Title : A national cancer database analysis of pleomorphic dermal sarcoma
Abstract:
Purpose: Pleomorphic dermal sarcoma (PDS) is a mesenchymal neoplasm thought to lie within the spectrum of atypical fibroxanthoma with more aggressive behavior. It tends to have deep invasion, tumor necrosis, and perineural involvement. The tumor has a predilection for males older than 75 and primarily involves the head and face. However, PDS remains a relatively rare tumor and no recent studies have examined its incidence and mortality on a national scale in the last 20 years. As such, the purpose of this study is to examine changes in the incidence of PDS over the last 40 years, as well as risk factors associated with mortality from PDS.
Design: An analysis was conducted of data procured from the National Cancer Institute’s SEER-18 registry. All cases of pleomorphic dermal sarcoma in between the years 1979-2019 was collected. Factors associated with the diagnosis including age at diagnosis, survival, cause of death, and patient characteristics were also procured. A descriptive (demographic) analysis was done on patient characteristics including age, race, and gender. SEER*Stat was used to further create a Kaplan-Meier survival curve. Lastly, R* was used to create a multivariable cox regression for 15-year-disease specific outcomes.
Findings: The net incidence between 1979-2019 was 0.508 per 100,000 cases. In the last 40 years, 45.3% of patients diagnosed with PDS have been between the ages of 60-79, 62.4% have been male, and 87.5% have been white. Mortality rates are lower in the periods between 2000-2018 as compared to 1981-1999. Moreover, the patient characteristics associated with mortality on multivariable cox regression include older age, non-white race, and non-head/neck site of involvement.
Summary: As shown by this national SEER database analysis, both the incidence of and mortality from PDS have been decreasing in the last 20 years. Most patients diagnosed with PDS in the last 40 years have been older, male, and white. Mortality rates, however, are highest in patients who are older, non-white, and those with malignancy of non-head/neck areas. Overall, this examination provides an update on the national incidence of PDS, as well as patient characteristics associated with development and mortality from PDS.
