Paget disease of the nipple
Prepared with OnCo (onco.cc/prep/paget-disease-of-the-nipple/). Orientation, not medical advice; your team knows your case.
My details
What I know, what is unclear, changes to discuss
Saved in this browserMy questions
14 on the sheet- 1.What is my exact diagnosis, stage, and grade, and which tests established them?
- 2.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?
- 3.Which subtype is my cancer, and does that change the recommended treatment?
- 4.Is germline (inherited) genetic testing recommended for me or my family?
- 5.For my situation (diagnosis), which of the standard options do you recommend and why?
- 6.For my situation (localised disease), which of the standard options do you recommend and why?
- 7.For my situation (axilla), which of the standard options do you recommend and why?
- 8.For my situation (systemic therapy), which of the standard options do you recommend and why?
- 9.Am I a candidate for Tamoxifen, Trastuzumab, and what side effects should I expect?
- 10.Are there clinical trials I could join, for example of MRI?
- 11.Would a second opinion at a high-volume centre change anything, and can you help arrange it?
- 12.What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
- 13.I read that “Months of delay while the nipple is treated as eczema”. How does that affect my plan?
- 14.I read that “No randomised evidence exists for any treatment choice”. How does that affect my plan?
The words I may hear
- 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.
Tests and results to bring
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.
Biomarker results to ask for: 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.
Scans and tests linked to this cancer: Histopathology & immunohistochemistry, Mammography & tomosynthesis, MRI.
Bring copies of scan reports, pathology and blood results, and a list of every medicine and supplement.
The treatments I may be offered
- 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), Mastectomy, 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)
- Systemic therapy: Determined by the underlying carcinoma: endocrine therapy, chemotherapy and HER2-directed treatment on the same criteria as other breast cancers. (Tamoxifen, Trastuzumab, HER2-positive breast cancer, HR-positive / HER2-negative breast cancer, Ductal carcinoma in situ (DCIS))
From the standard of care recorded for this cancer; which apply depends on your stage and biomarkers. Ask which the team recommends and why.