What Is Paget's Disease of the Nipple?

Paget’s disease, also called Paget’s disease of the nipple or mammary Paget’s disease (not to be confused with Paget's disease of the bone), is a rare form of breast cancer that accounts for about 1% of all breast cancer cases. Paget’s disease affects the nipple and often spreads to the areola. Patients diagnosed with Paget’s disease are usually found to have tumors in the same breast—either ductal carcinoma in situ or invasive breast cancer.

Causes

While the causes of Paget’s disease are not well understood, a widely accepted theory is that cells from a cancer elsewhere in the breast travel through the milk ducts and begin to affect the nipple. This would explain why additional cancers are frequently found in Paget’s disease patients. However, because additional tumors are not always found, it is also possible that the cells of the nipple become cancerous on their own.

Symptoms

Paget’s disease may cause the following symptoms:

• Flaky, crusty, red, or thickened skin around the nipple or areola
• Burning, tingling, or itching
• Pain or heightened sensitivity
• Flattened or inverted nipple
• Yellowish or bloody nipple discharge

Because Paget’s disease is relatively rare, some symptoms are often mistaken for symptoms of more common skin ailments like eczema or dermatitis.

Diagnosis

If Paget’s disease is suspected, many doctors will begin by performing a physical exam. This can help to identify other palpable cancers, which exist in as many as 50% of patients with Paget’s disease. You will then likely undergo a mammogram, which a radiologist will examine for evidence of cancer in the nipple and elsewhere in the breast. Additional MRI or ultrasound imaging may also be ordered. To diagnose Paget’s disease, a biopsy will be taken of the nipple. This can take a few different forms, from a surface biopsy, which involves the scraping of cells from the nipple’s surface, to procedures that take larger amounts of tissue. Ask your doctor about any questions you have about the biopsy that’s ordered for you.

Treatment

Depending on the extent of the cancer, the presence or absence of additional cancers, and lymph node involvement, the treatment for Paget’s disease can range from breast-conserving surgery, which includes removal of the nipple and areola followed by radiation treatment, to mastectomy. After mastectomy, radiation treatment, chemotherapy, and/or hormone therapy may also be recommended.

Prognosis

The prognosis for Paget’s disease patients depends largely on whether an additional palpable breast tumor is present. Among patients with no palpable tumor prior to surgery, the survival rate is very high—reported at 92–94% at 5 years and 82–91% at 10 years. The presence identifiable underlying tumors has a significant impact on prognosis, however, with patients surviving five years at a rate of 38–40% and 22–33% surviving 10 years. As with any type of breast cancer, early detection, prompt treatment, and healthy habits are the keys to having the best possible chance of long-term survival.

At Iowa Radiology, we understand that concerns about your health are stressful. We strive to make our patients as comfortable as possible by answering questions, sharing information, and providing exceptional patient care. We are designated by the American College of Radiology as a Breast Imaging Center of Excellence, indicating that we meet the organization’s high standards for imaging quality and safety as well as our expertise in mammography, breast ultrasound, breast MRI, and breast biopsy. Contact us with any questions you have about our services or facilities or to schedule your annual mammogram.

The information contained in the Iowa Radiology website is presented as public service information only. It is not intended to be nor is it a substitute for professional medical advice. You should always seek the advice of your physician or other qualified healthcare provider if you think you may have a medical problem before starting any new treatment, or if you have any questions regarding your medical condition. Iowa Radiology occasionally supplies links to other web sites as a service to its readers and is not in any way responsible for information provided by other organizations.

Sources

"Paget Disease of the Breast." Cancer.gov. National Cancer Institute, 10 April 2012. Web. 4 Nov 2016.

"Paget's Disease of the Breast." MayoClinic.org. Mayo Foundation for Medical Education and Research, 29 March 2016. Web. 4 Nov 2016.

"Paget's Disease of the Nipple." BreastCancer.org. 23 June 2016. Web. 4 Nov 2016.

"Paget's Disease of the Nipple." WebMD.com. WebMD, 24 July 2016. Web. 4 Nov. 2016.