Distinctive epidemiology and prognosis of de novo and secondary cutaneous angiosarcoma patients

Background Cutaneous angiosarcoma (cAS) is a rare and not well-established malignant neoplasm characterized by dismal prognosis. Treatment for cAS remains controversial. Objective From 1975 to 2016, a total of 872 patients with cAS (379 secondary cAS [s-cAS] patients and 493 de novo cAS [dn-cAS]) patients were identified from the Surveillance, Epidemiology, and End Results (SEER) database. Methods Survival analyses were performed utilizing Kaplan-Meier curves and Cox proportional hazards regression models. Results Both the number of s-cAS patients and dn-cAS patients climbed steadily over 42 years. There is a gradual increase in the proportion of s-cAS among whole cAS patients for each interval, ranging from 0% to 51.2%.Taking into account a combination of Kaplan-Meier and Cox regression methods, the survival of s-cAS is similar to dn-cAS. On multivariate analysis, older age, greater tumor size, primary site located at lower extremity, advanced stage, and no surgery were independent prognostic factors of decreased overall survival (OS) in both the s-cAS and dn-cAS groups. Conclusion Apparent increase in the number of s-cAS patients requires further attention. Surgery is still recommended for the treatment of both s-cAS and dn-cAS patients, and the requirement for treatment improvement is imperative.