title: Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute.
abstract: BACKGROUND
  Choroid plexus tumor is a rare brain tumor with variable clinical features according to the histological grade. We reviewed the treatment outcome of 23 children, focusing on the biological behavior of the atypical choroid plexus papilloma (ACPP) and the current therapeutic strategy in choroid plexus carcinoma (CPC).
  
  
  METHODS
  The demographics, clinical features, surgical treatments, adjuvant therapies, and survival were reviewed.
  
  
  RESULTS
  The median age at diagnosis was 18 months--55 months for choroid plexus papilloma (CPP), 8 months for ACPP, and 15 months for CPC. Gross total resections were achieved in seven of eight patients with CPP, seven of seven with ACPP, and three of eight with CPC. Seven patients with CPC received chemotherapy. Four patients received high-dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (aPBSCT), and three among them have survived. Four patients with CPC received radiotherapy. One CPP patient and one CPC patient underwent radiosurgery. All CPP and ACPP patients have survived. The overall survival rate of the CPC patients was 62.5% in the first year and 42.9% in the second year. The progression-free survival rate of the CPC patients was 50% in the first year and 0% in the second year. Seven patients underwent permanent cerebrospinal fluid diversion surgery because of hydrocephalus or subdural effusion.
  
  
  CONCLUSIONS
  CPP and ACPP were surgically curable. Multi-modal treatments are necessary in the management of CPC with poor prognosis. HDCT and aPBSCT may be important to treat infants for whom radiotherapy is limited. Hydrocephalus and subdural effusion should be resolved with appropriate management.
authors.affiliation: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, Republic of Korea.
authors.forename: Eun Jung
authors.initials: EJ
authors.lastname: Koh
authors.forename: Kyu-Chang
authors.initials: KC
authors.lastname: Wang
authors.forename: Ji Hoon
authors.initials: JH
authors.lastname: Phi
authors.forename: Ji Yeoun
authors.initials: JY
authors.lastname: Lee
authors.forename: Jung Won
authors.initials: JW
authors.lastname: Choi
authors.forename: Sung-Hye
authors.initials: SH
authors.lastname: Park
authors.forename: Kyung Duk
authors.initials: KD
authors.lastname: Park
authors.forename: Il Han
authors.initials: IH
authors.lastname: Kim
authors.forename: Byung-Kyu
authors.initials: BK
authors.lastname: Cho
authors.forename: Seung-Ki
authors.initials: SK
authors.lastname: Kim
mesh_terms: D002648:Child; D002675:Child, Preschool; D016545:Choroid Plexus Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies