Perivascular epithelioid cell tumors: A study of demographic and socioeconomic factors.
Authors: Khatib Rawaan , Nguyen Jacqueline , Grover Jasika , Chin Kat , Tandon Nikhita , Reddy Abhigna , Jahan Israa , Eldosougi Isra , Silberstein Peter T. , Lee Daniel , Demangone Michael , Rossi Anthony , Heise Craig , Hsia Beau 
Published: June 2024

e17594

Background: Perivascular epithelioid cell tumors (PEComas) are rare soft tissue tumors composed primarily of epithelioid cells and found most commonly in perivascular spaces. Previously, many different tumors, such as renal angiomyolipoma and leiomyosarcoma, were considered disparate conditions. However, the more recent identification of perivascular epithelioid cells common to these tumors has recategorized them as PEComas. Due to this, there is an increase in PEComa cases but development of curative treatments has not seen the same growth. Further investigation into the epidemiology of patients diagnosed with PEComas may improve treatment options and outcomes. Data obtained from the National Cancer Database (NCDB) were utilized to analyze the demographics of PEComa patients. Methods: A retrospective cohort study analyzed 114 individuals diagnosed with PEComa (ICD-8714-3) utilizing data from the 2018-2020 NCDB. The analysis used descriptive statistics to examine demographic factors including age, sex, race, Hispanic origin, educational attainment, insurance status, facility type, distance from facility, and Charlson/Deyo score. Regression analysis was used to interpret incidence trends. Results: A total of 114 patients were diagnosed with PEComa between the years 2018-2020, with a slight increase in diagnoses made each year (R2= 0.893). The average age of diagnosis was 53.1 years (SD = 15.2, range = 7-86) and women (89%) were more likely to be diagnosed than men (11%). Primary site of diagnosis was the uterus, NOS (22%). Primary treatment options were surgery (81%), chemotherapy (27%), and radiation (9%). Many individuals (83%) had no concurrent comorbidities (Charlson-Deyo comorbidity score = 0) and survival rates following treatment were 81% at 2 years and 63% at 5 years. The majority of patients identified as non-Spanish/non-Hispanic (83%) and White (71%). Most patients were treated in an academic/research program (49%), 33% qualified for the highest quartile of income earners ($74,063 or more), lived in a metropolitan area (61%), and were privately insured (63%). Patients lived on average 67.3 miles (SD=187.5) from the treatment facility. Conclusions: To the best of our knowledge, this is the first NCDB analysis on PEComa, which addresses a key knowledge gap within current literature regarding the topic. In concurrence with previous reports, female diagnoses predominated with the primary site of diagnosis in the uterus. The socioeconomic factors of PEComa patients have not been covered in the literature until now: patients are more likely to have a median income of $74,063 or more, be treated in an academic/research program, and live over 60 miles from the treatment facility. Additional research is essential to understand the impact of socioeconomic and demographic factors on diagnosis, treatments, and survival rates of patients with PEComa.