Prognosis in Cutaneous Paget's Disease

Mammary Paget's disease appears to be invariably associated with intraductal or parenchymal carcinoma of the breast; however, the nature of the extramammary form of the disease is still controversial.In a review of 145 patients with mammary Paget's disease and 10 patients with extramammary involvement, the authors found that (1) the average survival of patients in both groups was roughly equivalent; (2) regional or widespread metastatic involvement from underlying apocrine or regional carcinoma frequently occurs in extramammary Paget's disease; (3) metastatic lymphadenopathy reduces the survival rate by at least 50 per cent; (4) local recurrences seem the rule in extramammary involvement but are rare in mammary disease; and (5) metastatic carcinoma was the predominant cause of death in both groups of patients.