CHOROID PLEXUS TUMORS IN CHILDREN: ANALYSIS OF CLINICAL DATA
Authors:   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia, Valiakhmetova A.F. ,   N.N. Burdenko National Scientific and Practical Center for Neurosurgery, Moscow, Russia, Papusha L.I. ,   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia, Sanakoeva A.V. ,   Prof. V.F. Voino-Yasenetsky Scientific and Practical Center for Specialized Medical Care for Children of the Moscow Department of Healthcare, Moscow, Russia, Shishkina L.V. ,   N.N. Burdenko National Scientific and Practical Center for Neurosurgery, Moscow, Russia, Bydanov O.I. ,   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia, Novichkova G.A. ,   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia, Gorelyshev S.K. ,   N.N. Burdenko National Scientific and Practical Center for Neurosurgery, Moscow, Russia, Karachunsky A.I. ,   National Scientific and Practical Center for Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev, Moscow, Russia
Published: May 2021

Choroid plexus tumors (CPT) are rare neoplasms accounting for 2–5% of all brain tumours in children, and are most commonly under 2 years of age. Most of these tumors present with symptoms of intracranial hypertension. Survival directly depends on the histological variant of the CPT. Objective of the study: to analyze the current and outcome characteristics of patients with CPT depending on the morphological variant. This study is a multicenter, retrospective, open, uncontrolled, non-comparative, non-randomized, longitudinal study. Materials and methods of research: 152 children with CPT according to the WHO classification of CNS tumors (2007, 2016), aged 0 to 18 years, who received treatment and observed from 2009 to 2019 were included in this study. Results: 83 (54,6%) of 152 children with CPT had choroid plexus papillomas (CPPs), 37 (24,3%) – atypical choroid plexus papillomas (APPs), 32 (21,1%) – choroid plexus carcinomas (CPCs). The median age for CPP was 21 months, for APP – 6 months, for CPC – 30 months. CPTs occurred at the age of less than 24 months in 53,3% of cases. In 70,4% of cases CPT were localized in the lateral ventricles. In most children disease manifested by intracranial hypertension, and this symptomatology in children with CPC was statistically significantly more frequent than in children with CPP (p=0,0042). In 28,9% of patients with CPP, 24,3% with APP and 9,4% with CPC the disease was asymptomatic, and all these patients were diagnosed during routine preventive examination. Five-year event-free survival (EFS) and overall survival (OS) for CPP, APP and CPC were 96%±2% and 99%±1%, 81%±7% and 97%±3%, 44%±10% and 66%±10%, respectively (p<0,0001). Conclusion: for the first time in the national literature the results of a retrospective analysis of clinical characteristics and survival of children suffering from a rare group of diseases such as CPT are collected and presented.