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Published on: June 16, 2023
Clinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children
Wen-Jian-Long Zhou1, Xi Wang1, Jia-Yi Peng1
1Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China.
Insights
Pediatric choroid plexus tumors (CPTs) show varying outcomes based on type. Choroid plexus carcinoma (CPC) has a poorer prognosis, necessitating early intervention and adjuvant therapy for better outcomes in CPT patients.
Area of Science:
- Pediatric neuro-oncology
- Neurosurgery
- Clinical oncology
Background:
- Choroid plexus tumors (CPTs) in children present complex treatment decisions.
- Understanding CPT clinical features and prognostic factors is crucial for guiding management.
Purpose of the Study:
- To analyze clinical characteristics and prognostic risk factors of pediatric CPTs.
- To offer insights into clinical decision-making for CPT treatment.
Main Methods:
- Retrospective analysis of 96 pediatric CPT patients (<14 years).
- Investigated CPT pathology, hydrocephalus, treatment, outcomes, and recurrence.
- Statistical analysis included Chi-square, Mann-Whitney U, and log-rank tests for progression-free survival (PFS).
Main Results:
- Choroid plexus carcinoma (CPC) showed shorter disease course, higher hydrocephalus rates, and lower cure rates compared to choroid plexus papilloma (CPP) and atypical CPT (aCPP).
- Tumors in the lateral or third ventricles caused more severe hydrocephalus than those in the fourth ventricle.
- Gross total resection did not significantly improve PFS over partial resection due to adjuvant therapy use in the latter group.
Conclusions:
- CPT in the fourth ventricle may present early symptoms requiring prompt intervention.
- Adjuvant treatment is recommended for partially resected CPP, aCPP, and CPC to improve patient outcomes.
Background:
Decision-making concerning the treatment of choroid plexus tumor (CPT) in pediatric patients remains a topic of considerable debate. The aim of this work was to describe clinical features and prognostic risk factors of CPT in the pediatric population and to provide theoretical opinions regarding clinical decisions for CPT.
Methods:
The data of 96 patients with CPT and younger than 14 years were retrospectively analyzed. Clinical characteristics such as pathological type of CPTs, rate and severity of hydrocephalus, treatment and outcome, and recurrence were investigated. For categorical variables, the Pearson's Chi-square test was performed. The Mann-Whitney U-test was used for comparisons between nonnormally distributed parameters. Log-rank test was used for progression-free survival (PFS).
Results:
The study included 70 choroid plexus papilloma (CPP) cases, 17 atypical choroid plexus papilloma (aCPP) cases, and 9 choroid plexus carcinoma (CPC) cases. Compared with patients with CPP or aCPP, patients with CPC had a shorter disease course (median: CPP, 4 months; aCPP, 2 months; CPC, 1 month; H: 23.5, P < 0.001), higher rate of acute hydrocephalus (CPP, 27.1%; aCPP, 52.9%; CPC, 77.8%; χ2 = 10.9, P < 0.05), and lower incidence of cure rate (CPP, 85.7%; aCPP, 70.5%; CPC, 33.3%; χ2 = 13.5, P < 0.05). The severity of hydrocephalus with tumor in the lateral or third ventricle was significantly higher than that with tumors in the fourth ventricle (severe hydrocephalus: lateral ventricle, 51.7%; third ventricle, 47.0%; fourth ventricle, 11.1%; χ2 = 26.0, P < 0.001). Patients with gross total surgical resection had no better PFS than those with partial resection because of the use of adjuvant therapy in the latter (χ2 = 4.0, P > 0.05). Patients with CPC experienced shorter time for recurrence than those with CPP or aCPP (χ2 = 40.1, P < 0.0001).
Conclusions:
Our results indicated that CPP in the fourth ventricle could trigger serious clinical symptoms at an early stage, requiring early intervention. Adjuvant treatment might be necessary for patients with partially resected CPP, aCPP, and CPC to achieve a favorable outcome.
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