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Home > Professional Reference > Paget's Disease of Breast

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Paget's Disease of Breast

This PatientPlus article is written for healthcare professionals so the language may be more technical than the condition leaflets. You may find the abbreviations list helpful.

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.

Epidemiology

  • 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

Pathophysiology

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

Presentation

  • 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:
  • Underlying palpable breast lump (usually indicates an invasive nature).

Differential diagnosis

  • Skin disorders, e.g. eczema (aways consider Paget's disease of the breast first)
  • Melanoma of the nipple4,5
  • Nipple adenoma6

Investigations

  • 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

Paget's disease associations8

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

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

Staging

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.

Management

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.

Prognosis

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

Extramammary Paget's disease

  • 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.

Document references

  1. Subramanian A, Birch H, McAvinchey R, et al; Pagets disease of uncertain origin: case report. Int Semin Surg Oncol. 2007 May 6;4:12. [abstract]
  2. Piekarski J, Kubiak R, Jeziorski A; Clinically silent Paget disease of male nipple. J Exp Clin Cancer Res. 2003 Sep;22(3):495-6. [abstract]
  3. Giovannini M, D'Atri C, Piubello Q, et al; Mammary Paget's disease occurring after mastectomy. World J Surg Oncol. 2006 Aug 9;4:51. [abstract]
  4. Kinoshita S, Yoshimoto K, Kyoda S, et al; Malignant melanoma originating on the female nipple: a case report. Breast Cancer. 2007;14(1):105-8. [abstract]
  5. Lin CH, Lee HS, Yu JC; Melanoma of the nipple mimicking Paget's disease. Dermatol Online J. 2007 May 1;13(2):18.
  6. Sugai M, Murata K, Kimura N, et al; Adenoma of the nipple in an adolescent. Breast Cancer. 2002;9(3):254-6. [abstract]
  7. Echevarria JJ, Lopez-Ruiz JA, Martin D, et al; Usefulness of MRI in detecting occult breast cancer associated with Paget's disease of the nipple-areolar complex. Br J Radiol. 2004 Dec;77(924):1036-9. [abstract]
  8. Chen CY, Sun LM, Anderson BO; Paget disease of the breast: changing patterns of incidence, clinical presentation, and treatment in the U.S. Cancer. 2006 Oct 1;107(7):1448-58. [abstract]
  9. Marshall JK, Griffith KA, Haffty BG, et al; Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer. 2003 May 1;97(9):2142-9. [abstract]
  10. Piekarski J, Jeziorski A, Baklinska M, et al; Patients with Paget disease of nipple and with palpable mass in breast have unfavorable prognosis. J Exp Clin Cancer Res. 2004 Mar;23(1):33-7. [abstract]

Acknowledgements

EMIS is grateful to Dr Richard Draper for writing this article and to Dr Gurvinder Rull for earlier versions. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2009.
Document ID: 2562
Document Version: 21
Document Reference: bgp1273
Last Updated: 30 Jul 2009
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