{"entity":{"id":"paget-disease-of-the-nipple","kind":"cancer","name":"Paget disease of the nipple","aka":["Mammary Paget disease","Paget's disease of the breast","Paget disease of the nipple-areola complex"],"tldr":"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.","summary":"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.\n\nMastectomy 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.\n\nPrognosis 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.","asOf":"2026-09-17","wikipedia":"https://en.wikipedia.org/wiki/Paget%27s_disease_of_the_breast","links":[{"label":"NCI: Paget disease of the breast","url":"https://www.cancer.gov/types/breast/paget-breast-fact-sheet"},{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Paget%27s_disease_of_the_breast"}],"tags":["subtype-page"],"related":["ductal-carcinoma-in-situ"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"breast","burden":"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.","subtypes":["Paget disease with underlying ductal carcinoma in situ","Paget disease with underlying invasive ductal carcinoma (prognosis set by the invasive tumour)","Paget disease confined to the nipple epidermis (no underlying carcinoma found)","Paget disease with a palpable mass (higher chance of invasion and node involvement)","Pigmented Paget disease (melanoma mimic)"],"biomarkers":["Full-thickness nipple biopsy showing Paget cells (cytokeratin 7-positive, HER2 often overexpressed)","Mammography and breast MRI for the underlying tumour","Oestrogen receptor, progesterone receptor and HER2 of the underlying carcinoma","Nodal status by sentinel node biopsy when invasive disease is present","Margins of the nipple-areola excision"],"standardOfCare":[{"setting":"Diagnosis","approach":"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.","refs":["core-needle-biopsy","histopathology-ihc","mammography","mri"]},{"setting":"Localised disease","approach":"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.","refs":["lumpectomy","mastectomy","hypofractionated-radiotherapy"]},{"setting":"Axilla","approach":"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.","refs":["sentinel-node"]},{"setting":"Systemic therapy","approach":"Determined by the underlying carcinoma: endocrine therapy, chemotherapy and HER2-directed treatment on the same criteria as other breast cancers.","refs":["tamoxifen","trastuzumab","breast-her2-positive","breast-hr-positive","ductal-carcinoma-in-situ"]}],"stateOfArt":["Breast conservation with radiotherapy has replaced routine mastectomy for limited disease.","Breast MRI finds the underlying tumour that mammography misses in a substantial share of cases.","Nipple reconstruction restores appearance after central excision."],"history":[{"year":1874,"title":"James Paget describes eczema of the nipple preceding breast cancer","refs":[]},{"year":2001,"title":"EORTC 10873: breast conservation with radiotherapy for Paget disease","refs":["lumpectomy"]},{"year":2006,"title":"Population analysis shows survival matched by stage is equal after breast conservation and mastectomy","refs":["mastectomy"]}],"pipeline":["mri"],"openProblems":["Months of delay while the nipple is treated as eczema.","No randomised evidence exists for any treatment choice.","Whether Paget disease without an underlying carcinoma needs radiotherapy at all."],"parent":"breast-cancer"},"route":"/cancers/paget-disease-of-the-nipple/","neighbours":{"cancer":[{"id":"breast-cancer","kind":"cancer","name":"Breast cancer (all types)","route":"/cancers/breast-cancer/"},{"id":"ductal-carcinoma-in-situ","kind":"cancer","name":"Ductal carcinoma in situ (DCIS)","route":"/cancers/ductal-carcinoma-in-situ/"},{"id":"breast-her2-positive","kind":"cancer","name":"HER2-positive breast cancer","route":"/cancers/breast-her2-positive/"},{"id":"breast-hr-positive","kind":"cancer","name":"HR-positive / HER2-negative breast cancer","route":"/cancers/breast-hr-positive/"}],"term":[{"id":"core-needle-biopsy","kind":"term","name":"Core needle biopsy and fine-needle aspiration (FNA)","route":"/terms/core-needle-biopsy/"},{"id":"lumpectomy","kind":"term","name":"Lumpectomy (breast-conserving surgery)","route":"/terms/lumpectomy/"},{"id":"mastectomy","kind":"term","name":"Mastectomy","route":"/terms/mastectomy/"}],"technology":[{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"},{"id":"hypofractionated-radiotherapy","kind":"technology","name":"Hypofractionated radiotherapy","route":"/technologies/hypofractionated-radiotherapy/"},{"id":"mammography","kind":"technology","name":"Mammography & tomosynthesis","route":"/technologies/mammography/"},{"id":"mri","kind":"technology","name":"MRI","route":"/technologies/mri/"},{"id":"sentinel-node","kind":"technology","name":"Sentinel lymph node biopsy","route":"/technologies/sentinel-node/"}],"drug":[{"id":"tamoxifen","kind":"drug","name":"Tamoxifen","route":"/drugs/tamoxifen/"},{"id":"trastuzumab","kind":"drug","name":"Trastuzumab","route":"/drugs/trastuzumab/"}]}}