Paget disease of the breast: A national retrospective analysis of the US population

Article type: Research Article

Authors: Sisti, Andrea^a | Huayllani, Maria T.^a | Restrepo, David J.^a | Boczar, Daniel^a | Advani, Pooja^b | Lu, Xiaona^c | Spaulding, Aaron C.^d | Ball, Colleen T.^e | McLaughlin, Sarah A.^f | Forte, Antonio J.^a;

Affiliations: [a] Division of Plastic Surgery, Mayo Clinic, Jacksonville, FL, USA | [b] Department of Hematology and Oncology, Mayo Clinic, Jacksonville, FL, USA | [c] Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT, USA | [d] Department of Health Science Research, Mayo Clinic, Jacksonville, FL, USA | [e] Division of Biomedical Statistics and Informatics, Mayo Clinic, Jacksonville, FL, USA | [f] Department of Surgery, Mayo Clinic, Jacksonville, FL, USA

Correspondence: [*] Corresponding author: Antonio J. Forte, Department of Surgery, Mayo Clinic, 4500 San Pablo Rd, Jacksonville, FL 32224, USA. Tel.: +1 904 953 2073; Fax: +1 904 953 7368; E-mail: ajvforte@yahoo.com.br

Abstract: BACKGROUND:Paget’s disease of the breast is rare. The National Cancer Database (NCDB) is one of the largest tumor databases in the United States. METHODS:We queried the NCDB to identify male and female patients with Paget’s disease of the breast who were treated from January 1, 2004, through December 31, 2015. No age limitations were applied. Descriptive statistical analysis and survival analysis were performed. RESULTS:We analyzed the demographic, disease, and treatment characteristics of 7,191 patients with Paget’s disease of the breast. The median (range) age was 64 (20–90) years. Only 2.1% of patients were men, and 85.4% were white. The most common treatment was complete mastectomy (65.3%), followed by partial mastectomy (30.3%). Tumor destruction was performed in 0.1% of patients, and 4.3% of patients did not undergo any surgery. We examined survival in the subset of 6,864 patients who were treated surgically with complete mastectomy or partial mastectomy. The 5-year survival rate after surgery was 82.5% (95% CI, 81.1%–83.9%). Factors associated with shorter survival were older age, black race/ethnicity, higher tumor grade, regional lymph node metastasis, higher cancer stage, metastatic cancer, and larger tumor size. CONCLUSIONS:Paget’s disease of the breast usually affects patients older than 60 years, and the most common treatment is complete mastectomy. The 5-year overall survival rate of patients with Paget’s disease of the breast is greater than 80% but is lower among older and black people.

Keywords: Paget’s disease, breast, NCDB, characteristics, survival

DOI: 10.3233/BD-200439

Journal: Breast Disease, vol. 39, no. 3-4, pp. 119-126, 2020