Paget's Disease of the Nipple

Paget's disease of the nipple is a rare form of breast cancer that primarily affects the skin of the nipple and, sometimes, the areola. It is characterized by the presence of Paget cells, which are malignant cells that are typically found in the epidermis of the nipple.

Causes and Risk Factors

The exact cause of Paget's disease of the nipple is not well understood, but it is often associated with an underlying breast cancer, usually ductal carcinoma in situ (DCIS) or invasive breast cancer. The risk factors for developing Paget's disease of the nipple include:

• Being female, as it is very rare in men
• Age, typically occurring in women over 50 years old
• Having a family history of breast cancer
• Previous radiation therapy to the chest area

Symptoms

The symptoms of Paget's disease of the nipple can be subtle and may resemble those of benign conditions, making diagnosis challenging. Common symptoms include:

• Eczema-like changes to the skin of the nipple, including redness, scaling, and crusting
• Itching or burning sensation in the nipple area
• Nipple discharge, which may be bloody or serous
• Flattening or inversion of the nipple
• A palpable mass or lump in the breast

Diagnosis

Diagnosing Paget's disease of the nipple involves a combination of clinical evaluation, imaging studies, and biopsy. The diagnostic workup may include:

• Clinical breast examination
• Mammography
• Ultrasound
• Magnetic resonance imaging (MRI)
• Breast biopsy, including fine-needle aspiration cytology or core needle biopsy
• Skin biopsy of the nipple-areolar complex

Treatment

The treatment for Paget's disease of the nipple usually involves surgery, and the goal is to remove the cancerous cells and any underlying breast cancer. The surgical options may include:

• Simple mastectomy
• Breast-conserving surgery (lumpectomy), followed by radiation therapy
• Skin-sparing mastectomy with reconstruction

In some cases, additional treatments such as chemotherapy, hormone therapy, or targeted therapy may be recommended, depending on the presence and characteristics of any underlying breast cancer.

Prognosis

The prognosis for Paget's disease of the nipple is generally good if caught early, with a high survival rate when treated appropriately. However, if left untreated or diagnosed at an advanced stage, the disease can spread to other parts of the body, leading to a poorer prognosis.