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23-08-2024 Team Medicover Infectious-Diseases

Paget's Disease of the Breast: Expert Guide

Paget’s disease of the breast is a rare form of breast cancer that mainly affects the nipple and areola (the darker skin surrounding the nipple). It was first identified by Sir James Paget in 1874 and remains an essential diagnosis for early breast cancer detection.

Regular breast health check-ups and awareness of changes in your nipple or breast skin can help with early detection.

What Is Paget's Disease of the Breast?

Paget's disease of the breast, also known as Paget's disease of the nipple, is a rare form of breast cancer that manifests on the nipple and surrounding areola.

The condition is characterized by Paget cells, large, abnormal cells found in the epidermis of the nipple.

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Stages of Paget's Disease Breast

Understanding the stages of Paget's breast disease can help patients and their families make informed decisions about care and treatment. Like other types of breast cancer, Paget's breast disease is staged based on the extent of the cancer, its spread, and the involvement of nearby tissues or lymph nodes.

Here’s a simple breakdown of the stages of Paget's breast disease:

Stage 0 (In Situ): Cancer cells are present in the nipple and may involve nearby ducts but have not spread to surrounding breast tissue. This is also called Paget’s disease in situ.
Stage 1: Cancer is small (less than 2 cm) and limited to the breast. Nearby lymph nodes are not affected.
Stage 2: Tumor size is larger (2-5 cm), or cancer may have spread to nearby lymph nodes under the arm.
Stage 3: Cancer has spread to multiple lymph nodes or chest wall, indicating more advanced Paget's disease breast.
Stage 4: Cancer has spread to distant organs like lungs, bones, or liver, representing metastatic Paget’s disease breast.

Paget's Disease of the Breast Symptoms

Recognizing the symptoms of Paget’s disease breast early can help with timely diagnosis and treatment. While the symptoms may resemble common skin conditions like eczema, they persist and worsen over time.

Redness and irritation of the nipple and areola
Persistent itching or tingling sensation in the nipple
Flaky, scaly skin on the nipple, which may resemble a rash
Thickening of the skin around the nipple area
Yellowish or bloody discharge from the nipple
Nipple inversion (when the nipple turns inward)
A lump within the breast, which could indicate underlying breast cancer

Early detection of these symptoms is critical for timely diagnosis and treatment.

Paget's Disease of the Breast Causes

While the exact causes of Paget’s disease of the breast are not fully understood, experts believe that most cases are linked to underlying breast cancer, either ductal carcinoma in situ (DCIS) or invasive breast cancer.

Underlying Breast Cancer: In most cases, Paget cells travel from a tumour inside the breast to the nipple and areola.
Cellular Changes: Abnormal changes in the milk ducts' cells can extend toward the nipple surface, leading to Paget’s disease.
Genetic Factors: People with genetic mutations like BRCA1 or BRCA2 may have a higher chance of developing Paget's disease of the breast.
Hormonal Influence: Changes in hormones related to aging or menopause could contribute to cellular abnormalities in the breast.
Chronic Inflammation: Prolonged irritation or inflammation of the nipple could trigger abnormal cell development over time.

Risk Factors of Paget's Disease

Age: Most cases occur in women over the age of 50.
Family History: A family history of breast cancer can elevate the risk.
Genetic Mutations: Mutations in genes such as BRCA1 and BRCA2 are associated with a higher risk of breast cancer, including Paget's disease.
Previous breast surgery or radiation therapy: If you’ve had surgery or radiation for any breast condition in the past, your risk may slightly increase.
Hormonal factors: Long-term use of hormone replacement therapy (HRT) after menopause can contribute to risk.
Lifestyle factors: Being overweight, drinking alcohol regularly, and leading a sedentary lifestyle may also increase the likelihood of Paget’s disease of the breast.

Diagnosis Paget's Disease of the Breast

Early and accurate diagnosis of Paget's disease is essential for effective treatment. Diagnostic procedures typically involve a combination of clinical examination, imaging, and biopsy.

Clinical Examination

Paget’s disease can be diagnosed primarily from a complete physical examination by a doctor who will look for visible signs. The doctor will check the nipple and areola for any changes.

Imaging Tests

Breast imaging tests like mammography and ultrasound are used to uncover hidden breast cancer. These tests visualize the internal structure of the breast and help identify any suspicious areas.

Biopsy

A definitive diagnosis is made from a biopsy, in which a small tissue sample from the nipple or breast is examined under a microscope. Paget cells are present in the diagnostic presence .

Paget's Disease of the Breast Treatment Options

Treatment for Paget's disease of the breast often involves a combination of surgery, radiation therapy, and medication.

Surgical Treatment

Surgery is the most common treatment for Paget's disease.

Mastectomy: Removal of the entire breast, often recommended if there is invasive cancer.
Breast-Conserving surgery: Also known as lumpectomy, this involves removing only the affected tissue while preserving the rest of the breast. This is typically followed by radiation therapy.

Radiation Therapy

Radiation therapy is often used after breast-conserving surgery to destroy any remaining cancer cells. It may also be recommended for patients who are not candidates for surgery.

Systemic Treatments

Systemic treatments such as chemotherapy, hormone therapy, and targeted therapy may be used depending on the cancer's characteristics. These treatments help kill cancer cells throughout the body and prevent recurrence.

Paget's Disease of the Breast Prognosis

The prognosis of Paget's disease of the breast is heavily dependent on the stage of diagnosis and the presence of any underlying invasive cancer.

Long-Term Outlook

Patients with Paget's disease and no associated invasive cancer tend to have a favourable prognosis. At the same time, regular monitoring and follow-up is needed to identify any recurrence or disease spread.

Recurrence and Management

There may be a risk of return, especially if the disease is linked to invasive cancer. Long-term management can involve routine mammograms, physical examinations, and even additional treatments should a recurrence occur.

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Conclusion

Even though the condition is rare, recognizing its symptoms, risk factors, and treatment options can significantly reduce the risk of severe outcomes. If you notice any of these changes persisting, speak to a healthcare professional who can conduct a proper examination.

Frequently Asked Questions

The prognosis for Paget's disease of the breast is relatively favourable. When no palpable mass is detected and a physician surgically removes the cancer, the 10-year survival rate for individuals with Paget's disease of the breast stands at 82%.

No, Paget's disease is not an autoimmune disease. It is a chronic bone disorder characterized by abnormal bone growth and remodelling.

The survival rate depends on cancer stage. Early diagnosis leads to better outcomes, with 5-year survival rates around 80-90%.

Most cases link to underlying breast cancer, but not always. Tests confirm if invasive cancer is present.

Paget’s affects the nipple directly, linked to cancer. Eczema affects the skin around the nipple and isn’t cancer-related.

It’s rare but possible. Most cases occur in one breast. Regular screenings help detect any changes early.

It can cause itching, burning, and tenderness. Pain varies by person and stage of the disease.