Paget Disease of the Breast: Comprehensive Health Information

Paget Disease of the Breast: A Comprehensive Guide

General Information

Paget disease of the breast is a rare type of cancer, accounting for approximately 1-4% of all breast cancer diagnoses, as per the National Cancer Institute. The disease typically begins on the nipple and often spreads to the surrounding areola (the darker skin around the nipple). In many patients, Paget disease is also found in conjunction with an underlying tumour or tumours within the affected breast.

While diagnosis can sometimes be delayed because its symptoms often mimic common skin conditions like eczema or dermatitis, the prognosis is good. If the disease is caught early, the chances for a complete cure are high.

Symptoms

If you notice any persistent changes to your breasts, you must consult your doctor immediately. The signs and symptoms of Paget disease of the breast may include:

  • Eczema-like changes to the skin of the nipple and/or areola, such as crusting, thickening, and flakiness.
  • An open sore or ulcer on the nipple and/or areola.
  • A persistent sensation of tingling or burning in the nipple and/or areola.
  • A nipple that becomes flattened or turns inward (inverted).
  • Discharge from the nipple that is yellow or bloody.
  • The presence of a lump or lumps in the same breast as the affected nipple.

These symptoms do not automatically mean you have breast cancer, but discussing them with a doctor is essential as they may signal other health issues.

Diagnosis

If a patient presents with suspicious symptoms or has an abnormal screening mammogram result, a thorough investigation is required to confirm a diagnosis. This process typically begins with a physical examination and is followed by imaging and a biopsy.

Physical Exam

A doctor will manually check your breasts and armpit for any lumps, masses, or abnormalities.

Imaging Exams

These are used to take a closer look at the breast tissue. The most common procedures are:

  • Mammogram: X-ray pictures of the breasts are taken from different angles to look for signs of cancer.
  • Ultrasound: A device uses high-energy sound waves to create a picture (sonogram) of the tissue.
  • Occasionally, doctors may also use magnetic resonance imaging (MRI) or other specialised examinations.

Biopsy

If imaging suggests a suspicious mass or skin thickening, a tissue sample is needed for a definitive diagnosis.

  • For breast cancer, an image-guided core needle biopsy is often performed during the initial imaging session to expedite the diagnosis.
  • For suspected Paget disease, if initial deeper biopsies are clear, a direct biopsy of the nipple and/or areola tissue will be performed to check for cancerous cells.
  • If cancer is confirmed, additional images and biopsies may be needed to determine the exact extent of the disease, including whether it has spread to nearby lymph nodes.

Molecular Diagnosis

The cancer cells will be analysed to determine their molecular receptor subtype. Understanding this subtype is crucial for developing a comprehensive, personalised treatment plan.

Treatment

The treatment approach for Paget disease of the breast is similar to that for other breast cancers, but with a specific surgical distinction. Treatment plans are personalised and often involve a combination of surgery, chemotherapy, and/or radiation therapy. Other advanced options like targeted therapy and angiogenesis inhibitors may also be included.

Surgery

All surgical procedures for Paget disease—whether a lumpectomy (removal of the tumour and surrounding tissue) or a mastectomy (removal of the entire breast)—require the removal of the nipple and areola.

Lumpectomy

This procedure is typically for small, early-stage tumours. It is often an outpatient procedure followed by radiation therapy.

Mastectomy

This involves removing the entire breast. Breast reconstruction can often be performed at the same time. A double mastectomy (removing both breasts) may be recommended for patients at a very high risk of new cancer development due to genetic or family history (e.g., a BRCA mutation).

Lymph Node Removal

Surgeons often remove nearby lymph nodes to examine them for cancer cells. This information helps assess the risk of the disease spreading and determines the necessity of further treatment.

Systemic and Radiation Therapies

Chemotherapy

Uses potent drugs to directly kill cancer cells, control their growth, or relieve pain. It may be given before surgery to shrink the tumour or after surgery to kill remaining cells.

Radiation Therapy

Uses powerful energy beams to destroy cancer cells. It can be used before surgery to shrink tumours or after a lumpectomy or mastectomy to eliminate any residual cancer cells. Treatment courses, which involve daily sessions, typically last between one to six weeks, depending on the specific situation. Advanced techniques include 3D conformal radiation therapy, Intensity-Modulated Radiation Therapy (IMRT), Volumetric Arc Therapy (VMAT), and specialised treatments like Stereotactic Body Radiation Therapy and Stereotactic Radiosurgery (often for cancer that has spread to the brain).

Targeted Therapy

These therapies interfere with specific molecules (e.g., proteins) that cancer cells rely on to survive and multiply.

  • Endocrine Therapy (Hormone Therapy): Given to patients with hormone receptor-positive breast cancer to prevent recurrence or disease progression.
  • HER2-Positive Targeted Therapies: Specific targeted drugs are given to patients with HER2-positive breast cancer.
  • Angiogenesis Inhibitors: These drugs disrupt the process of angiogenesis, which is how tumours create new blood vessels to increase their blood supply and grow rapidly.

Expert Care at SSCHRC

Choosing a specialist team with expertise is essential for treating a rare condition like Paget disease. The Breast Center at SSCHRC is one of the largest and most active breast centre, giving our team unmatched experience in treating Paget disease. At SSCHRC, all breast cancer patients benefit from a coordinated, multidisciplinary approach, with a dedicated breast surgeon, medical oncologist, and radiation oncologist working together to plan your personalised care. Our dedicated specialists use the most effective and least-invasive techniques to ensure the best possible medical and cosmetic outcomes.

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