Paget’s disease of the breast
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Paget’s disease of the breast is a particularly uncommon type of breast cancer that affects the black circle of skin (areola) around the nipple and begins on the nipple. It is important to note that Paget’s disease of the breast is unrelated to the metabolic bone illness, Paget’s disease of the bone.
This type of breast cancer usually develops after the age of 50 and is commonly associated with ductal breast cancer in situ (meaning in its original location) or, less frequently, invasive breast cancer. It is rare for Paget’s disease of the breast to only affect the nipple.
Initially, Paget’s disease of the breast may be mistaken for a common rash since it can look similar to eczema on the nipple. Symptoms may include nipple discharge, red or raised plaques on the skin, scaling, and itching. However, it is important to note that most individuals with these symptoms do not have Paget’s disease. Nevertheless, if you do have these symptoms, they might be the first obvious indications of underlying breast cancer.
Symptoms
The symptoms of Paget’s disease of the breast typically impact the nipple and the skin around it, known as the areola. These symptoms can be similar to those of a benign skin condition such as dermatitis, which can make it challenging to identify Paget’s disease.
Paget’s disease of the breast could manifest as any of the following:
- Itchiness
- Reddish discoloration
- An ache or tingling sensation
- Thickening of the breast skin
- Skin that is flaky or scaly on your nipple
- A nipple that has been flattened or turned inward
- Breast bulge under the skin that can be felt.
- Bloody, pus-like or straw-colored nipple discharge
- Eczema-like skin that is crusty, seeping, or hardened on the areola, nipple, or both
Usually, signs and symptoms of this condition are only present in one breast, starting in the nipple and potentially spreading to other parts of the breast. Skin changes may appear and disappear early on or improve with topical treatment, leading to the illusion of healing. Typically, signs and symptoms persist for several months before a diagnosis is confirmed.
It is important to monitor any changes in your breasts. If you detect a lump or experience persistent itching or skin irritation for over a month, schedule an appointment with your doctor. Additionally, if treatment for a breast injury does not resolve the issue, schedule a follow-up appointment with your doctor.
Causes
The exact cause of Paget’s disease of the breast remains unknown. However, it is understood that cancer involves abnormal changes in specific cells that result in uncontrolled division and replication. The current understanding is that these changes may originate in the DNA or genetic code of cells. Nonetheless, the reasons for these changes occurring at specific times remain unknown.
Risk factors
The risk factors that increase your chances of developing Paget’s disease of the breast are similar to those that raise your risk of developing other types of breast cancer.
Some factors that increase your risk of developing breast cancer are as follows:
- Aging. Risk increases with age
- Early menstruation. Your risk of developing breast cancer rises if you start menstruating before age 12.
- Late menopause. Breast cancer is more likely to strike women who started menopause later in life.
- Late primary pregnancy. Your risk of breast cancer may increase if you have your first child after turning 30.
- Never experience pregnancy. Your risk of developing breast cancer may be higher if you have never been pregnant.
- Genetically inheriting genes that poses risk for having cancer. It is possible for parents to pass on some DNA mutations that enhance the risk of breast cancer to their offspring. The BRCA1 and BRCA2 gene mutations are the most well-known ones. These genes can significantly increase your risk of developing breast cancer and other malignancies, but they do not guarantee that you will develop one.
- Postmenopausal hormone therapy. Breast cancer risk may increase with hormone therapy drugs that combine estrogen and progesterone to treat menopause symptoms and indications. When you quit using these drugs, the danger goes down.
- History of breast problem. You are more likely to develop breast cancer if a breast biopsy revealed Lobular Carcinoma in Situ (LCIS) or atypical hyperplasia of the breast.
- Breast cancer in the past. You run a higher risk of getting breast cancer in the other breast if you’ve already had it in the first.
- Breast cancer history in the family. Your risk of developing breast cancer is enhanced if a parent, sibling, or child, particularly one who was diagnosed at a young age, had the disease. Nevertheless, the majority of breast cancer patients do not have a family history of the condition.
- Being exposed to radiation. Your risk of developing breast cancer is higher if you had chest radiation treatments as a kid or young adult.
- Being overweight. Your risk of developing breast cancer rises if you are obese.
- Alcohol consumption. Alcohol consumption raises the possibility of breast cancer.
Diagnosis
The following tests and techniques are used to identify Paget’s disease of the breast:
- Breast exam. Your doctor will feel for lumps or other abnormalities in your lymph nodes in the armpit as well as both of your breasts.
- Mammogram. An X-ray breast imaging is done with a mammogram. Breast cancer screening with mammograms is quite widespread. Your doctor might advise a diagnostic mammography if a screening mammogram reveals an anomaly that has to be further investigated.
- Breast ultrasound. Ultrasound employs sound waves to create pictures of inside organs at great depths. If a new breast lump is found to be a solid mass or a cyst filled with fluid, ultrasound may be performed to identify it.
- Breast Magnetic Resonance Imaging (MRI). A magnet and radio waves are used by an MRI machine to produce images of your breast’s interior. You get a dye injection before to a breast MRI.
- Biopsy. A doctor takes a sample of suspect tissue to determine whether the cells are malignant. The biopsy samples are sent to a lab for investigation in order to analyze the type of cells involved in the breast cancer, its aggressiveness (grade), and whether the cancer cells have hormone receptors or other receptors that could affect treatment options.
Depending on your circumstances, additional tests and treatments might be used.
Treatment
It’s likely that surgery will be required if you have Paget’s disease of the breast. The kind of surgery you need will depend on the health of the skin around your nipple and how far along the cancer is in its progression.
The following are treatment options that are available:
Surgery:
- Mastectomy. The entire breast tissue is removed during a mastectomy. In a total or simple mastectomy, all of the breast tissue is removed, including the lobules, ducts, fatty tissue, and some skin, including the nipple and areola.
- Lumpectomy. A lumpectomy, also known as breast-conserving surgery or wide local excision, involves the removal of the malignancy and a small margin of healthy tissue around it by the surgeon. You will then undergo radiation therapy if you decide to pursue this course of action with your doctor.
- Sentinel node biopsy. Your surgeon will go through with you the importance of removing the lymph nodes that are the first to receive the lymph drainage from your malignancy in order to establish whether cancer has spread to your lymph nodes.
The likelihood of detecting cancer in any of the remaining lymph nodes is low if those lymph nodes are cancer-free, thus no additional nodes need to be removed.
- Axillary lymph node dissection. Your surgeon will go over the pros and cons of removing extra lymph nodes from your armpit with you if cancer is discovered in the sentinel lymph nodes.
- Removing both breasts. If they have a very high chance of developing cancer in the other breast due to a genetic predisposition or a significant family history, some women with cancer in one breast may elect to have their second (healthy) breast removed (contralateral preventive mastectomy).
After surgery, you can choose to undergo breast reconstruction. Discuss your options and preferences with your surgeon. Prior to the procedure, consider seeking a plastic surgeon’s recommendation. You may be able to reconstruct your breast using your own tissue or a breast implant. These procedures can be done at the same time as your mastectomy or at a later time.
Adjuvant therapy
Your doctor may advise extra treatment (adjuvant therapy) following surgery, such as hormone therapy, radiation therapy, or chemotherapy to stop the spread of breast cancer. The type of treatment you receive will depend on the cancer’s stage and whether it tests positive for specific traits like estrogen or progesterone receptors.
