Paget's disease of the breast epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Epidemiology

Paget's disease of the breast is a rare disease that corresponds to 1-4.3% of all breast cancers and is frequently associated with intraductal, in situ or invasive neoplasms.[1]

  • It is more prevalent in postmenopausal women, usually after the sixth decade of life, but it has also been reported in adolescent and elderly patients. It occurs in 1 – 4% of all female breast carcinoma cases and is invariably associated with underlying malignancy either overt or occult. The majority of these cases are invasive disease although 40–45% are associated with DCIS.[2]

Prognosis

  • Some factors indicate an unfavorable prognosis, among them:[1]
    • The presence of a palpable breast tumor
    • Enlarged lymph nodes
    • Histological type of breast cancer
    • Patients younger than 60 years
  • Lymph node involvement is correlated with median survival rates, reaching 75-95% when negative and 20 to 25% when positive.
  • In another study, the mean 10-year survival rate was estimated at 47% in cases with positive lymph nodes and 93% in those with negative lymph nodes.
  • When the disease occurs in males, the prognosis is poor, with an average 5-year survival rate of 20-30%.

References

  1. 1.0 1.1 Lopes Filho, Lauro Lourival; Lopes, Ione Maria Ribeiro Soares; Lopes, Lauro Rodolpho Soares; Enokihara, Milvia M. S. S.; Michalany, Alexandre Osores; Matsunaga, Nobuo (2015). "Mammary and extramammary Paget's disease". Anais Brasileiros de Dermatologia. 90 (2): 225–231. doi:10.1590/abd1806-4841.20153189. ISSN 1806-4841.
  2. Subramanian, Ashok; Birch, Hilary; McAvinchey, Rita; Stacey-Clear, Adam (2007). "Pagets disease of uncertain origin: case report". International Seminars in Surgical Oncology. 4 (1): 12. doi:10.1186/1477-7800-4-12. ISSN 1477-7800.