Paget Disease of the Breast: Everything You Need to Know
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Paget disease of the breast is a rare form of breast cancer involving the skin of the nipple and the areola (dark circle around the nipple). This type of cancer accounts for only 1% to 4% of all breast cancer cases. In most cases of Paget’s disease, about 80% to 90%, a tumor or multiple tumors are found within the breast. These tumors are usually either ductal carcinoma in situ (DCIS) or invasive breast cancer. Although both men and women can develop Paget’s disease of the breast, it is more common in post-menopausal women, who have a higher risk of breast cancer in general. Please know that there are resources available to help navigate this diagnosis and make informed decisions about proper care.
Symptoms of Paget disease of the breast
Paget disease of the breast tends to present similar symptoms as certain benign skin conditions such as dermatitis and eczema. These include:
- Crusty, scaly, red skin of the nipple and areola
- Blood or yellow discharge coming out of the nipple
- Flat or inverted nipple
- Burning, itchy and tingling nipple
As Paget disease shares similar symptoms with benign skin conditions, it may sometimes be misdiagnosed initially. This is further complicated by the fact that it is also a rare disease. If you notice any symptoms, it is best to check in with a medical professional.
Diagnosis of Paget disease of the breast
Paget disease is normally diagnosed through a biopsy. There are several types that may be recommended to you by your doctor:
- Shave biopsy
A razor-like tool is used to remove the top layer of skin.
- Punch biopsy
A circular cutting tool is used to remove a disk-shaped piece of tissue.
- Wedge biopsy
A small wedge of tissue is removed using a scalpel.
The cells obtained from the biopsy are then observed under a microscope by a pathologist. Since tumors are often found in people with Paget disease of the breast, imaging tests and clinical breast exams may also be performed to check for tumors. These include:
Treatment of Paget disease of the breast
- Mastectomy (surgical removal of the whole breast)
It is common for patients with Paget disease to have one or more tumors in the breast affected by Paget disease. As it is rare that all the tumors can be removed with just the removal of the nipple and areola alone, mastectomy is commonly used as a treatment option.
- Central lumpectomy (surgical removal of part of the breast including the nipple and areola)
If no lump or tumor is found in the affected breast, a central lumpectomy (breast-conserving surgery) may be recommended to save as much of the breast. A lumpectomy is often followed by whole-breast-radiation therapy.
It is possible to have breast reconstruction after a central lumpectomy to restore the symmetry between both breasts. This may involve the use of implants (implant-based reconstruction) or tissue from another part of the body (autologous tissue (flap) reconstruction). It is also possible to restore the look of the breast with nipple reconstruction and areolar tattooing.
- Sentinel lymph node biopsy
If invasive cancer is found in a biopsy, a sentinel lymph node biopsy is normally recommended to check if cancer has spread to axillary lymph nodes. If DCIS is found in a biopsy and mastectomy is chosen, a sentinel lymph node biopsy may also be done.
- Adjuvant therapy (therapy done after surgical procedures)
Adjuvant chemotherapy or hormone therapy may be recommended depending on the outcome of the surgery or depending on the situation.ddd
Prognosis of Paget disease of the breast
The prognosis is dependent on several factors:
- Presence of tumor(s) in the affected breast
- If the tumor(s) is DCIS or invasive breast cancer
- Stage of cancer (if tumor(s) is invasive)
The five-year relative survival rate for all cases of Paget disease of the breast is 82.6% while that of the Paget disease with invasive tumors are as follows:
Stage of cancer | Five-year relative survival rate |
|---|---|
I | 95.8% |
II | 77.7% |
III | 46.3% |
IV | 14.3% |
