# Paget disease of the nipple

Source: https://onco.cc/cancers/paget-disease-of-the-nipple/  
OnCo record `paget-disease-of-the-nipple` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: Mammary Paget disease; Paget's disease of the breast; Paget disease of the nipple-areola complex
- Tags: subtype-page
- 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

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/paget-disease-of-the-nipple/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/paget-disease-of-the-nipple/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/paget-disease-of-the-nipple/#what-it-is [5 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/paget-disease-of-the-nipple/#finding-it [5 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/paget-disease-of-the-nipple/#treating-it [4 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/paget-disease-of-the-nipple/#evidence [1 key paper, 3 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/paget-disease-of-the-nipple/#science [2 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/paget-disease-of-the-nipple/where-you-are/ [16 UK centres]
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/paget-disease-of-the-nipple/#living-with-it [14 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/paget-disease-of-the-nipple/coming/ [2 medicines, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/paget-disease-of-the-nipple/data/ [21 connected records]

## Standard of care

- 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. ([Core needle biopsy and fine-needle aspiration (FNA)](https://onco.cc/terms/core-needle-biopsy/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [Mammography & tomosynthesis](https://onco.cc/technologies/mammography/), [MRI](https://onco.cc/technologies/mri/))
- 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. ([Lumpectomy (breast-conserving surgery)](https://onco.cc/terms/lumpectomy/), [Mastectomy](https://onco.cc/terms/mastectomy/), [Hypofractionated radiotherapy](https://onco.cc/technologies/hypofractionated-radiotherapy/))
- 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. ([Sentinel lymph node biopsy](https://onco.cc/technologies/sentinel-node/))
- Systemic therapy: Determined by the underlying carcinoma: endocrine therapy, chemotherapy and HER2-directed treatment on the same criteria as other breast cancers. ([Tamoxifen](https://onco.cc/drugs/tamoxifen/), [Trastuzumab](https://onco.cc/drugs/trastuzumab/), [HER2-positive breast cancer](https://onco.cc/cancers/breast-her2-positive/), [HR-positive / HER2-negative breast cancer](https://onco.cc/cancers/breast-hr-positive/), [Ductal carcinoma in situ (DCIS)](https://onco.cc/cancers/ductal-carcinoma-in-situ/))

## State of the art

- 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.

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Paget%27s_disease_of_the_breast
- NCI: Paget disease of the breast: https://www.cancer.gov/types/breast/paget-breast-fact-sheet
- Wikipedia: https://en.wikipedia.org/wiki/Paget%27s_disease_of_the_breast

## Connected records

- cancers: [Breast cancer (all types)](https://onco.cc/cancers/breast-cancer/), [Ductal carcinoma in situ (DCIS)](https://onco.cc/cancers/ductal-carcinoma-in-situ/), [HER2-positive breast cancer](https://onco.cc/cancers/breast-her2-positive/), [HR-positive / HER2-negative breast cancer](https://onco.cc/cancers/breast-hr-positive/), [Inflammatory breast cancer](https://onco.cc/cancers/inflammatory-breast-cancer/), [Phyllodes tumour of the breast](https://onco.cc/cancers/phyllodes-tumour/)
- key papers: [Paget disease of the nipple as a marker of multifocal, higher-risk underlying breast cancer](https://onco.cc/key-papers/paper-kothari-paget-disease-nipple-multifocal-cancer-2002/)
- terms: [Core needle biopsy and fine-needle aspiration (FNA)](https://onco.cc/terms/core-needle-biopsy/), [Lumpectomy (breast-conserving surgery)](https://onco.cc/terms/lumpectomy/), [Mastectomy](https://onco.cc/terms/mastectomy/), [Which staging edition a breast report uses: UICC TNM 8, TNM 9 and the AJCC prognostic stage](https://onco.cc/terms/tnm-breast-cancer-editions/)
- technologies: [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [Hypofractionated radiotherapy](https://onco.cc/technologies/hypofractionated-radiotherapy/), [Mammography & tomosynthesis](https://onco.cc/technologies/mammography/), [MRI](https://onco.cc/technologies/mri/), [Sentinel lymph node biopsy](https://onco.cc/technologies/sentinel-node/)
- drugs: [Tamoxifen](https://onco.cc/drugs/tamoxifen/), [Trastuzumab](https://onco.cc/drugs/trastuzumab/)

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JSON: https://onco.cc/api/v1/entities/paget-disease-of-the-nipple.json