Paget disease of the breast
- Michael S Sabel, MD
Michael S Sabel, MD
- Associate Professor of Surgery
- University of Michigan Medical School
- Donald L Weaver, MD
Donald L Weaver, MD
- Professor of Pathology
- University of Vermont College of Medicine
- Section Editors
- Anees B Chagpar, MD, MSc, MA, MPH, MBA, FACS, FRCS(C)
Anees B Chagpar, MD, MSc, MA, MPH, MBA, FACS, FRCS(C)
- Section Editor — Breast Surgery
- Associate Professor, Department of Surgery
- Yale University School of Medicine
- Daniel F Hayes, MD
Daniel F Hayes, MD
- Section Editor — Breast Cancer
- Professor of Medicine
- University of Michigan School of Medicine
- Lori J Pierce, MD
Lori J Pierce, MD
- Section Editor — Radiation Therapy
- Professor of Radiation Oncology
- University of Michigan School of Medicine
In 1874, Sir James Paget described 15 women with chronic nipple ulceration who all went on to develop cancer of the involved breast within two years . The ulceration was described as an eczema-like eruption on the nipple and areola with a copious clear yellowish exudate. Ultimately, this would become known as Paget disease of the breast (PDB) or mammary Paget disease. While Paget believed that the nipple changes were themselves benign, it was subsequently discovered that the characteristic cells within the epidermis of the nipple (Paget cells) were in fact malignant .
This topic review will discuss the clinical presentation, pathogenesis, pathology, diagnosis, and management of mammary Paget disease.
PDB is much less common than other presentations of breast cancer, accounting for only 1 to 3 percent of new cases of female breast cancer that are diagnosed annually in the United States [3-7]. PDB can occur in men; however, this is an extremely rare finding. Although cases have been described in patients ranging in age from 26 to 88, the peak incidence is between 50 and 60 [8-11].
Histologic (subclinical) evidence of PDB may be more frequent. In a series of 3000 consecutive mastectomy specimens, the incidence of clinical Paget disease was 0.7 percent, but histologic evidence of the disease was present in 4.9 percent of the mastectomies .
At least some epidemiologic data suggest that the incidence of PDB is decreasing over time . In a report of 1738 cases of PDB reported to the SEER database, the incidence of PDB decreased by 45 percent between 1988 and 2002, while the overall incidence of breast cancer increased over this same time period.
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- CLINICAL PRESENTATION
- DIAGNOSTIC WORKUP
- History and physical examination
- Differential diagnosis
- Skin biopsy and histology
- Mammography and ultrasonography
- Magnetic resonance imaging
- Biopsy of underlying abnormalities
- Epidermotropic theory
- Transformation theory
- PDB with a palpable mass or abnormal imaging
- PDB with no palpable mass or imaging abnormality
- Management of the axilla
- Alternative approaches
- Use of tamoxifen
- SUMMARY AND RECOMMENDATIONS