exceptional.
Paget´s cells, are large round intraepidermal cells with abundant clear pale cytoplasm and enlarged pleomorfic an hypercromatic nuclei, which do not form intercellular bridges. Their citoplasm are PAS and CEA positive.
To confirm the diagnosis we have to make a full thickness biopsy of the nipple and areola with confirmatory stains and we can complete the study with different imaging metods like mammography or ultrasound examination. The differential diagnosis may include chronic eczema, basal cell carcinoma, or malignant melanoma. Prognosis depends on the presence or not of a palpable mass and lymph node involvement.
The tradicional treatment has been mastectomy with axillary lymph node dissection but now physicians are questioning the need for modified radical mastectomy in cases of Paget´s disease of the breast without an associated breast cancer.


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