Paget's disease, mammary

=Paget's Disease of the Breast = Paget's disease of the breast, also known as mammary Paget's disease, is a rare type of cancer that typically affects the skin of the nipple and, often, the darker circle of skin around it, known as the areola. It is a rare manifestation of breast cancer, accounting for 1-4% of all breast cancer cases.

Symptoms

The symptoms of Paget's disease of the breast can be similar to those of other skin conditions, such as eczema or dermatitis, which can lead to misdiagnosis. Common symptoms include:

• Itching, tingling, or redness in the nipple and/or areola.
• Flaky or scaly skin on the nipple.
• Yellowish or bloody discharge from the nipple.
• A flattened or inverted nipple.
• A lump in the breast, which may indicate an underlying breast cancer.

Diagnosis

Diagnosis of Paget's disease of the breast typically involves a combination of:

• Mammography: An imaging test to look for underlying breast cancer.
• Biopsy: A sample of the skin and tissue is taken from the nipple and areola to look for cancer cells.
• Magnetic Resonance Imaging (MRI): May be used to get a more detailed view of the breast tissue.

Pathophysiology

Paget's disease of the breast is characterized by the presence of Paget cells, which are large cells with clear cytoplasm and prominent nuclei. These cells are found in the epidermis of the nipple and areola. The exact mechanism by which these cells appear is not fully understood, but it is believed that they originate from an underlying ductal carcinoma in situ (DCIS) or invasive breast cancer.

Treatment

Treatment for Paget's disease of the breast usually involves surgery, which may include:

• Mastectomy: Removal of the entire breast, often recommended if there is an underlying breast cancer.
• Breast-conserving surgery: Removal of the nipple and areola, along with a margin of surrounding tissue, if the cancer is localized.

Additional treatments may include:

• Radiation therapy: To destroy any remaining cancer cells.
• Chemotherapy: If there is invasive cancer or if the cancer has spread.
• Hormone therapy: If the cancer is hormone receptor-positive.

Prognosis

The prognosis for Paget's disease of the breast depends on several factors, including the presence and extent of any underlying breast cancer. Early detection and treatment are crucial for a better outcome.

Epidemiology

Paget's disease of the breast is rare, with a higher incidence in women over the age of 50. It is less common in men, but can occur.

History

The disease is named after Sir James Paget, who first described the condition in 1874. He noted the association between changes in the nipple and underlying breast cancer.

See Also

• Breast cancer
• Ductal carcinoma in situ
• Invasive breast cancer
• Paget, J. (1874). "On Disease of the Mammary Areola Preceding Cancer of the Mammary Gland." St. Bartholomew's Hospital Reports.
• National Cancer Institute. "Paget Disease of the Breast." Cancer.gov.

NIH genetic and rare disease info

• NIH info on Paget's disease, mammary

Paget's disease, mammary is a rare disease.