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Paget disease of the nipple looks like eczema: a scaly, red, itchy or weeping patch on the nipple that creams do not heal. Cancer cells have spread along the milk ducts into the nipple skin, and in most cases a ductal carcinoma in situ or invasive cancer lies underneath. Surgery removes the nipple and areola with the disease beneath, by mastectomy or central breast conservation with radiotherapy.
Paget disease presents as a unilateral eczema-like change of the nipple that spreads to the areola: scaling, redness, itching, crusting, discharge or nipple retraction, often treated as dermatitis for months before biopsy. Under the microscope the epidermis contains Paget cells, large pale cells with abundant cytoplasm that stain for cytokeratin 7 and, in most cases, overexpress HER2; they are thought to migrate from an underlying ductal carcinoma along the lactiferous ducts into the nipple skin. A full-thickness punch or wedge biopsy of the nipple makes the diagnosis, and bilateral mammography with breast MRI then looks for the disease behind it, which is present in the large majority of cases as ductal carcinoma in situ, invasive ductal carcinoma or both; a palpable mass makes invasion and node involvement more likely. Paget disease confined to the nipple without an underlying carcinoma is the exception, and extramammary Paget disease of the vulva or perianal skin is a different condition.
Mastectomy was the standard for a century because the underlying disease is often extensive or multicentric. The EORTC 10873 study of 61 women treated with excision of the nipple-areola complex and underlying tumour followed by whole-breast radiotherapy reported a five-year local recurrence rate of about five percent, and population analyses show survival matched by stage is the same after breast conservation with radiotherapy as after mastectomy, so central lumpectomy with radiotherapy is now offered when the underlying disease is limited and the margins are clear. Sentinel node biopsy is performed when invasive disease is present or when mastectomy is planned, because the nipple cannot be re-sampled afterwards. Systemic treatment, endocrine, chemotherapy and HER2-directed, follows the stage and receptors of the underlying carcinoma, not the nipple.
Prognosis is set by what lies beneath. Paget disease with in situ disease alone is cured in almost all cases, and Paget disease with an invasive tumour behaves like that tumour, with node status the strongest factor. The frequent HER2 overexpression of Paget cells has made the disease a model for HER2 biology in the skin and an occasional candidate for topical or HER2-directed approaches, but there are no randomised trials of anything in a condition this rare, and delay in diagnosis remains the main avoidable harm.
Rare, about one to three percent of breast cancers, mostly in women in their fifties and sixties; in the large majority an underlying ductal carcinoma in situ or invasive cancer sits behind the nipple change.
Most cancers start in the ducts and drain first to the axillary nodes, which is why the armpit is checked and a sentinel node is sampled.
Same organ: Triple-negative breast cancer (TNBC), Breast cancer (all types), HR-positive / HER2-negative breast cancer, HER2-positive breast cancer, Male breast cancer, Ductal carcinoma in situ (DCIS), High-risk early HR-positive breast cancer, HR-positive metastatic breast cancer after CDK4/6 inhibitors, HER2-low and HER2-ultralow metastatic breast cancer, Early HER2-positive breast cancer, HER2-positive breast cancer with brain metastases, Early triple-negative breast cancer, Metastatic triple-negative breast cancer, Basal-like 1 triple-negative breast cancer (BL1), Basal-like 2 triple-negative breast cancer (BL2), Mesenchymal triple-negative breast cancer (M), Mesenchymal stem-like triple-negative breast cancer (MSL), Luminal androgen receptor triple-negative breast cancer (LAR), Immunomodulatory triple-negative breast cancer (IM), Metaplastic breast carcinoma, Carcinoma with medullary pattern (medullary breast cancer), Adenoid cystic carcinoma of the breast, Apocrine carcinoma of the breast, Secretory carcinoma of the breast, BRCA-associated triple-negative breast cancer, Inflammatory breast cancer, Phyllodes tumour of the breast, Invasive lobular carcinoma of the breast, Invasive breast carcinoma of no special type (invasive ductal carcinoma), Tubular carcinoma of the breast, Mucinous carcinoma of the breast, Papillary carcinomas of the breast (encapsulated, solid and invasive papillary), Invasive cribriform carcinoma of the breast, Invasive micropapillary carcinoma of the breast, Neuroendocrine neoplasms of the breast, Lobular carcinoma in situ (LCIS)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Punch or wedge biopsy of the nipple for any eczema-like change not settling within a few weeks, with bilateral mammography and breast MRI to map the disease behind it.
Central breast-conserving surgery removing the nipple-areola complex and underlying tumour with clear margins followed by whole-breast radiotherapy, or mastectomy for extensive or multicentric disease.
Sentinel node biopsy when invasive carcinoma is present or when mastectomy is planned; not needed for Paget disease with in situ disease treated by breast conservation.
Determined by the underlying carcinoma: endocrine therapy, chemotherapy and HER2-directed treatment on the same criteria as other breast cancers.
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Query for this cancer: (TITLE:"Paget disease of the nipple" OR ABSTRACT:"Paget disease of the nipple" OR TITLE:"Mammary Paget disease" OR ABSTRACT:"Mammary Paget disease" OR TITLE:"Paget's disease of the breast" OR ABSTRACT:"Paget's disease of the breast" OR TITLE:"Paget disease of the nipple-areola complex" OR ABSTRACT:"Paget disease of the nipple-areola complex") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Paget disease of the nipple, not a curated reading list.
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A swollen painful calf, or sudden breathlessness with chest pain; the tamoxifen boxed warning covers pulmonary embolism and stroke.
Possible QT prolongation. Check ECG and electrolytes; review other QT-prolonging drugs.
Heart damage from cancer treatment: anthracyclines weaken the heart muscle permanently in a dose-related way, trastuzumab does so reversibly, and some kinase inhibitors raise blood pressure or disturb rhythm. Heart function (LVEF) is monitored by ultrasound during treatment.
Gradual thinning at the parting and crown that builds over months on tamoxifen, an aromatase inhibitor or ovarian suppression. It is not the sudden shedding of chemotherapy, it lasts as long as the treatment does, and it is often dismissed because it is mild on a clinician's scale and not on the patient's.
HER2 drugs can weaken the heart's pumping, usually reversibly, so heart function is checked every three months during treatment.
Blood clots in the leg veins or lungs. Cancer makes blood clot more easily and some treatments (IMiDs, anti-VEGF drugs, hormone therapy, central lines, surgery) add risk; clots are the second commonest cause of death in cancer patients after the cancer itself.
See all on the product pages:TamoxifenTrastuzumab·Printable cards in the navigator
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