Clinical Characteristics and Long-Term Outcomes of Atypical Choroid Plexus Papillomas: A Single-Center Experience of 40 Cases

BACKGROUND AND OBJECTIVES: Atypical choroid plexus papillomas (ACPPs) are exceedingly uncommon. The treatment strategies for managing this condition remain a topic of debate. The main aim of this study was to elucidate the clinical features of ACPPs, scrutinize the outcomes of surgical interventions, and strive to formulate treatment protocols grounded in our institutional expertise. METHODS: We retrospectively analyzed a cohort of 40 cases of ACPPs that underwent surgical treatment at our institution between January 2011 and March 2023. Clinical data from these cases were collated, and prognostic risk factors were evaluated using Cox proportional hazards model and Kaplan-Meier method. RESULTS: The cohort consists of 40 cases, including 23 pediatric and young adult patients (≤35 years) and 17 middle-aged and older patients (>35 years). Among pediatric and young adult patients, the mean age was 9.9 ± 12.3 years (16 males, 7 females). Gross total resection was achieved in 19 (82.6%) patients. After a mean follow-up of 102.9 ± 43.7 months, 1 (4.3%) patient experienced recurrence and 1 (4.3%) died. Among middle-aged and older patients, the mean age was 50.8 ± 8.0 years (5 males, 12 females). Gross total resection was performed in 13 (76.5%) patients. After a mean follow-up of 65.7 ± 36.6 months, 5 (29.4%) patients experienced recurrence and 1 (5.9%) died. Multivariate Cox regression analysis revealed that middle-aged and older age (>35 years) was an independent adverse factor for progression-free survival. CONCLUSION: Our study found that, for ACPPs, pediatric and young adult patients had significantly better prognoses than middle-aged and older patients. We recommend gross total resection as the standard treatment strategy. The role of radiotherapy did not demonstrate a significant benefit in our study. Further studies with larger cohorts are required to validate our conclusions.