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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis | 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. | not mapped |
| Localised disease | 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. | not mapped |
| Axilla | 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. | not mapped |
| Systemic therapy | Determined by the underlying carcinoma: endocrine therapy, chemotherapy and HER2-directed treatment on the same criteria as other breast cancers. | not mapped |