The symptoms of Paget’s breast disease can resemble those of a skin condition, such as eczema or psoriasis. This sometimes delays diagnosis, increasing the risk that the condition will advance. Breast or nipple disease involves dry skin, flaking, or changes in the color or shape of the nipple. It may look like eczema or psoriasis, but it can be a sign of breast cancer.
Affected populations
Paget’s disease of the breast is a rare form of breast cancer that almost exclusively affects women, with rare cases reported in men. It most commonly affects middle-aged individuals, primarily between 50 and 60 years, but has also been documented in individuals in their 20s. Representing less than 5 percent of all breast cancer cases, its exact prevalence and incidence in the general population remain unknown, underscoring its rarity and importance in clinical awareness.
Only 8% of people with Paget’s disease of the breast have no other underlying cancer. Understanding that most cases are associated with different cancers can help healthcare professionals feel more prepared and thorough in their assessments. Paget’s disease of the breast may resemble other common skin issues, such as wounds, breastfeeding-related injuries, or eczema.
How Aggressively Does Paget’s Breast Disease Progress?
That depends on whether you have underlying ductal carcinoma and what stage it’s in. If you only have cancer in your milk ducts and it hasn’t spread to the surrounding breast tissues, it’s called “ductal carcinoma in situ” (DCIS). This is considered stage 0. This type of cancer is treated with surgical removal of the tumor(s) and nipple, followed by radiation.
When ductal carcinoma does spread beyond the milk ducts, it’s called infiltrating or invasive ductal carcinoma. This cancer progresses in stages. In the early stages, you can still treat it by removing the affected breast tissue and axillary lymph nodes. Invasive breast cancer becomes more aggressive and challenging to treat in the later stages when it spreads beyond the breast.
Symptoms
Paget’s Disease of the breast includes:
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- Flaky or scaly skin on the nipple.
- Crusty, oozing, or hardened skin that looks like eczema on the nipple, areola, or both.
- Itching.
- A burning sensation.
- Straw-colored or bloody nipple discharge.
- A turned-in nipple.
- A lump in the breast.
- Thickening skin on the breast.
Paget disease usually affects only one breast. In 80-90% of cases, Symptoms usually occur in one breast only. The symptoms typically start on the nipple and may spread to the areola and other areas of the breast. Your doctor might first treat this as eczema and, if it does not improve, recommend a biopsy.
Diagnoses of Paget’s Breast Disease
As many as half of Paget’s disease patients also have a palpable breast lump, according to the NCI, so a doctor may recommend a breast biopsy, which involves removing a small tissue sample for testing. Under a microscope, these cancer cells appear large and round and may be grouped or appear as single cells. In some circumstances, doctors may have to remove the entire nipple for the biopsy. Most people with Paget disease of the breast also have tumors in the same breast. One or more of the following imaging tests may be done to check for other breast changes:
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- Diagnostic mammogram
- Breast ultrasound
- Breast MRI (magnetic resonance imaging) scan
or a combination of these.
Treatment of Paget Breast Disease
Treatment options for Paget’s breast disease include surgical procedures such as breast-conserving surgery and mastectomy, with specific procedures depending on disease extent, to inform healthcare providers and patients about available interventions. If the disease involves only a small area of the breast tissue, a breast-conserving surgery (lumpectomy) may be performed to remove malignant tissue while preserving most of the breast, typically involving removal of the nipple and areola, along with noninvasive cancer like ductal carcinoma in situ (DCIS). If invasive cancer is present, a total mastectomy might be necessary.
A minority of patients with Paget’s disease have cancer confined to the nipple itself, with no sign of cancer elsewhere in the breast. For these patients, surgical removal of the nipple and areola is typically recommended.
During surgery for a Paget’s disease patient with invasive breast cancer, clinicians routinely evaluate the lymph nodes to detect any cancer spread, guiding further treatment decisions. Both a mastectomy and a lumpectomy are typically followed by radiation therapy for breast cancer, but breast cancer chemotherapy may be recommended as well.
Paget’s Disease of the Breast Prevention
There are many risk factors that you can’t control, such as getting older, but there are some lifestyle modifications that may reduce your risk of breast cancer and Paget’s disease. These include:
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- Getting regular physical activity
- Avoiding or limiting alcohol
- Quitting smoking
- Maintaining a healthy weight
In addition, discuss breast cancer screenings with your healthcare provider so that if you develop breast cancer, it can be detected as early as possible. Be familiar with how your breasts usually look and feel, and report any changes to a healthcare provider immediately.
For High-Risk People
If you have a high risk of breast cancer, such as a family history or a gene mutation that raises your risk, there are additional steps you can take to try to prevent it and/or catch it early if it does occur. These options include:
Genetic counseling
More frequent screenings and self-checks to look for early signs of breast cancer
Medicines to lower breast cancer risk
Preventive (prophylactic) surgery
SUMMARY
Paget’s disease of the breast is an early-stage breast cancer of the milk ducts that occurs in the nipple. It’s sometimes accompanied by more invasive breast cancer. You may notice nipple changes, such as crusting, skin flaking, discharge, or a raw area that doesn’t heal. Recognizing these signs early is crucial because early detection and treatment lead to better outcomes. Many people make a full recovery, but it will require surgery to remove it.
Paget’s Disease of the Breast: Life Expectancy
When diagnosed and treated early, Paget’s disease of the breast has an excellent outlook, helping patients feel hopeful about their recovery options. With Paget’s disease of the breast, the relative five-year survival is 87.1%. This means that 87 out of 100 people treated for Paget’s disease will live at least five years. Early detection and treatment contribute to these positive outcomes, and some individuals go on to live many more years with normal life expectancy. The noticeable symptoms of Paget’s disease often lead to early treatment, giving patients a sense of control over their health and future.
The five-year survival rate for Paget’s disease also varies by the stage of cancer. This is described by the National Cancer Institute as follows:
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- Stage 1: 98.8%
- Stage 2: 77.7%
- Stage 3: 46.3%
- Stage 4: 14.3%
Because Paget’s disease is rare, research is limited, but patients can access a dedicated NCI-supported database of clinical trials that offers hope and ongoing options.
References
American Breast Society
City of Hope
Cleveland Clinic
Mayo Clinic
Medical News Today
NCI (National Cancer Institute)
NORD (National Organization for Rare Diseases)
Verywell Health
