Paget's Disease of Breast

Synonyms: Paget's disease of the nipple

Also see article on Breast Cancer.

Paget's disease of the breast was described by Sir James Paget in 1874 in association with intraductal breast carcinoma. It presents as an eczematous lesion of the nipple and is associated with an underlying breast malignancy.

• Seen in 1-4% of cases of female breast cancer¹
• Also seen in male breast cancer but extremely rare²
• Most cases of Paget's disease of the breast are associated with invasive breast cancer - although 40% are associated with ductal carcinoma in situ¹

Malignant cells infiltrate into the epidermis via the mammary duct epithelium.¹ The cells proliferate leading to thickening of the affected skin.

• Usually unilateral but can occur bilaterally.
• Some cases may occur following mastectomy despite no breast tissue being present.³
• Usually involves the nipple but can extend to involve other areolar skin.
• Presents as chronic eczematous change of the nipple with:
  • Itching
  • Erythema
  • Scale formation
  • Erosions
  • Nipple discharge including bleeding
• Underlying palpable breast lump (usually indicates an invasive nature).

• Skin disorders, e.g. eczema (aways consider Paget's disease of the breast first)
• Melanoma of the nipple^4,5
• Nipple adenoma⁶

• Biopsy with immunohistochemistry and special stains
• Investigation of breast lump if palpable
• Investigations looking for underlying malignancy if no lump palpable, e.g. mammography or MRI (may be more useful)⁷

Presence of Paget's disease is associated with the following:

• Higher histological grade
• Oestrogen receptor negative breast cancer
• Progesterone receptor negative breast cancer

Paget's disease of the nipple with no associated tumour is staged as carcinoma in situ. Otherwise these are classified according to the size of the underlying tumour.

This is surgical with breast-conserving surgery. Studies have revealed no difference between mastectomy or lumpectomy for breast cancer associated with Paget's disease of the breast.⁸ Furthermore, the addition of radiotherapy to breast-conserving surgery may be better in patients with Paget's disease of the breast associated with no palpable mass nor abnormality on mammography (despite a high suspicion of underlying breast malignancy).⁹ Medical management and chemotherapy for the underlying breast lesion may also be required.

Paget's disease of the breast is associated with a poor prognosis, which is worse if the lesion is associated with an underlying palpable mass.¹⁰

• This is Paget's disease affecting sites other than the breast.
• Has a predilection for areas rich in apocrine glands, e.g. axillae.
• 75% are not associated with underlying malignancy.
• Others are associated with underlying invasive carcinoma or represent primary cutaneous adenocarcinoma.