QL-03 * CHOROID PLEXUS TUMORS: THE CLEVELAND CLINIC EXPERIENCE

Abstract

BACKGROUND: Choroid plexus tumors (CPT) are rare neoplasms divided into Choroid Plexus Papilloma (CPP), Atypical Choroid Plexus Papilloma (APP) and Choroid Plexus Carcinoma (CPC). Due to limited clinical data, many pediatric single cases have been described and more are necessary to complete the picture, particularly in populations that are not age limited. Here we reviewed the Cleveland Clinic experience -adult and pediatric. METHODS: A retrospective analysis of the course of therapy and clinical outcome was assessed in all patients with CPT seen in Cleveland Clinic form 1990 to 2014. RESULTS: We found 11 patients with CPT. The median age at presentation was 18.5 years (range 8 months to 51 years) 6 females, 5 males. The most common presenting symptom was raised intracranial pressure (5 cases) followed by seizures, CN VI palsy, vertigo, diplopia, poor feeding and weight loss (one patient each). The tumors were predominantly observed in the lateral ventricle (4 cases), fourth ventricle (4 cases), posterior fossa (1 case) and unknown location (2 cases). Gross total surgical resection was achieved in 100% of CPT. Complications were minimal, including post-operative residual weakness, gait instability or visual changes. The histology was CPP in nine patients, two of whom developed recurrence requiring repeat resection and adjuvant therapy. Event free survival (EFS) for CPP patients was 12 years. The CPC patients required adjuvant therapy. One CPC patient developed spinal drop metastasis and received craniospinal radiation and gamma knife stereotactic radiosurgery. EFS of our CPC patients was 4.3 years. All patients had great performance status (Average Lansky: 88, Karnofsky: 86). CONCLUSION: The age distribution in our population differed from most other clinical series, suggesting that the tumors may be more frequent in adult patients than originally thought. Our data confirms that gross total resection provides the best prognosis for patients with Choroid Plexus Tumors.