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Paget disease of the breast is a rare type of breast cancer involving the skin of the nipple and the areola (the dark circle around the nipple). Paget disease usually affects only one breast. In 80-90% of cases, it’s usually found along with either ductal carcinoma in situ (DCIS) or infiltrating ductal carcinoma (invasive breast cancer).
The skin of the nipple and areola often looks crusted, scaly, and red. There may be blood or yellow fluid coming out of the nipple. Sometimes the nipple looks flat or inverted. It also might burn or itch. Your doctor might try to treat this as eczema first, and if it does not improve, recommend a biopsy.
Most people with Paget disease of the breast also have tumors in the same breast. One or more of the following imaging tests may be done to check for other breast changes:
Paget disease of the breast is diagnosed by a biopsy, removing a small piece of the breast tissue and looking at it closely in the lab. In some cases, the entire nipple may be removed. Only a biopsy can show for sure that it is cancer.
Paget disease can be treated by removing the entire breast (mastectomy) or breast-conserving surgery (BCS) followed by whole-breast radiation therapy. If BCS is done, the entire nipple and areola area also needs to be removed. If invasive cancer is found, the lymph nodes under the arm will be checked for cancer.
If no lump is felt in the breast tissue, and your biopsy results show the cancer has not spread within the breast tissue, the outlook (prognosis) is excellent.
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Henry NL, Shah PD, Haider I, Freer PE, Jagsi R, Sabel MS. Chapter 88: Cancer of the Breast. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical Oncology. 6th ed. Philadelphia, Pa: Elsevier; 2020.
Jagsi R, King TA, Lehman C, Morrow M, Harris JR, Burstein HJ. Chapter 79: Malignant Tumors of the Breast. In: DeVita VT, Lawrence TS, Lawrence TS, Rosenberg SA, eds. DeVita, Hellman, and Rosenberg’s Cancer: Principles and Practice of Oncology. 11th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2019.
National Comprehensive Cancer Network (NCCN). Practice Guidelines in Oncology: Breast Cancer. Version 7.2021. Accessed at https://www.nccn.org/professionals/physician_gls/pdf/breast.pdf on August 30, 2021.
Sabel MS and Weaver DL. Paget disease of the breast. In Chen W, ed. UpToDate. Waltham, Mass.: UpToDate, 2021. https://www.uptodate.com. Last updated June 2, 2020. Accessed August 30, 2021.
Last Revised: November 19, 2021