title: The neuroendocrine sequelae of paediatric craniopharyngioma: a 40-year meta-data analysis of 185 cases from three UK centres.
abstract: OBJECTIVE
  The management of paediatric craniopharyngiomas was traditionally complete resection (CR), with better reported tumour control compared to that by partial resection (PR) or limited surgery (LS). The subsequent shift towards hypothalamic sparing, conservative surgery with adjuvant radiotherapy (RT) to any residual tumour aimed at reducing neuroendocrine morbidity, has not been systematically studied. Hence, we reviewed the sequelae of differing management strategies in paediatric craniopharyngioma across three UK tertiary centres over four decades.
  
  
  METHODS
  Meta-data was retrospectively reviewed over two periods before (1973-2000 (Group A: n = 100)) and after (1998-2011 (Group B: n = 85)) the introduction of the conservative strategy at each centre.
  
  
  RESULTS
  Patients had CR (A: 34% and B: 19%), PR (A: 48% and B: 46%) or LS (A: 16% and B: 34%), with trends reflecting the change in surgical approach over time. Overall recurrence rates between the two periods did not change (A: 38% vs B: 32%). More patients received RT in B than A, but recurrence rates were similar: for A, 28% patients received RT with 9 recurrences (32%); for B, 62% received RT with 14 recurrences (26%). However, rates of diabetes insipidus (P = 0.04), gonadotrophin deficiency (P < 0.001) and panhypopituitarism (P = 0.001) were lower in B than those in A. In contrast, post-operative obesity (BMI SDS >+2.0) (P = 0.4) and hypothalamic (P = 0.1) and visual (P = 0.3) morbidity rates were unchanged.
  
  
  CONCLUSIONS
  The shift towards more conservative surgery has reduced the prevalence of hormone deficiencies, including diabetes insipidus, which can be life threatening. However, it has not been associated with reduced hypothalamic and visual morbidities, which remain a significant challenge. More effective targeted therapies are necessary to improve outcomes.
authors.affiliation: Royal Manchester Children's HospitalManchester, UK.|The School of Medical SciencesFaculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
authors.forename: Timothy Shao Ern
authors.initials: TSE
authors.lastname: Tan
authors.affiliation: Royal Manchester Children's HospitalManchester, UK.|The School of Medical SciencesFaculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
authors.forename: Leena
authors.initials: L
authors.lastname: Patel
authors.affiliation: Royal Manchester Children's HospitalManchester, UK.
authors.forename: Jaya Sujatha
authors.initials: JS
authors.lastname: Gopal-Kothandapani
authors.affiliation: Royal Manchester Children's HospitalManchester, UK.
authors.forename: Sarah
authors.initials: S
authors.lastname: Ehtisham
authors.affiliation: Great Ormond Street Hospital for Children NHS Foundation TrustLondon, UK.
authors.forename: Esieza Clare
authors.initials: EC
authors.lastname: Ikazoboh
authors.affiliation: Great Ormond Street Hospital for Children NHS Foundation TrustLondon, UK.
authors.forename: Richard
authors.initials: R
authors.lastname: Hayward
authors.affiliation: Great Ormond Street Hospital for Children NHS Foundation TrustLondon, UK.
authors.forename: Kristian
authors.initials: K
authors.lastname: Aquilina
authors.affiliation: Royal Manchester Children's HospitalManchester, UK.
authors.forename: Mars
authors.initials: M
authors.lastname: Skae
authors.affiliation: Alder Hey Children's NHS Foundation TrustLiverpool, UK.
authors.forename: Nicky
authors.initials: N
authors.lastname: Thorp
authors.affiliation: Alder Hey Children's NHS Foundation TrustLiverpool, UK.
authors.forename: Barry
authors.initials: B
authors.lastname: Pizer
authors.affiliation: Alder Hey Children's NHS Foundation TrustLiverpool, UK.
authors.forename: Mohammed
authors.initials: M
authors.lastname: Didi
authors.affiliation: Alder Hey Children's NHS Foundation TrustLiverpool, UK.
authors.forename: Conor
authors.initials: C
authors.lastname: Mallucci
authors.affiliation: Alder Hey Children's NHS Foundation TrustLiverpool, UK.
authors.forename: Joanne C
authors.initials: JC
authors.lastname: Blair
authors.affiliation: Great Ormond Street Hospital for Children NHS Foundation TrustLondon, UK.|University College London Hospitals NHS Foundation TrustLondon, UK.
authors.forename: Mark N
authors.initials: MN
authors.lastname: Gaze
authors.affiliation: Royal Manchester Children's HospitalManchester, UK.
authors.forename: Ian
authors.initials: I
authors.lastname: Kamaly-Asl
authors.affiliation: Great Ormond Street Hospital for Children NHS Foundation TrustLondon, UK.|University College London Hospitals NHS Foundation TrustLondon, UK.
authors.forename: Helen
authors.initials: H
authors.lastname: Spoudeas
authors.affiliation: Royal Manchester Children's HospitalManchester, UK.|The School of Medical SciencesFaculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
authors.forename: Peter E
authors.initials: PE
authors.lastname: Clayton
mesh_terms: D002648:Child; D002675:Child, Preschool; D003397:Craniopharyngioma / Q000473:pathology* / Q000601:surgery*; D005260:Female; D006801:Humans; D007031:Hypothalamus / Q000601:surgery; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery; D010911:Pituitary Neoplasms / Q000473:pathology* / Q000601:surgery*; D006113:United Kingdom