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Synonyms: Paget's disease of the nipple, mammary Paget's disease
Paget's disease of the breast was described by Sir James Paget in 1874. It is a rare type of cancer of the nipple-areola complex presenting as an eczematous lesion of the nipple and is often associated with an underlying in-situ or invasive carcinoma of the breast.
- It is seen in 1-4% of cases of female breast cancer.
- It is also seen in male breast cancer but extremely rarely.
- Most cases of Paget's disease of the breast are associated with invasive breast cancer - although 40% are associated with ductal carcinoma in situ.
Malignant cells infiltrate into the epidermis via the mammary duct epithelium. The cells proliferate leading to thickening of the affected skin.
- It is usually unilateral but can occur bilaterally.
- Some cases may occur following mastectomy despite no breast tissue being present.
- It usually involves the nipple but can extend to involve other areolar skin.
- It presents as chronic eczematous change of the nipple with:
- Scale formation.
- Nipple discharge including bleeding.
- An underlying palpable breast lump (usually indicates an invasive nature).
- Skin disorders - eg, eczema (always consider Paget's disease of the breast first).
- Melanoma of the nipple.[5, 6]
- Nipple adenoma.
- Biopsy with immunohistochemistry and special stains.
- Investigation of a breast lump, if palpable.
- Investigations looking for underlying malignancy if there is no lump palpable - eg, mammography or MRI (may be more useful).
Paget's disease associations
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.
The surgical treatment of Paget's disease is controversial. Mastectomy with or without axillary lymph node dissection has been regarded as the standard therapy for Paget's disease, even in the absence of other clinical signs of malignancy. High recurrence rates have been reported for patients treated with local excision alone, whose mammograms suggested in-situ changes confined to the nipple area. It has therefore been concluded that local excision alone is not an appropriate treatment for patients with Paget's disease of the nipple.
However, with increasing diagnosis of early breast cancers, findings from multiple trials have shown that breast-conserving therapy is a feasible alternative for patients with disease limited to the central segment of the breast. Recent reports from several studies with long-term follow-up have shown that breast-conserving surgery is equivalent to mastectomy in terms of overall and disease-free survival in patients with breast cancer.[1, 9]
The addition of radiotherapy to breast-conserving surgery may be better in patients with Paget's disease of the breast associated with neither palpable mass nor abnormality on mammography (despite a high suspicion of underlying breast malignancy). Medical management and chemotherapy for the underlying breast lesion may also be required.
- In one study, 62% of patients with mammary Paget's disease presented with a detectable breast mass.
- 50% of patients with Paget's disease presenting with a palpable breast mass have associated axillary lymph node metastasis.
- Even in patients with mammary Paget's disease and no underlying tumour, 30% may develop an invasive carcinoma at a later date and 20% of patients already have an associated in-situ carcinoma of the breast.
- Factors of unfavorable prognosis include the presence of a palpable breast tumour, lymph node enlargement, histological type of breast cancer, and age younger than 60 years.
Extramammary Paget's disease
- Approximately 25% of the cases are associated with an underlying in-situ or invasive neoplasm, particularly an adnexal apocrine carcinoma. Other associated malignancies include carcinomas of Bartholin's glands, urethra, bladder, vagina, cervix, endometrium and prostate.
- It is a rare condition with only several hundred cases in the world reported. It most commonly appears in those aged 50-60 years and is more common in women.
- It usually presents as chronic dermatitis of the groin, genitalia or perianal area, which is resistant to treatment. It is often associated with intense pruritus and long-standing lesions may cause pain and bleeding.
Further reading and references
Karakas C; Paget's disease of the breast. J Carcinog. 201110:31. doi: 10.4103/1477-3163.90676. Epub 2011 Dec 8.
Subramanian A, Birch H, McAvinchey R, et al; Pagets disease of uncertain origin: case report. Int Semin Surg Oncol. 2007 May 64:12.
Piekarski J, Kubiak R, Jeziorski A; Clinically silent Paget disease of male nipple. J Exp Clin Cancer Res. 2003 Sep22(3):495-6.
Giovannini M, D'Atri C, Piubello Q, et al; Mammary Paget's disease occurring after mastectomy. World J Surg Oncol. 2006 Aug 94:51.
Kinoshita S, Yoshimoto K, Kyoda S, et al; Malignant melanoma originating on the female nipple: a case report. Breast Cancer. 200714(1):105-8.
Lin CH, Lee HS, Yu JC; Melanoma of the nipple mimicking Paget's disease. Dermatol Online J. 2007 May 113(2):18.
Sugai M, Murata K, Kimura N, et al; Adenoma of the nipple in an adolescent. Breast Cancer. 20029(3):254-6.
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 Dec77(924):1036-9.
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 1107(7):1448-58.
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 197(9):2142-9.
Kanitakis J; Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol. 2007 May21(5):581-90.
Lam C, Funaro D; Extramammary Paget's disease: Summary of current knowledge. Dermatol Clin. 2010 Oct28(4):807-26. doi: 10.1016/j.det.2010.08.002.
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