Clinical characteristics and survival outcomes of perianal Paget's disease: A SEER population-based study*

Abstract

Abstract Objective The aim of the study was to analyze the clinical features of patients with perianal Paget's disease (PPD) and investigate prognosis risk factors. Methods The SEER*Stat software was used to identify 116 PPD patients from 1975 to 2015 in the SEER research database. The Kaplan-Meier method was used to conduct a univariate analysis for PPD patients. The differences in survival rates were evaluated using the log-rank test. The differences in the clinicopathological features of PPD patients with or without anorectal carcinoma were compared using the chi-square test. Results The median survival time of PPD patients was 44 months. The median age of onset was 73 years old. The 43.10% of the patients were alive at the end of follow up, and only 12.93% of the patients died of PPD. Elderly (age > 70 years; χ2 = 9.453, P = 0.002), poor differentiation (χ2 = 46.557, P = 0.000) and abdominal perineal resection (APR; χ2 = 46.557, P = 0.000) were unfavorable risk factors of prognosis. Nearly 50% of PPD had combined with other malignancies, and over 22.41% of those had multiple primary neoplasms (3 or more). PPDs predisposed concurrent malignancy, and 48.21% of PPD patients with other malignancies combined with anorectal carcinoma in the study. Stage (χ2 = 10.127, P = 0.018), and surgical method (χ2 = 12.245, P = 0.007) were statistically significant in the PPD patients with or without anorectal carcinoma. The 16.07% of patients had multiple lesions of Paget's. Conclusion Patients with PPD have a favorable survival, while the disease-specific mortality is low. Diagnosed age, differentiation, and surgical methods were the influence factors of prognosis in PPD patients. PPDs with anorectal carcinoma is of most important in further investigation.