Clear Cell Meningioma in the Central Nervous System: Analysis of Surveillance, Epidemiology, and End Results Database

BACKGROUND: Clear cell meningioma (CCM) is a rare subtype of meningioma, accounting for approximately 0.2% of all meningiomas. The present study aimed to analyze the epidemiology and outcome of CCMs using the Surveillance, Epidemiology, and End Results (SEER) database. METHODS: Patients diagnosed with central nervous system CCM between 2004 and 2016 were identified from the SEER database. Descriptive analyses were performed to evaluate the distribution of patients and tumor-related characteristics. The survival analysis was performed using Kaplan-Meier curves. The Cox proportional hazards model was used for the univariate and multivariate analyses. RESULTS: The age-adjusted incidence rate was 0.032 per 1,000,000 person-years. The median age was 52 years. Most of the CCMs were intracranial CCMs that were larger than 3 cm. The overall cumulative survival rates at 1, 3, and 5 years were 97.6, 93.2, and 86.9%, respectively. The log‐rank test and Cox proportional hazards regression analysis revealed that age at diagnosis and primary site of the tumor were independent prognostic factors. CONCLUSION: CCM is an extremely rare entity with a favorable survival rate. CCMs usually affect patients during the fourth to fifth decades of life. Patients diagnosed at 21–60 years old and patients with spinal CCMs have a better prognosis.