Overall and cancer-specific survival in patients with breast Paget disease: A population-based study

Paget disease of the breast is an uncommon malignant tumor with an inferior outcome. Therefore, establishing nomograms to predict the survival outcomes of breast Paget disease patients is urgent. Clinicopathological and follow-up data of breast Paget disease patients diagnosed between 2010 and 2016 were retrieved through the Surveillance, Epidemiology, and End Result (SEER) database. The significant factors were screened out, and then those factors were utilized to build two valuable nomograms. The discriminative ability of nomograms was investigated using concordance-index (C-index), while the predictive accuracy and benefits were evaluated using calibration curves and decision curve analysis. Finally, a total of 417 breast Paget disease patients were enrolled. Tumor grade, histological type, American Joint Committee on Cancer (AJCC) stage, surgery, chemotherapy, and marital status were confirmed as independent overall survival (OS)-related factors; tumor grade, histological type, AJCC stage, and age were associated with independent cancer-specific survival (CSS)-related factors. The values of the C-index for OS nomogram acquired were 0.827 and 0.745 for training and validation cohorts, respectively. Meanwhile, the corresponding values of the C-index to CSS nomogram were 0.890 and 0.655, respectively. The calibration curves and decision curve analysis indicated that both nomograms had an excellent performance. Finally, the nomogram-based risk stratification system indicated that all breast Paget disease patients could be classified into low- and high-risk groups and showed distinct outcomes. In conclusion, two valuable nomograms incorporating various clinicopathological indicators were established for breast Paget disease patients. These prognostic nomograms provide accurate prognostic assessment for breast Paget disease patients and help clinicians select appropriate treatment strategies.

Rectal Phenotype of Perianal Paget Disease: Rare Concomitant Phenomena

Aim: Classically, ‘Paget disease’ refers to a distinct histological pattern in breast carcinoma. Here, we review the clinicopathological features of anorectal adenocarcinoma with ‘pagetoid’ spread. Materials and Methods: Histological and immunohistochemical records for 11 cases of anorectal adenocarcinoma with pagetoid spread among 958 Japanese patients with primary rectal/anal carcinoma were reviewed. Results: Grossly, nine of 11 cases had areas of invasive carcinoma: Tubular adenocarcinoma in eight and neuroendocrine carcinoma in one. Pagetoid components were positive for cytokeratin 7 in eight cases, cytokeratin 20 and caudal type homeobox 2 in all 11 cases, and p63 in one case, but were negative for estrogen receptor, progesterone receptor, human epidermal growth factor receptor 2 (HER2), gross cystic disease fluid protein-15, and GATA binding protein 3. Conclusion: The prevalence of perianal Paget disease in this series was 1.1%, with two cases of genuine perianal Paget disease with a rectal phenotype without invasive carcinoma. The rectal phenotype of perianal Paget disease may not be associated with HER2 overexpression.