Can Paget's Disease Occur in Males?
Yes, Paget's disease definitively occurs in males, though it is significantly rarer than in females.
Paget's Disease of Bone in Males
Paget's disease of bone is a well-established risk factor for osteosarcoma development in both sexes. 1 The disease affects males with a male-to-female ratio of approximately 1.4:1 for associated osteosarcoma, indicating males are actually slightly more commonly affected when this complication develops. 1
- Age distribution: Paget's disease of bone contributes to a second peak of osteosarcoma incidence in the seventh and eighth decades of life, affecting both men and women. 1
- Clinical significance: When osteosarcoma arises in the context of Paget's disease, it represents a secondary osteosarcoma with distinct epidemiologic characteristics compared to primary disease. 1
Paget's Disease of the Breast (Mammary Paget Disease) in Males
Mammary Paget disease occurs in males but is exceedingly rare:
- Incidence: Male mammary Paget disease comprises only 1.45% of all male breast cancers (compared to 0.68% of female breast cancers), and males represent only 2.1% of all Paget's disease of the breast cases. 2, 3
- Presentation: Males typically present in the fifth and sixth decades with ulceration, eczematous changes, nipple discharge, bleeding, itching, and induration of the nipple-areolar complex. 2, 4
- Diagnostic delay: There is frequently a delay in diagnosis because patients are initially treated for a presumed rash rather than undergoing early biopsy. 2, 4
- Associated malignancy: At presentation, approximately 50% of male patients have a palpable breast mass, positive lymph nodes, or both, with underlying ductal carcinoma in situ or invasive ductal carcinoma present in most cases. 2, 4
Prognosis in Males
The 5-year survival for males with mammary Paget disease is worse than females: 20-30% in males versus 30-50% in females. 4 The overall 5-year survival rate after surgical treatment exceeds 80%, but this is lower among older patients and black individuals. 3
Treatment Approach
Current treatment for male mammary Paget disease involves modified radical mastectomy or radical mastectomy for Stage I and II tumors, with adjuvant chemotherapy, radiation, and tamoxifen used depending on nodal and receptor status. 4
Important Clinical Pitfalls
- Do not dismiss nipple changes as simple dermatitis in male patients—early biopsy is critical to identify underlying malignancy. 2, 4
- Always consider underlying carcinoma when Paget's disease is diagnosed, as the superficial lesion may be the only sign of ductal carcinoma. 5, 4
- Differential diagnosis must include malignant melanoma, Bowen's disease (intraepithelial squamous cell carcinoma), eczema, and squamous cell carcinoma, which can be distinguished by specific cell staining and tumor markers. 5, 4