Choroid plexus tumors in children - single center experience

Choroid plexus tumors (CPT) are rare brain tumors defined as papillary neoplasms derived from choroid plexus epithelium. Three types of CPT can be distinguished, choroid plexus papilloma (CPP), atypical papilloma (APP) and choroid plexus carcinoma (CPC). We have evaluated characteristics, treatment and outcome of patients with CPTs diagnosed within 1982–2013 in our pediatric oncology center. Our cohort consisted of 30 patients (16 boys vs. 14 girls) with CPTs; 14 were CPC, 2 APP and 14 CPP. Median age at the time of diagnosis was 4.1 years (0.25 to 18.3) with 48% of patients younger than 3. The primary tumor site was located predominantly in lateral ventricles (62.5%), less frequently in 4^th ventricle (25%), in 3^rd ventricle (2 pts) and spinal cord (1 pt). Only one patient with CPC initially presented with metastatic disease, all others had localized disease. Patients with CPP and APP were treated with neurosurgical resection only, except one patient with CPP located in spinal cord, who received focal irradiation (XRT). Two out of 16 patients with CPP and APP died of neurosurgical complications and one patient with APP experienced metastatic recurrence. Patients with CPC were treated with surgery and chemotherapy alone (<3 years) or in combination with focal XRT (≥3 years). In 45% of patients gross total resection (GTR) was achieved. Six patients with CPC experienced recurrence; four of them were initially resected subtotally (STR). Two patients with CPC developed metachronous tumors (glioblastoma and hemangioendothelioma) and one patient was diagnosed with synchronous adrenal neuroblastoma at the time of diagnosis of CPC. Four patients with CPC have died, three of them with progressive disease and one of surgical complications. Probability of 5-years EFS of patients with CPC was 35.9% and 5-years OS 77.6%. Six CPC patients were tested for TP53 germ-line mutations and Li-Fraumeni syndrome was confirmed in four of them. Examination of four more patients is currently in process. Experience with our cohort of CPC indicates that patients after STR are in higher risk of recurrence. Some patients with recurrent disease can be cured. Higher 5y OS observed in our cohort compared to published data is probably caused by lower incidence of metastatic disease among our patients. Furthermore all CPC patients should be tested for TP53 germ-line mutation to reveal Li-Fraumeni syndrome.

Supported by MH CZ – DRO, University Hospital Motol, Prague,Czech Republic 00064203.

No conflict of interest.

Keywords:
Choroid plexus carcinoma
Li-Fraumeni syndrome
Children

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