Are there clinical and subclinical/pathological forms of Paget's disease of the breast?

Introduction: Breast disease management has changed over recent decades, related to molecular subtype, oncoplastic surgery and targeted therapies. Nevertheless, literature on Paget's disease of the breast (PDB), initially described as a clinical entity and now considered a multifocal/multicentric disease. Methods: PDB was classified as clinical in the presence of areolar abnormalities and as subclinical/pathological in all other cases. Clinical and prognostic data were evaluated and compared between the different presentation forms. Statistics comprised descriptive analysis, inter-group comparison (chi-square and Mann-Whitney tests) and overall and cancer-specific survival rates (Kaplan-Meier method and the log-rank test). Results: Of 85 patients included in this series, PDB was clinical in 58.8%. Overall, 27.1% had stage 0 and 92.9% had multifocal/multicentric disease. Most patients (83.5%) had the HER2 or luminal HER2 molecular subtype. Patients with clinical PDB had a higher rate of in situ disease (p=0.028) and were more likely to undergo breast-conserving surgery (p<0.001). Most of the 43 patients with HER2 invasive disease received anti-HER therapy. Mean follow-up time was 71.2 ± 43.3 months. Cancer-specific actuarial survival at 60 and 120 months was 92.3% and 83.1%, respectively. Survival was unaffected by the clinical form of PDB (p=0.275), anti-HER therapy (p=0.509) or oncoplastic surgery (p=0.821). Conversely, clinical stage affected survival significantly (p ≤ 0.001). Conclusion: PDB is a rare condition associated with multifocality/multicentricity and HER2 overexpression. Cases of clinical disease and those of subclinical/pathological disease differ significantly. Further studies are required to evaluate the clinical/areolar disease and the impact of advances in breast disease management on PDB.

Oncoplastic surgery for Paget's disease of the breast.

Introduction: Paget's disease of the breast (PDB) is a rare nipple entity associated with multifocality. Due to its location, resection of the entire nipple-areolar complex is necessary. Historically central quadrantectomy and mastectomy have the surgical treatments of choice. The feasibility of oncoplastic breast surgery (OBS) for PDB is unknown. Methods: This was a retrospective study performed in a Brazilian oncological hospital. We evaluated the factors related to the performance of OBS in PDB. In addition, the impact of OBS on local recurrence and survival was analysed. Comparisons were made between groups using the chi-square test, Mann-Whitney U test, and Kaplan-Meier method. To assess the impact factor of the variables on the performance of OBS, logistic regression was performed. Results: Eighty-five patients were evaluated. OBS was performed in 69.4% (n=59), and of these, 16 (27.2%) were symmetrized with contralateral surgery. Mastectomy without reconstruction was performed in 28.3% of the patients. The primary procedure performed was mastectomy with reconstruction (n=38; 44.7%), and the preferential technique for immediate reconstruction was skin-sparing mastectomy with prosthesis; for late reconstruction, the preferred technique was using the latissimus dorsi. Breast conserving-surgery was performed in 27.0% (n=23), primarily using the plug-flap technique (OBS). Age was associated with the use of OBS; as patients aged 40-49 exhibited a higher rate of OBS (p = 0.002; odds ratio 3.22). OBS did not influence local recurrence (p=1.000), overall survival (p=0.185), or cancer-specific survival (p=0.418). Conclusion: OBS improves options related to surgical treatment in PDB without affecting local recurrence or survival rates.