title: Choroid plexus tumors in children less than 36 months: the Canadian Pediatric Brain Tumor Consortium (CPBTC) experience.
abstract: BACKGROUND
  Choroid plexus tumors (CPT) are rare pediatric tumors. A population-based study on choroid plexus carcinoma (CPC) and choroid plexus papilloma (CPP) was carried out to describe the incidence, demographic, and outcome data and to identify potential prognostic factors.
  
  
  METHODS
  The CPT population from the Canadian databank of CNS tumor in children ≤ 36 months diagnosed between 1990 and 2005 was reviewed
  
  
  RESULTS
  Out of the 579 reported cases of CNS tumors, 37 were CPT. The annual age-adjusted incidence rate was 0.22 + 0.12 (95% CI 0.16-0.28)/100,000 children < 3 years. There were 21 (56.7%) CPP and 16 (43.3.5%) CPC. Twenty patients (54%) were males. Median age at diagnosis was 7 months(range 0-30). Ten patients(62.5%) with CPC and one with CPP were metastatic at diagnosis. Twenty patients with CPP (95%) had a complete resection, whereas 6/16 CPC (37.5%) achieved a resection >90%. Fourteen CPC patients received adjuvant chemotherapy. None of the 37 patients received adjuvant radiation. At completion of survey, all CPP and five CPC were alive. Median survival time for CPC patients was 15 months (0-120). One death was related to intraoperative hemorrhage, another to chemotherapy-induced toxicity, and one to secondary AML. Age at diagnosis, degree of resection and metastatic status were not significant prognostic factors for CPC.
  
  
  CONCLUSIONS
  By contrast to CPC, CPP have an excellent prognosis following surgery alone. Survival of CPC remains poor. However, these data may suggest adjuvant chemotherapy can alter the aggressive natural history of CPC. As with other rare CNS tumors, international collaboration is required to identify optimal therapy.
authors.affiliation: Pediatric Hematology Oncology and Bone marrow transplantation program, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada. lucie.lafay-cousin@albertahealthservices.ca
authors.forename: Lucie
authors.initials: L
authors.lastname: Lafay-Cousin
authors.forename: Daniel
authors.initials: D
authors.lastname: Keene
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Carret
authors.forename: Chris
authors.initials: C
authors.lastname: Fryer
authors.forename: Josee
authors.initials: J
authors.lastname: Brossard
authors.forename: Bruce
authors.initials: B
authors.lastname: Crooks
authors.forename: David
authors.initials: D
authors.lastname: Eisenstat
authors.forename: Donna
authors.initials: D
authors.lastname: Johnston
authors.forename: Valerie
authors.initials: V
authors.lastname: Larouche
authors.forename: Marianna
authors.initials: M
authors.lastname: Silva
authors.forename: Beverly
authors.initials: B
authors.lastname: Wilson
authors.forename: Shayna
authors.initials: S
authors.lastname: Zelcer
authors.forename: Ute
authors.initials: U
authors.lastname: Bartels
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
mesh_terms: D017668:Age of Onset; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002170:Canada / Q000453:epidemiology; D002277:Carcinoma / Q000453:epidemiology* / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D016545:Choroid Plexus Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D019635:Neurosurgical Procedures; D020288:Papilloma, Choroid Plexus / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy*; D011379:Prognosis
chemical_list: D000970:Antineoplastic Agents
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