title: Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes.
abstract: BACKGROUND
  Quality of life (QoL) and long-term prognosis are frequently, and often severely, impaired in craniopharyngioma (CP) patients. Knowledge of risk factors for long-term outcome is important for optimization of treatment.
  
  
  METHODS
  Overall survival (OS) and progression-free survival (PFS), body mass index (BMI), neuropsychological status (EORTCQLQ-C30, MFI-20), and psychosocial status were analyzed in 261 patients with childhood-onset CP diagnosed before 2000 and longitudinally observed in HIT-Endo.
  
  
  RESULTS
  Twenty-year OS was lower (P = .006) in CP with hypothalamic involvement (HI) (n = 132; 0.84 ± 0.04) when compared with CP without HI (n = 82; 0.95 ± 0.04). OS was not related to degree of resection, sex, age at diagnosis, or year of diagnosis (before/after 1990). PFS (n = 168; 0.58 ± 0.05) was lower in younger patients (<5 y at diagnosis) (n = 30; 0.39 ± 0.10) compared with patients aged 5-10 years (n = 66; 0.52 ± 0.08) and older than 10 years (n = 72; 0.77 ± 0.06) at diagnosis. PFS was not associated with HI, degree of resection, or sex. HI led to severe weight gain during the first 8-12 years of follow-up (median BMI increase: +4.59SD) compared with no HI (median increase: +1.20SD) (P = .00). During >12 years of follow-up, patients with HI presented no further increase in BMI. QoL in CP patients with HI was impaired by obesity, physical fatigue, reduced motivation, dyspnea, diarrhea, and nonoptimal psychosocial development.
  
  
  CONCLUSIONS
  OS and QoL are impaired by HI in long-term survivors of CP. HI is associated with severe obesity, which plateaus after 12 years. OS/PFS are not related to degree of resection, but gross-total resection should be avoided in cases of HI to prevent further hypothalamic damage, which exacerbates sequelae.
authors.affiliation: Department of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Oldenburg, Germany (A.S.S., A.H., U.G., A.M.M.D., H.L.M.); University of Groningen, Groningen, Netherlands (A.S.S., A.M.M.D.); Department of Neuroradiology, University of Würzburg, Würzburg, Germany (M.W.-M.).
authors.forename: Anthe S
authors.initials: AS
authors.lastname: Sterkenburg
authors.affiliation: Department of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Oldenburg, Germany (A.S.S., A.H., U.G., A.M.M.D., H.L.M.); University of Groningen, Groningen, Netherlands (A.S.S., A.M.M.D.); Department of Neuroradiology, University of Würzburg, Würzburg, Germany (M.W.-M.).
authors.forename: Anika
authors.initials: A
authors.lastname: Hoffmann
authors.affiliation: Department of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Oldenburg, Germany (A.S.S., A.H., U.G., A.M.M.D., H.L.M.); University of Groningen, Groningen, Netherlands (A.S.S., A.M.M.D.); Department of Neuroradiology, University of Würzburg, Würzburg, Germany (M.W.-M.).
authors.forename: Ursel
authors.initials: U
authors.lastname: Gebhardt
authors.affiliation: Department of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Oldenburg, Germany (A.S.S., A.H., U.G., A.M.M.D., H.L.M.); University of Groningen, Groningen, Netherlands (A.S.S., A.M.M.D.); Department of Neuroradiology, University of Würzburg, Würzburg, Germany (M.W.-M.).
authors.forename: Monika
authors.initials: M
authors.lastname: Warmuth-Metz
authors.affiliation: Department of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Oldenburg, Germany (A.S.S., A.H., U.G., A.M.M.D., H.L.M.); University of Groningen, Groningen, Netherlands (A.S.S., A.M.M.D.); Department of Neuroradiology, University of Würzburg, Würzburg, Germany (M.W.-M.).
authors.forename: Anna M M
authors.initials: AM
authors.lastname: Daubenbüchel
authors.affiliation: Department of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Oldenburg, Germany (A.S.S., A.H., U.G., A.M.M.D., H.L.M.); University of Groningen, Groningen, Netherlands (A.S.S., A.M.M.D.); Department of Neuroradiology, University of Würzburg, Würzburg, Germany (M.W.-M.).
authors.forename: Hermann L
authors.initials: HL
authors.lastname: Müller
mesh_terms: D000293:Adolescent; D000328:Adult; D015992:Body Mass Index; D003397:Craniopharyngioma / Q000150:complications / Q000453:epidemiology* / Q000401:mortality / Q000523:psychology; D018450:Disease Progression; D005260:Female; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications / Q000453:epidemiology*; D008137:Longitudinal Studies; D008297:Male; D009483:Neuropsychological Tests; D009765:Obesity / Q000150:complications / Q000453:epidemiology; D055815:Young Adult
keywords: craniopharyngioma; hypothalamus; obesity; pediatrics; quality of life
references.citation: Dev Med Child Neurol. 2004 Apr;46(4):220-9
references.pmid: 15077699
references.citation: Obes Rev. 2012 Sep;13(9):780-98
references.pmid: 22577758
references.citation: Radiother Oncol. 1993 Apr;27(1):13-21
references.pmid: 8327728
references.citation: Neuro Oncol. 2013 Jun;15(6):767-74
references.pmid: 23486689
references.citation: J Am Coll Cardiol. 2013 Aug 13;62(7):569-76
references.pmid: 23770180
references.citation: Eur J Endocrinol. 2009 Nov;161(5):671-9
references.pmid: 19667040
references.citation: J Neurosurg. 1990 Jul;73(1):3-11
references.pmid: 2352020
references.citation: Eur J Clin Nutr. 1991 Jan;45(1):13-21
references.pmid: 1855495
references.citation: Eur J Endocrinol. 2006 Mar;154(3):483-9
references.pmid: 16498063
references.citation: Endocr Rev. 2014 Jun;35(3):513-43
references.pmid: 24467716
references.citation: Int J Radiat Oncol Biol Phys. 2004 Mar 1;58(3):714-20
references.pmid: 14967425
references.citation: J Psychosom Res. 1995 Apr;39(3):315-25
references.pmid: 7636775
references.citation: Radiother Oncol. 2005 Oct;77(1):99-104
references.pmid: 16216361
references.citation: Radiother Oncol. 1993 Jan;26(1):1-10
references.pmid: 8438080
references.citation: J Neurosurg. 1996 Jul;85(1):73-81
references.pmid: 8683285
references.citation: J Clin Endocrinol Metab. 2013 Aug;98(8):3253-62
references.pmid: 23771923
references.citation: Childs Nerv Syst. 2005 Aug;21(8-9):729-46
references.pmid: 16044343
references.citation: J Natl Cancer Inst. 1993 Mar 3;85(5):365-76
references.pmid: 8433390
references.citation: J Neurosci Nurs. 2010 Dec;42(6):323-8; quiz 329-30
references.pmid: 21207770
references.citation: Int J Radiat Oncol Biol Phys. 1993 Sep 30;27(2):189-95
references.pmid: 8407391
references.citation: J Clin Endocrinol Metab. 2004 Jan;89(1):81-6
references.pmid: 14715831
references.citation: Nat Rev Endocrinol. 2010 Nov;6(11):609-18
references.pmid: 20877295
references.citation: Clin Endocrinol (Oxf). 2011 Jun;74(6):750-5
references.pmid: 21521265
references.citation: Klin Padiatr. 2004 Nov-Dec;216(6):343-8
references.pmid: 15565549
references.citation: Klin Padiatr. 2014 May;226(3):161-8
references.pmid: 24819386
references.citation: Childs Nerv Syst. 1998 Oct;14(10):558-63
references.pmid: 9840379
references.citation: Klin Padiatr. 2006 Nov-Dec;218(6):315-20
references.pmid: 17080333
references.citation: Pediatr Neurosurg. 1994;21 Suppl 1:75-81
references.pmid: 7841082
references.citation: Clin Endocrinol (Oxf). 2005 Apr;62(4):397-409
references.pmid: 15807869
references.citation: Pediatr Endocrinol Rev. 2008 Dec;6(2):220-7
references.pmid: 19202508
references.citation: Int J Radiat Oncol Biol Phys. 1992;24(4):611-7
references.pmid: 1429082
references.citation: J Clin Endocrinol Metab. 2012 Nov;97(11):3935-43
references.pmid: 22969141
references.citation: J Clin Endocrinol Metab. 2011 Jul;96(7):1981-91
references.pmid: 21508127
references.citation: J Clin Endocrinol Metab. 2012 Apr;97(4):1258-67
references.pmid: 22319039
references.citation: Clin Endocrinol (Oxf). 2005 Feb;62(2):197-204
references.pmid: 15670196
references.citation: Neurogastroenterol Motil. 2009 Feb;21(2):149-59
references.pmid: 18684212
references.citation: J Neurooncol. 2010 Oct;100(1):105-11
references.pmid: 20204458
references.citation: Neurosurgery. 1994 Dec;35(6):1001-10; discussion 1010-1
references.pmid: 7885544
references.citation: Eur J Endocrinol. 2011 Jul;165(1):17-24
references.pmid: 21490122
references.citation: Klin Padiatr. 2003 Nov-Dec;215(6):310-4
references.pmid: 14677094
references.citation: Klin Padiatr. 2014 Nov;226(6-7):344-50
references.pmid: 25431867
references.citation: Klin Padiatr. 2001 Jul-Aug;213(4):244-9
references.pmid: 11528558
references.citation: J Clin Endocrinol Metab. 1998 Nov;83(11):3897-904
references.pmid: 9814465
references.citation: J Pediatr Endocrinol Metab. 2015 Jan;28(1-2):35-43
references.pmid: 25503864
references.citation: Int J Radiat Oncol Biol Phys. 1999 May 1;44(2):255-63
references.pmid: 10760417