Quality of life is significantly impacted. Craniopharyngioma is best thought of as a chronic disease that is multi-dimensional.[61]Müller HL. Childhood craniopharyngioma. Pituitary. 2013 Mar;16(1):56-67.
http://rd.springer.com/article/10.1007%2Fs11102-012-0401-0
http://www.ncbi.nlm.nih.gov/pubmed/22678820?tool=bestpractice.com
Additionally, adults with craniopharyngioma have a substantially elevated risk of cardiovascular disease relative to the general population, added to the reduced health already common to these patients.[62]Erfurth EM. Endocrine aspects and sequel in patients with craniopharyngioma. J Pediatr Endocrinol Metab. 2015 Jan;28(1-2):19-26.
http://www.ncbi.nlm.nih.gov/pubmed/25514328?tool=bestpractice.com
Completely resected tumours
Recurrence risks range from 10% to 35%. The lowest recurrence rates are seen in tumours completely resected via a trans-sphenoidal surgical approach, although there is an approximately 1% operative mortality.[10]Dhellemmes P, Vinchon M. Radical resection for craniopharyngiomas in children: surgical technique and clinical results. J Pediatr Endocrinol Metab. 2006 Apr;19(suppl 1):329-35.
http://www.ncbi.nlm.nih.gov/pubmed/16700308?tool=bestpractice.com
[63]Fahlbusch R, Honegger J, Paulus W, et al. Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg. 1999 Feb;90(2):237-50.
http://www.ncbi.nlm.nih.gov/pubmed/9950494?tool=bestpractice.com
Most recurrences occur in the first 5 years after primary surgery. More than 70% of patients are functionally independent following surgery and with long-term follow-up.[24]Sorva R, Heiskanen O, Perheetupa J. Craniopharyngioma surgery in children: endocrine and visual outcome. Childs Nerv Syst. 1988 Apr;4(2):97-9.
http://www.ncbi.nlm.nih.gov/pubmed/3401877?tool=bestpractice.com
[50]Poretti A, Grotzer MA, Ribi K, et al. Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol. 2004 Apr;46(4):220-9.
http://onlinelibrary.wiley.com/doi/10.1111/j.1469-8749.2004.tb00476.x/pdf
http://www.ncbi.nlm.nih.gov/pubmed/15077699?tool=bestpractice.com
[51]Kiehna EN, Mulhem RK, Li C, et al. Changes in attentional performance of children and young adults with localized primary brain tumors after conformal radiation therapy. J Clin Oncol. 2006 Nov 20;24(33):5283-90.
https://ascopubs.org/doi/full/10.1200/JCO.2005.03.8547
http://www.ncbi.nlm.nih.gov/pubmed/17114662?tool=bestpractice.com
[64]Dekkers OM, Biermasz NR, Smit JW, et al. Quality of life in treated adult craniopharyngioma patients. Eur J Endocrinol. 2006 Mar;154(3):483-9.
https://eje.bioscientifica.com/view/journals/eje/154/3/1540483.xml
http://www.ncbi.nlm.nih.gov/pubmed/16498063?tool=bestpractice.com
[65]Gupta DK, Ojha BK, Sarkar C, et al. Recurrence in craniopharyngiomas: analysis of clinical and histological features. J Clin Neurosci. 2006 May;13(4):438-42.
http://www.ncbi.nlm.nih.gov/pubmed/16678722?tool=bestpractice.com
[66]De Vile CJ, Grant DB, Kendall BE, et al. Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg. 1996 Jul;85(1):73-81.
http://www.ncbi.nlm.nih.gov/pubmed/8683285?tool=bestpractice.com
Radical surgery increases the risk of hypothalamic injury, which may manifest with hyperphagia resulting in obesity and neurobehavioural abnormalities. The consequences of hypothalamic injury are a major source of long-term morbidity.[14]Puget S, Garnett M, Wray A, et al. Pediatric craniopharyngiomas: classification and treatment according to the degree of hypothalamic involvement. J Neurosurg. 2007 Jan;106(1 suppl):3-12.
http://www.ncbi.nlm.nih.gov/pubmed/17233305?tool=bestpractice.com
[34]Hoffman HJ, De Silva M, Humphreys RP, et al. Aggressive surgical management of craniopharyngiomas in children. J Neurosurg. 1992 Jan;76(1):47-52.
http://www.ncbi.nlm.nih.gov/pubmed/1727168?tool=bestpractice.com
[52]Müller HL, Gebhardt U, Teske C, et al. Post-operative hypothalamic lesions and obesity in childhood craniopharyngioma: results of the multinational prospective trial KRANIOPHARYNGEOM 2000 after three year follow-up. Eur J Endocrinol. 2011 Jul;165(1):17-24.
https://eje.bioscientifica.com/view/journals/eje/165/1/17.xml
http://www.ncbi.nlm.nih.gov/pubmed/21490122?tool=bestpractice.com
[66]De Vile CJ, Grant DB, Kendall BE, et al. Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? J Neurosurg. 1996 Jul;85(1):73-81.
http://www.ncbi.nlm.nih.gov/pubmed/8683285?tool=bestpractice.com
Following surgery, some improvement or deterioration in vision may be noted. However, visual impairment remains in up to 80% of patients.[24]Sorva R, Heiskanen O, Perheetupa J. Craniopharyngioma surgery in children: endocrine and visual outcome. Childs Nerv Syst. 1988 Apr;4(2):97-9.
http://www.ncbi.nlm.nih.gov/pubmed/3401877?tool=bestpractice.com
[50]Poretti A, Grotzer MA, Ribi K, et al. Outcome of craniopharyngioma in children: long-term complications and quality of life. Dev Med Child Neurol. 2004 Apr;46(4):220-9.
http://onlinelibrary.wiley.com/doi/10.1111/j.1469-8749.2004.tb00476.x/pdf
http://www.ncbi.nlm.nih.gov/pubmed/15077699?tool=bestpractice.com
Multiple endocrinopathies are seen in the majority of patients (>80%), requiring hormone replacement therapy.[49]Müller HL. Consequences of craniopharyngioma surgery in children. J Clin Endocrinol Metab. 2011 Jul;96(7):1981-91.
https://academic.oup.com/jcem/article/96/7/1981/2833879
http://www.ncbi.nlm.nih.gov/pubmed/21508127?tool=bestpractice.com
[67]Honegger J, Buchfelder M, Fahlbusch R. Surgical treatment of craniopharyngiomas: endocrinological results. J Neurosurg. 1999 Feb;90(2):251-7.
http://www.ncbi.nlm.nih.gov/pubmed/9950495?tool=bestpractice.com
[68]Duff JM, Meyer FB, Ilstrup DM, et al. Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery. 2000 Feb;46(2):291-302.
http://www.ncbi.nlm.nih.gov/pubmed/10690718?tool=bestpractice.com
Incompletely resected tumours treated with radiotherapy
There is a 25% to 50% recurrence risk.[39]Weiss M, Sutton L, Marcial V, et al. The role of radiation therapy in the management of childhood craniopharyngioma. Int J Radiat Oncol Biol Phys. 1989 Dec;17(6):1313-21.
http://www.ncbi.nlm.nih.gov/pubmed/2689398?tool=bestpractice.com
[40]Habrand JL, Ganry O, Couanet D, et al. The role of radiation therapy in the management of craniopharyngioma: a 25-year experience and review of the literature. Int J Radiat Oncol Biol Phys. 1999 May 1;44(2):255-63.
http://www.ncbi.nlm.nih.gov/pubmed/10760417?tool=bestpractice.com
[41]Varlotto JM, Flickinger JC, Kondziolka D, et al. External beam irradiation of craniopharyngioma: long-term analysis of tumor control and morbidity. Int J Radiat Oncol Biol Phys. 2002 Oct 1;54(2):492-9.
http://www.ncbi.nlm.nih.gov/pubmed/12243827?tool=bestpractice.com
[47]Scarzello G, Buzzaccarini MS, Perilongo G, et al. Acute and late morbidity after limited resection and focal radiation therapy in craniopharyngiomas. J Pediatr Endocrinol Metab. 2006 Apr;19 Suppl 1:399-405.
http://www.ncbi.nlm.nih.gov/pubmed/16700317?tool=bestpractice.com
Most recurrences occur in the first 5 years after primary surgery.