What Is Paget’s Disease Of The Breast?

Overview

Paget disease of the breast, sometimes referred to as Paget disease of the nipple and mammary Paget disease, is a rare kind of cancer that typically affects the skin of the nipple and the darker circle of skin around it, known as the areola. In the majority of cases, women with Paget disease of the breast also have one or more tumours within the same breast. Either ductal carcinoma in situ or invasive breast cancer characterises these breast cancers.

Paget disease of the breast is named after Sir James Paget, a 19th century British clinician who discovered a link between nipple alterations and breast cancer in 1874.¹

Causes of paget’s disease of the breast

The cause of Paget's disease of the nipple is uncertain. In general, but not specifically with regard to Paget's illness, some women seem to be at higher risk of developing breast cancer. An identifiable cause of a particular form of breast cancer is unknown.²

Signs and symptoms of paget’s disease of the breast

Your nipple and areola are both affected by Paget's disease of the breast. It is simple to confuse the symptoms of Paget's disease of the breast with dermatitis or another benign (noncancerous) skin disorder.

Paget's disease of the breast could manifest as any of the following:

• Skin that is flaky or scaly on your nipple
• Eczema-like skin that is crusty, seeping, or hardened on the areola, nipple, or both
• Itching 
• Redness
• Burning or tingling feeling
• Crimson or straw-coloured nipple discharge
• A nipple that is flattened or turned inward(inverted) 
• Breast lump
• Increasing breast skin thickness³

Management and treatment for paget’s disease of the breast

Treatment involves removing the malignant portion of the breast or in certain cases, the entire breast using a procedure called mastectomy.

Surgery 

Surgery is frequently the first kind of treatment given to someone with Paget's disease of the nipple.

The two main surgical procedures are:

Breast conserving surgery

A small amount of good tissue and the cancer are removed during breast-conserving surgery in an effort to save as much of your breast as possible.

Your nipple and areola will be removed if you have Paget's disease of the nipple. To enhance the appearance of your breast following surgery, reconstructive surgery should be offered to you.

The quantity of breast tissue removed during breast-conserving surgery will depend on:

• The tumour's size in relation to the size of your breast 
• Whether it is centralised to one area or dispersed across the breast

To check for signs of cancer, your surgeon will remove some healthy breast tissue from the area surrounding the malignancy. More tissue may need to be removed from your breast if cancerous cells are discovered in the tissue surrounding the affected area.

After having breast-conserving surgery, it's likely you'll need to have radiotherapy to destroy any remaining cancer cells.

Mastectomy

Your entire breast, including your nipple, will be removed during a mastectomy. You might require a mastectomy if:

• The tumour is large or in the centre of your breast
• There's more than 1 area of breast cancer
• Breast-conserving surgery will not provide acceptable results⁴

Radiation therapy

High-energy radiation that kills cancer cells is known as radiation therapy. In addition to other treatments, radiation may be necessary for some breast cancer patients.

Radiation therapy may be utilised in a variety of circumstances, depending on the stage of the breast cancer and other variables:

• Following breast-conserving surgery (BCS), to lessen the likelihood that the cancer would return in the same breast or surrounding lymph nodes.
• Following a mastectomy, particularly if the cancer was greater than 5 cm (about 2 inches), if cancer was discovered in numerous lymph nodes, or if specific surgical margins, including the skin or muscle, had cancer cells.
• If cancer has spread to other parts of the body, such as the bones, spinal cord, or brain.

Types of radiation therapy for breast cancer

The main types of radiation therapy that can be used to treat breast cancer are:

• External beam radiation therapy
• Brachytherapy

External beam radiation therapy (EBRT): The most popular radiation therapy for breast cancer patients is EBRT. Radiation is directed toward the cancerous tissue by a device outside the body. Whether you underwent a mastectomy or breast-conserving surgery (BCS), and whether the cancer has spread to adjacent lymph nodes, will determine which areas require radiation.

Brachytherapy: Another method of administering radiation therapy is called brachytherapy, also referred to as internal radiation. A device containing radioactive seeds or pellets is put into the breast tissue for a brief period of time in the region where the cancer had been excised (tumour bed), replacing the use of radiation beams that are directed from outside the body.

Chemotherapy

Anti-cancer medications are used during chemotherapy (chemo), which can be either orally or intravenously (injected into a vein). The majority of the body's cancer cells are reached by the medications via the bloodstream. Chemotherapy may occasionally be administered directly into this region if cancer has spread to the spinal fluid, which surrounds and protects the brain and spinal cord (called intrathecal chemotherapy)

After surgery (adjuvant chemotherapy): Adjuvant chemotherapy may be administered in an effort to eradicate any cancer cells that may have survived or spread but are invisible to imaging examinations. Because they are invisible to the unaided eye, these cells are regarded as microscopic. Breast cancer recurrence risk can be decreased by adjuvant chemotherapy.

Before surgery (neoadjuvant chemotherapy): The goal of neoadjuvant chemotherapy is to reduce the tumour's size so that it can be removed with less invasive surgery. Neoadjuvant chemotherapy is therefore frequently used to treat tumours that are too large to be surgically removed at the time of diagnosis, have several lymph nodes involved, or have inflammatory breast cancers.

Hormone therapy

Hormones like progesterone and oestrogen can affect some kinds of breast cancer. The oestrogen and progesterone receptors (proteins) on the surface of breast cancer cells aid in the growth of these cells. Hormone or endocrine therapy refers to treatments that prevent these hormones from binding to these receptors. Nearly every part of the body, not only the breast, can be treated with hormone therapy when presenting cancer cells. It is advised for females with hormone receptor-positive malignancies. It is useless for women whose tumours lack hormone receptors (these tumours are called hormone receptor-negative).

When is hormone therapy used for breast cancer?

In order to lower the likelihood of the cancer recurring, hormone therapy is commonly used as adjuvant therapy following surgery. Sometimes it begins prior to surgery (as neoadjuvant therapy).

Cancer that has returned after treatment or spread to other places of the body, can also be treated with hormone therapy.

Hormone therapy comes in a variety of forms for breast cancer. The majority of hormone therapy treatments either reduce the body's oestrogen levels or prevent oestrogen from promoting the growth of breast cancer cells.

Targeted drug therapy:

Targeted drug therapy involves administering drugs that are intended to (target) proteins on breast cancer cells that support their ability to proliferate, disseminate, and develop. Drugs that are specifically targeted either kill cancer cells or inhibit their growth. They can be administered intravenously (IV), subcutaneously (under the skin), or as a tablet, and they have different adverse effects than chemotherapy.

Some targeted treatment medications such as monoclonal antibodies, have many mechanisms of action against cancer cells. These medications may also be referred to as immunotherapy, because they strengthen the immune system.

Similar to chemotherapy, these medications are absorbed into the bloodstream and reach practically every part of the body, making them effective against malignancies that have spread to distant regions of the body. Sometimes, targeted medications succeed where chemotherapies fail. Some targeted medications can improve the efficacy of other forms of therapy.

Immunotherapy:

Immunotherapy is the use of drugs to strengthen a person's immune system so that it can more easily identify and eliminate cancer cells. Immunotherapy often targets particular immune system proteins to strengthen the immune response. The negative effects of these medications are distinct from those of chemotherapy.

The immune system's capacity to refrain from attacking healthy body cells is an essential component of it. It does this by activating proteins on immune cells known as "checkpoints," which must be switched on (or off) in order to initiate an immunological response. These checkpoints are sometimes used by breast cancer cells to avoid immune system attacks. Drugs that specifically target these checkpoint proteins aid in reestablishing the immune response to breast cancer cells.⁵

FAQs

How is Paget’s disease of the breast diagnosed?

The majority of people who have breast Paget disease also have malignancies in the same breast. To look for further breast changes, one or more of the imaging tests listed below could be performed:

• Diagnostic mammogram
• Breast ultrasound
• Breast MRI (magnetic resonance imaging) scan

Breast Paget disease is identified through a biopsy, which involves taking a small sample of breast tissue and closely examining it in a lab. The entire nipple may be removed in some circumstances. Only a biopsy can definitively determine whether it is cancer.⁶

How can I prevent from Paget’s disease of the breast?

Your chance of acquiring certain types of cancer, such as breast cancer, may be decreased by changing certain lifestyle variables, such as limiting the amount of alcohol you consume and making sure you exercise regularly.²

How common is Paget’s disease of the breast?

The typical age of diagnosis is 57, but both very young and very old persons have also been diagnosed with it. Paget's disease of the breast is present in less than 4% of cases of breast cancer.⁷

Who are at risks of Paget’s disease of the breast?

Paget's disease of the breast has no recognised cause, but there does seem to be a connection between the disease and an underlying ductal carcinoma.

Risk elements consist of:

• Age: As people get older, their risk of acquiring the illness rises.
• Benign breast disease history
• A personal breast cancer history
• A history of breast cancer in the family
• Thick breast tissue
• Obesity
• Radioactive contamination
• Race: Caucasian women are more likely to develop breast cancer.
• After menopause, using combined estrogen-progestin hormone replacement therapy.⁸

When should I see a doctor?

If you observe any alterations in your breasts. Make an appointment with your doctor if you see a lump in your breast, or if itching or skin irritation lasts more than a month.

Make a follow-up visit with your doctor if the condition you are being treated for in relation to a skin damage on your breast doesn't improve.³

Summary

Paget's disease of the breast is a rare disorder of the nipple-areola complex that is often associated with an underlying in situ or invasive carcinoma. The cause of Paget's disease of the nipple is uncertain. A change in sensation of the nipple-areola, such as itching and burning, is a common presenting symptom. Surgical biopsy is the diagnostic standard and therefore the diagnosis should always be confirmed by open (surgical) biopsy. The main treatment for pagets disease of the nipple is surgery. Other treatment modalities include radiotherapy, chemotherapy, hormone therapy, targeted drug therapy and immunotherapy.