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Choroid plexus tumors in pediatric patients
Hideki Ogiwara1, Arthur J Dipatri, Tord D Alden
1Division of Neurosurgery, Children's Memorial Hospital, Chicago, IL, USA. hideki_o@d5.dion.ne.jp
Insights
Choroid plexus papilloma is surgically curable in children, with excellent outcomes. Choroid plexus carcinoma has a 50% survival rate, and postoperative extraventricular drainage can reduce shunt needs.
Area of Science:
- Pediatric Neurosurgery
- Oncology
- Neuropathology
Background:
- Choroid plexus tumors are rare intraventricular neoplasms, comprising <1% of intracranial and 2-4% of pediatric brain tumors.
- These tumors present a diagnostic and therapeutic challenge due to their location and potential for aggressive behavior.
Purpose of the Study:
- To present institutional experience in managing pediatric choroid plexus tumors.
- To review the literature on the management and outcomes of these rare tumors.
Main Methods:
- Retrospective analysis of pediatric patients with choroid plexus tumors.
- Surgical resection was the primary treatment modality.
- Data collected included demographics, clinical presentation, surgical details, and follow-up outcomes.
Main Results:
- Eighteen tumors were analyzed: 14 papillomas, 2 atypical papillomas, and 2 carcinomas.
- All patients underwent surgical resection with no perioperative mortality.
- Choroid plexus papilloma patients had a 100% survival rate with excellent functional outcomes.
- Choroid plexus carcinoma patients had a 50% survival rate.
- Postoperative extraventricular drainage (EVD) was used in 12 patients, and 27.8% required a ventriculoperitoneal shunt for persistent hydrocephalus.
Conclusions:
- Choroid plexus papilloma is a surgically curable pediatric brain tumor.
- Postoperative EVD can mitigate shunt requirements by clearing cerebrospinal fluid (CSF) and tumor debris.
Objective:
Choroid plexus tumors are rare intraventricular tumors, accounting for less than 1% of all intracranial tumors and 2-4% of brain tumors in children. The authors present their experience in the management of these lesions, and a review of the literature is performed.
Methods:
We retrospectively analyzed the outcome of pediatric patients with choroid plexus tumors treated with surgical resection. The patients' charts were reviewed for demographic data, clinical presentation, surgical therapy and follow-up.
Results:
This study involves 18 consecutive choroid plexus tumors: 14 papillomas, 2 atypical papillomas and 2 carcinomas. The tumor was located in the lateral ventricles (12), the fourth ventricle (4) and the third ventricle (2). The mean age at presentation was 4.6 years. Surgical resection was performed in all cases and no patients died perioperatively. Survival rate of papilloma patients was 100% without evidence of recurrent disease (mean follow-up for 73 months). Survival rate of carcinoma patients was 50% (mean follow-up for 23.5 months). One carcinoma patient died of disseminated disease 13 months after surgery. The functional outcome in long-term survivors after papilloma surgery was excellent. Postoperative extraventricular drainage (EVD) was performed in 12 patients. Five patients (27.8%) had persistent hydrocephalus after tumor resection and required a ventriculoperitoneal shunt.
Conclusion:
Choroid plexus papilloma is a surgically curable disease. Postoperative EVD was considered effective in lowering the rate of shunt requirement through releasing the blood-tinged CSF and small particles of tumor residue.
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