The first 60 days: Paget disease of the nipple
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. Below, week by week, is what OnCo's record of Paget disease of the nipple says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
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.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Diagnosis, Axilla.
- RadiologistNamed in the standard of care for: Diagnosis.
- SurgeonNamed in the standard of care for: Localised disease, Axilla.
- Medical oncologistNamed in the standard of care for: Localised disease, Systemic therapy.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised disease.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
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.
- 2.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.
Determined by the underlying carcinoma: endocrine therapy, chemotherapy and HER2-directed treatment on the same criteria as other breast cancers.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Full-thickness nipple biopsy showing Paget cells, 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), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Paget disease with underlying ductal carcinoma in situ, Paget disease with underlying invasive ductal carcinoma, Paget disease confined to the nipple epidermis.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Diagnosis
- For my situation (diagnosis), which of the standard options do you recommend and why?Guideline options include: 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.
Localised disease
- For my situation (localised disease), which of the standard options do you recommend and why?Guideline options include: 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.
Axilla
- For my situation (axilla), which of the standard options do you recommend and why?Guideline options include: 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.
Systemic therapy
- For my situation (systemic therapy), which of the standard options do you recommend and why?Guideline options include: Determined by the underlying carcinoma: endocrine therapy, chemotherapy and HER2-directed treatment on the same criteria as other breast cancers.
- Am I a candidate for Tamoxifen, Trastuzumab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of MRI?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Months of delay while the nipple is treated as eczema”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “No randomised evidence exists for any treatment choice”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Paget disease of the nipple: the full pagePaget 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.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Core needle biopsy and fine-needle aspiration (FNA): Taking a sliver of tissue (core) or a few cells (fine-needle aspiration) through a needle guided by ultrasound, CT or MRI, to diagnose the cancer and test its markers without surgery.
- Lumpectomy (breast-conserving surgery): Removing only the tumour with a rim of normal breast, keeping the breast; almost always followed by radiotherapy.
- Mastectomy: Removing the whole breast, either for cancer or preventively in BRCA1/2 carriers, where bilateral risk-reducing mastectomy cuts breast cancer risk by 90% or more.
Every term links to the glossary.