What is Paget’s disease of the nipple

• eczematous change in skin of nipple due to ductal adenocarcinoma infiltrating into the epidermis of nipple


What is the demography of Paget’s disease of the nipple

• uncommon; <5% breast cancers
- most patients are elderly


Pathology

Almost all are  associated with an underlying malignancy (10% DCIS)
- hypothesises that ductal cells migrate along basement membrane into nipple epidermis.
Malignant Paget cells are derived from luminal lactiferous ductal epithelium (A) of breast tissue with retrograde extension of cancerous Paget cells into epidermis of overlying nipple (B).
Enlarged circle shows details that reveal thickening both of lining epithelium of breast duct and of nipple skin

- Associated with Paget’s cells (large cells with pale cytoplasm and prominent nucleoli) in the epidermis of the nipple

 

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Symptoms
- burning, itching, change in sensation.
- raised irregular scaling erythematous nipple


 

What is the Ix and treatment for Paget’s disease of the nipple

• Begin with detailed mammographic examination with magnified views of the subareola region

USS breast and axilla
Confirm diagnosis with core biopsy or wedge biopsy of nipple

• total mastectomy with axillary sampling – suitable for women with diffuse disease or disease at a distance from nipple.
- often in combination with radioRx, but little evidence as rare.

• DCIS?
--> WLE of nipple and underlying ducts
an option when disease localized to subareolar area or nipple areola complex
--> mastectomy if extensive DCIS.