Mammary Paget's disease

Mammary Paget's Disease Mammary Paget's disease is a rare form of breast cancer that primarily affects the skin of the nipple and the areola. It is often associated with underlying ductal breast carcinoma. This article provides a comprehensive overview of Mammary Paget's disease, including its symptoms, diagnosis, treatment, and prognosis.

Symptoms

The symptoms of Mammary Paget's disease can be subtle and are often mistaken for benign skin conditions. Common symptoms include:

• Redness and flakiness of the nipple skin
• Itching or tingling sensation
• Yellowish or bloody discharge from the nipple
• A lump in the breast, which may indicate underlying breast cancer

Diagnosis

Diagnosing Mammary Paget's disease involves a combination of clinical examination and diagnostic tests. These may include:

• Biopsy: A skin biopsy of the affected area is crucial for confirming the diagnosis. The presence of Paget cells, which are large cells with clear cytoplasm, is indicative of the disease.
• Mammography and Ultrasound: These imaging techniques help identify any underlying breast cancer.
• Magnetic Resonance Imaging (MRI): MRI may be used to assess the extent of the disease and to detect any additional lesions.

Treatment

The treatment of Mammary Paget's disease typically involves a combination of surgery, radiation therapy, and sometimes chemotherapy. Treatment options include:

• Surgery: The primary treatment is surgical removal of the affected tissue. This may involve a lumpectomy or mastectomy, depending on the extent of the disease.
• Radiation Therapy: Often used after surgery to eliminate any remaining cancer cells.
• Chemotherapy: May be recommended if there is invasive breast cancer or if the cancer has spread to other parts of the body.

Prognosis

The prognosis for Mammary Paget's disease depends on several factors, including the presence and extent of underlying breast cancer. Early detection and treatment are crucial for a favorable outcome. The prognosis is generally better if the disease is confined to the nipple and areola without invasive cancer.

Epidemiology

Mammary Paget's disease is rare, accounting for 1-4% of all breast cancer cases. It predominantly affects women, although rare cases in men have been reported. The average age of diagnosis is around 50-60 years.

Pathophysiology

The exact cause of Mammary Paget's disease is not well understood. It is believed to originate from ductal carcinoma cells that migrate to the epidermis of the nipple. The presence of Paget cells in the epidermis is a hallmark of the disease.

History

Mammary Paget's disease was first described by Sir James Paget in 1874. He noted the association between changes in the nipple and underlying breast cancer, which led to the recognition of this distinct clinical entity.

See Also

• Breast Cancer
• Ductal Carcinoma
• Rare Diseases
• Paget, J. (1874). "On Disease of the Mammary Areola Preceding Cancer of the Mammary Gland." St. Bartholomew's Hospital Reports.
• National Cancer Institute. "Paget's Disease of the Breast." Retrieved from [1]

NIH genetic and rare disease info

• NIH info on Mammary Paget's disease

Mammary Paget's disease is a rare disease.