Demographic characteristics and survival outcomes of pediatric clear cell sarcoma of the kidney: A National Cancer Database retrospective study.

Abstract

10033 Background: Clear Cell Sarcoma of the Kidney, CCSK, is a rare malignancy diagnosed during childhood. Compared to other pediatric renal neoplasms, CCSK is particularly aggressive due to its propensity for bone metastasis, resulting in a poorer overall survival rate. CCSK has a higher incidence in males than females. Given its rarity, investigating diagnostic patterns may reveal important epidemiological insights. This study analyzed the demographic factors in CCSK patients from the National Cancer Database (NCDB). Methods: A retrospective National Cancer Database (NCDB) from 2004 to 2020 analyzed patients who had a histologically confirmed diagnosis of CCSK (ICD-O-3 8964). Descriptive statistics were used to analyze Demographic factors (age, sex, race, Hispanic status, educational attainment, insurance status, facility type, distance from facility, and Charles/Deyo score). Regression analysis was utilized to interpret incidence trends. Results: Between 2004 and 2020, the NCBD identified 237 patients with confirmed CCSK, indicating a stable incidence rate (R^2 = 0.0043). The median age at diagnosis was 3 years old (SD = 24.83; range, 0–86). Males comprised 56% of the cohort, and 44% were females. Most patients were White (78%) and non-Hispanic (79%). Nearly all primary tumors (99%) occurred in the kidney and renal pelvis, with a median tumor size of 120 mm (SD = 42.33; range, 13–202). Over half of patients (54%) had private insurance, while 27% were on Medicaid. The majority (93%) had a Charlson/Deyo comorbidity score of 0. Most patients (99%) did not receive palliative therapy. 71% of the patients underwent a surgical procedure at the primary site, 70% received radiotherapy, and 76% received it as part of their primary treatment. Most patients (90%) lived in metropolitan areas, and they resided a median distance of 15 miles (SD = 57.1; range, 0.5-377.3) from the reporting hospital. The two-year, five-year, and ten-year survival rates were 86%, 81%, and 76%, respectively, with a mean survival of 163 months. Conclusions: To the best of our knowledge, this is the first NCDB analysis of CCSK, addressing a significant knowledge gap. Nearly all CCSK cases originated in the kidney or renal pelvis, consistent with literature indicating the kidney as the primary site with a stable incidence over time. These are the first socioeconomic factors of CCSK patients that have been described in the literature: CCSK patients are more likely to be white and non-Hispanic, male, have private insurance, and live in metropolitan areas. Further research is essential to better understand how demographic and socioeconomic factors influence the diagnosis, treatment choices, and overall survival of patients.