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Paget Disease of Nipple
Katie M. Davis, DO; Robin N. Sobolewski, MD; Wendie A. Berg, MD, PhD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Adenocarcinoma of epidermis of nipple
                • Associated with local inflammatory response, pruritus, pain and excoriation
                • Malignant cells extend to nipple surface through lactiferous ducts
                • Characteristic eczematoid skin changes of nipple

            IMAGING

            • General Features

              • Mammographic Findings

                • Ultrasonographic Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        • Image-Guided Biopsy

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Ogata H et al: Sonographic visualization of nipple blood flow can help differentiate Paget disease from benign eczematous nipple lesions. PLoS One. 13(5):e0197156, 2018
                                                2. Zhao Y et al: Clinicopathological characteristics and survival outcomes in Paget disease: a SEER population-based study. Cancer Med. 7(6):2307-18, 2018
                                                3. Broecker JS et al: An atypical presentation of Paget's Disease of the breast without nipple involvement: Case report and review of the literature. Pathol Res Pract. 213(11):1454-1456, 2017
                                                4. Elbendary A et al: Diagnostic criteria in intraepithelial pagetoid neoplasms: a histopathologic study and evaluation of select features in Paget disease, Bowen disease, and melanoma in situ. Am J Dermatopathol. 39(6):419-27, 2017
                                                5. Adams SJ et al: Paget's disease of the male breast in the 21st century: a systematic review. Breast. 29:14-23, 2016
                                                6. Helme S et al: Breast-conserving surgery in patients with Paget's disease. Br J Surg. 102(10):1167-74, 2015
                                                7. Wong SM et al: Modern trends in the surgical management of Paget's disease. Ann Surg Oncol. 22(10):3308-16, 2015
                                                8. Li YJ et al: Local Breast cancer recurrence after mastectomy and breast-conserving surgery for Paget's disease: A meta-analysis. Breast Care (Basel). 9(6):431-4, 2014
                                                9. Durkan B et al: Paget's disease of the nipple with parenchymal ductal carcinoma in situ is associated with worse prognosis than Paget's disease alone. Am Surg. 79(10):1009-12, 2013
                                                10. Moon JY et al: Malignant invasion of the nipple-areolar complex of the breast: usefulness of breast MRI. AJR Am J Roentgenol. 201(2):448-55, 2013
                                                11. Peters MS et al: Dermatopathology of the female breast. Am J Dermatopathol. 35(3):289-304; quiz 305-7, 2013
                                                12. Spanu A et al: Breast scintigraphy with breast-specific γ-camera in the detection of ductal carcinoma in situ: a correlation with mammography and histologic subtype. J Nucl Med. 53(10):1528-33, 2012
                                                13. Karakas C: Paget's disease of the breast. J Carcinog. 10:31, 2011
                                                14. Lim HS et al: Paget disease of the breast: mammographic, US, and MR imaging findings with pathologic correlation. Radiographics. 31(7):1973-87, 2011
                                                15. Marczyk E et al: The routine immunohistochemical evaluation in Paget disease of the nipple. Pol J Pathol. 62(4):229-35, 2011
                                                16. Nicholson BT et al: Nipple-areolar complex: normal anatomy and benign and malignant processes. Radiographics. 29(2):509-23, 2009
                                                17. Da Costa D et al: Common and unusual diseases of the nipple-areolar complex. Radiographics. 27 Suppl 1():S65-77, 2007
                                                18. Chen CY et al: Paget disease of the breast: changing patterns of incidence, clinical presentation, and treatment in the U.S. Cancer. 107(7):1448-58, 2006
                                                19. Frei KA et al: Paget disease of the breast: findings at magnetic resonance imaging and histopathologic correlation. Invest Radiol. 40(6):363-7, 2005
                                                20. Kawase K et al: Paget's disease of the breast: there is a role for breast-conserving therapy. Ann Surg Oncol. 12(5):391-7, 2005
                                                21. Anderson JM et al: Assessment of Her-2/Neu status by immunohistochemistry and fluorescence in situ hybridization in mammary Paget disease and underlying carcinoma. Appl Immunohistochem Mol Morphol. 11(2):120-4, 2003
                                                22. Hanna W et al: The role of HER-2/neu oncogene and vimentin filaments in the production of the Paget's phenotype. Breast J. 9(6):485-90, 2003
                                                23. Marshall JK et al: Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer. 97(9):2142-9, 2003
                                                24. Kothari AS et al: Paget disease of the nipple: a multifocal manifestation of higher-risk disease. Cancer. 95(1):1-7, 2002
                                                25. Polgar C et al: Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients. Cancer. 94(6):1904-5, 2002
                                                26. Bijker N et al: Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients. Cancer. 91(3):472-7, 2001
                                                27. Fu W et al: Paget disease of the breast: analysis of 41 patients. Am J Clin Oncol. 24(4):397-400, 2001
                                                28. Kollmorgen DR et al: Paget's disease of the breast: a 33-year experience. J Am Coll Surg. 187(2):171-7, 1998
                                                29. Desai DC et al: Paget's disease of the male breast. Am Surg. 62(12):1068-72, 1996
                                                30. Jamali FR et al: Paget's disease of the nipple-areola complex. Surg Clin North Am. 76(2):365-81, 1996
                                                31. Ikeda DM et al: Paget disease of the nipple: radiologic-pathologic correlation. Radiology. 189(1):89-94, 1993
                                                32. Classic articles in colonic and rectal surgery: Sir James Paget, 1814-1899, on disease of the mammary areola preceeding cancer of the mammary gland. Dis Colon Rectum. 23(4):280-1, 1980
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Pathology

                                                        • Clinical Issues

                                                          TERMINOLOGY

                                                          • Definitions

                                                            • Adenocarcinoma of epidermis of nipple
                                                              • Associated with local inflammatory response, pruritus, pain and excoriation
                                                              • Malignant cells extend to nipple surface through lactiferous ducts
                                                              • Characteristic eczematoid skin changes of nipple

                                                          IMAGING

                                                          • General Features

                                                            • Mammographic Findings

                                                              • Ultrasonographic Findings

                                                                • MR Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      • Image-Guided Biopsy

                                                                        DIFFERENTIAL DIAGNOSIS

                                                                          PATHOLOGY

                                                                          • General Features

                                                                            • Staging, Grading, & Classification

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Ogata H et al: Sonographic visualization of nipple blood flow can help differentiate Paget disease from benign eczematous nipple lesions. PLoS One. 13(5):e0197156, 2018
                                                                                              2. Zhao Y et al: Clinicopathological characteristics and survival outcomes in Paget disease: a SEER population-based study. Cancer Med. 7(6):2307-18, 2018
                                                                                              3. Broecker JS et al: An atypical presentation of Paget's Disease of the breast without nipple involvement: Case report and review of the literature. Pathol Res Pract. 213(11):1454-1456, 2017
                                                                                              4. Elbendary A et al: Diagnostic criteria in intraepithelial pagetoid neoplasms: a histopathologic study and evaluation of select features in Paget disease, Bowen disease, and melanoma in situ. Am J Dermatopathol. 39(6):419-27, 2017
                                                                                              5. Adams SJ et al: Paget's disease of the male breast in the 21st century: a systematic review. Breast. 29:14-23, 2016
                                                                                              6. Helme S et al: Breast-conserving surgery in patients with Paget's disease. Br J Surg. 102(10):1167-74, 2015
                                                                                              7. Wong SM et al: Modern trends in the surgical management of Paget's disease. Ann Surg Oncol. 22(10):3308-16, 2015
                                                                                              8. Li YJ et al: Local Breast cancer recurrence after mastectomy and breast-conserving surgery for Paget's disease: A meta-analysis. Breast Care (Basel). 9(6):431-4, 2014
                                                                                              9. Durkan B et al: Paget's disease of the nipple with parenchymal ductal carcinoma in situ is associated with worse prognosis than Paget's disease alone. Am Surg. 79(10):1009-12, 2013
                                                                                              10. Moon JY et al: Malignant invasion of the nipple-areolar complex of the breast: usefulness of breast MRI. AJR Am J Roentgenol. 201(2):448-55, 2013
                                                                                              11. Peters MS et al: Dermatopathology of the female breast. Am J Dermatopathol. 35(3):289-304; quiz 305-7, 2013
                                                                                              12. Spanu A et al: Breast scintigraphy with breast-specific γ-camera in the detection of ductal carcinoma in situ: a correlation with mammography and histologic subtype. J Nucl Med. 53(10):1528-33, 2012
                                                                                              13. Karakas C: Paget's disease of the breast. J Carcinog. 10:31, 2011
                                                                                              14. Lim HS et al: Paget disease of the breast: mammographic, US, and MR imaging findings with pathologic correlation. Radiographics. 31(7):1973-87, 2011
                                                                                              15. Marczyk E et al: The routine immunohistochemical evaluation in Paget disease of the nipple. Pol J Pathol. 62(4):229-35, 2011
                                                                                              16. Nicholson BT et al: Nipple-areolar complex: normal anatomy and benign and malignant processes. Radiographics. 29(2):509-23, 2009
                                                                                              17. Da Costa D et al: Common and unusual diseases of the nipple-areolar complex. Radiographics. 27 Suppl 1():S65-77, 2007
                                                                                              18. Chen CY et al: Paget disease of the breast: changing patterns of incidence, clinical presentation, and treatment in the U.S. Cancer. 107(7):1448-58, 2006
                                                                                              19. Frei KA et al: Paget disease of the breast: findings at magnetic resonance imaging and histopathologic correlation. Invest Radiol. 40(6):363-7, 2005
                                                                                              20. Kawase K et al: Paget's disease of the breast: there is a role for breast-conserving therapy. Ann Surg Oncol. 12(5):391-7, 2005
                                                                                              21. Anderson JM et al: Assessment of Her-2/Neu status by immunohistochemistry and fluorescence in situ hybridization in mammary Paget disease and underlying carcinoma. Appl Immunohistochem Mol Morphol. 11(2):120-4, 2003
                                                                                              22. Hanna W et al: The role of HER-2/neu oncogene and vimentin filaments in the production of the Paget's phenotype. Breast J. 9(6):485-90, 2003
                                                                                              23. Marshall JK et al: Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results. Cancer. 97(9):2142-9, 2003
                                                                                              24. Kothari AS et al: Paget disease of the nipple: a multifocal manifestation of higher-risk disease. Cancer. 95(1):1-7, 2002
                                                                                              25. Polgar C et al: Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients. Cancer. 94(6):1904-5, 2002
                                                                                              26. Bijker N et al: Breast-conserving therapy for Paget disease of the nipple: a prospective European Organization for Research and Treatment of Cancer study of 61 patients. Cancer. 91(3):472-7, 2001
                                                                                              27. Fu W et al: Paget disease of the breast: analysis of 41 patients. Am J Clin Oncol. 24(4):397-400, 2001
                                                                                              28. Kollmorgen DR et al: Paget's disease of the breast: a 33-year experience. J Am Coll Surg. 187(2):171-7, 1998
                                                                                              29. Desai DC et al: Paget's disease of the male breast. Am Surg. 62(12):1068-72, 1996
                                                                                              30. Jamali FR et al: Paget's disease of the nipple-areola complex. Surg Clin North Am. 76(2):365-81, 1996
                                                                                              31. Ikeda DM et al: Paget disease of the nipple: radiologic-pathologic correlation. Radiology. 189(1):89-94, 1993
                                                                                              32. Classic articles in colonic and rectal surgery: Sir James Paget, 1814-1899, on disease of the mammary areola preceeding cancer of the mammary gland. Dis Colon Rectum. 23(4):280-1, 1980