Highlights & Basics
- Craniopharyngiomas are benign nonglial epithelial central nervous system tumor, constituting 1% to 3% of intracranial tumors in adults, most often located near the optic chiasm.
- Bimodal age incidence with 50% in children aged between 5 and 14 years and a second peak in adults 50-70 years of age.
- Common acute presentations are sudden visual loss and symptoms of intracranial hypertension.
- Pituitary dysfunction is also common; children may present with faltering growth and adults with diabetes insipidus and sexual dysfunction.
- Primary treatment consists of surgery. Following surgery, radiation therapy may be required.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Craniopharyngioma: adamantinous histology (low power) with complex arrangements of epithelium, cysts, and gliotic brain
Craniopharyngioma: adamantinous histology (medium power) with epithelial ribbons showing reticular areas and nodules of keratin
Craniopharyngioma: adamantinous histology (high power) with basal-aligned columnar cells, stellate reticulum, and epithelial keratinization
Craniopharyngioma: coronal postcontrast MRI
Craniopharyngioma: axial postcontrast MRI
Craniopharyngioma: sagittal postcontrast MRI
Craniopharyngioma: sagittal postcontrast MRI
Craniopharyngioma (postoperative): sagittal postcontrast MRI
Citations
Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-51.[Abstract][Full Text]
Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006 Jun;27(4):371-97.[Abstract]
Gan HW, Morillon P, Albanese A, et al. National UK guidelines for the management of paediatric craniopharyngioma. Lancet Diabetes Endocrinol. 2023 Sep;11(9):694-706.[Abstract]
Hoffman LM, Jaimes C, Mankad K, et al. Response assessment in pediatric craniopharyngioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) Working Group. Neuro Oncol. 2023 Feb 14;25(2):224-33.[Abstract][Full Text]
1. Adamson TE, Wiestler OD, Kleihues P, et al. Correlation of clinical and pathological features in surgically treated craniopharyngiomas. J Neurosurg. 1990;73:12-17.[Abstract]
2. Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-51.[Abstract][Full Text]
3. Van Effenterre R, Boch AL. Craniopharyngioma in adults and children: a study of 122 surgical cases. J Neurosurg. 2002 Jul;97(1):3-11.[Abstract]
4. Carmel PW, Antunes JL, Chang CH. Craniopharyngiomas in children. Neurosurgery. 1982 Sep;11(3):382-9.[Abstract]
5. Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev. 2006 Jun;27(4):371-97.[Abstract]
6. Baskin DS, Wilson CB. Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg. 1986 Jul;65(1):22-7.[Abstract]
7. Defoort-Dhellemmes S, Moritz F, Bouacha I, et al. Craniopharyngioma: ophthalmological aspects at diagnosis. J Pediatr Endocrinol Metab. 2006 Apr;19(suppl 1):321-4.[Abstract]
8. Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998 Oct;89(4):547-51.[Abstract]
9. Halac I, Zimmerman D. Endocrine manifestations of craniopharyngioma. Childs Nerv Syst. 2005 Aug;21(8-9):640-8.[Abstract]
10. Hopper N, Albanese A, Ghirardello S, et al. The preoperative endocrine assessment of craniopharyngiomas. J Pediatr Endocrinol Metab. 2006 Apr;19(suppl 1):325-7.[Abstract]
11. 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.[Abstract]
12. Garre ML, Cama A. Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. 2007 Aug;19(4):471-9.[Abstract]
13. Haupt R, Magnani C, Pavanello M, et al. Epidemiological aspects of craniopharyngioma. J Pediatr Endocrinol Metab. 2006 Apr;19(suppl 1):289-93.[Abstract]
14. Price M, Ballard C, Benedetti J, et al. CBTRUS statistical report: primary brain and other central nervous system tumors dDagnosed in the United States in 2017-2021. Neuro Oncol. 2024 Oct 6;26(supplement_6):vi1-85.[Abstract][Full Text]
15. Nielsen EH, Feldt-Rasmussen U, Poulsgaard L, et al. Incidence of craniopharyngioma in Denmark (n = 189) and estimated world incidence of craniopharyngioma in children and adults. J Neurooncol. 2011 Sep;104(3):755-63.[Abstract]
16. Zacharia BE, Bruce SS, Goldstein H, et al. Incidence, treatment and survival of patients with craniopharyngioma in the surveillance, epidemiology and end results program. Neuro Oncol. 2012 Aug;14(8):1070-8.[Abstract]
17. Lithgow K, Pohl U, Karavitaki N. Craniopharyngiomas. In: Feingold KR, Anawalt B, Boyce A, et al, eds. Endotext [internet]. South Dartmouth, MA: MDText.com, Inc; 2000 (last updated 2019).[Abstract][Full Text]
18. Crotty TB, Scheithauer BW, Young WF, et al. Papillary craniopharyngiomas: a clinicopathological study of 48 cases. J Neurosurg. 1995 Aug;83(2):206-14.[Abstract]
19. Hofmann B, Kreutzer J, Saeger W, et al. Nuclear beta-catenin accumulation as reliable marker for the differentiation between cystic craniopharyngiomas and rathke cleft cysts: a clinico-pathologic approach. Am J Surg Path. 2006 Dec;30(12):1595-603.[Abstract]
20. Larkin S, Karavitaki N. Recent advances in molecular pathology of craniopharyngioma. F1000Res. 2017 Jul 24;6:1202.[Abstract][Full Text]
21. 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.[Abstract]
22. Rajan B, Ashley S, Thomas DG, et al. Craniopharyngioma: improving outcome by early recognition and treatment of acute complications. Int J Radiat Oncol Biol Phys. 1997 Feb 1;37(3):517-21.[Abstract]
23. Hukin J, Visser J, Sargent M, et al. Childhood craniopharyngioma: Vancouver experience. Childs Nerv Syst. 2005 Aug;21(8-9):758-65.[Abstract]
24. Zada G, Lin N, Ojerholm E, et al. Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships. Neurosurg Focus. 2010 Apr;28(4):E4.[Abstract]
25. Sorva R, Heiskanen O, Perheetupa J. Craniopharyngioma surgery in children: endocrine and visual outcome. Childs Nerv Syst. 1988 Apr;4(2):97-9.[Abstract]
26. Gan HW, Morillon P, Albanese A, et al. National UK guidelines for the management of paediatric craniopharyngioma. Lancet Diabetes Endocrinol. 2023 Sep;11(9):694-706.[Abstract]
27. Price DA, Jönsson P. Effect of growth hormone treatment in children with craniopharyngioma with reference to the KIGS (Kabi International Growth Study) database. Acta Paediatr Suppl. 1996 Oct;417:83-5.[Abstract]
28. Sorva R. Children with craniopharyngioma. Early growth failure and rapid postoperative weight gain. Acta Paediatr Scand. 1988 Jul;77(4):587-92.[Abstract]
29. Curran JG, O'Connor E. Imaging of craniopharyngioma. Childs Nerv Syst. 2005 Aug;21(8-9):635-9.[Abstract]
30. Hoffman LM, Jaimes C, Mankad K, et al. Response assessment in pediatric craniopharyngioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) Working Group. Neuro Oncol. 2023 Feb 14;25(2):224-33.[Abstract][Full Text]
31. Fischer EG, Welch K, Shillito J, et al. Craniopharyngiomas in children. Long-term effects of conservative surgical procedures combined with radiation therapy. J Neurosurg. 1990 Oct;73(4):534-40.[Abstract]
32. Clark AJ, Cage TA, Aranda D, et al. Treatment-related morbidity and the management of pediatric craniopharyngioma: a systematic review. J Neurosurg Pediatr. 2012 Oct;10(4):293-301.[Abstract]
33. Hankinson TC, Palmeri NO, Williams SA, et al. Patterns of care for craniopharyngioma: survey of members of the american association of neurological surgeons. Pediatr Neurosurg. 2013;49(3):131-6.[Abstract]
34. Mortini P, Losa M, Pozzobon G, et al. Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series. J Neurosurg. 2011 May;114(5):1350-9.[Abstract]
35. Children's Cancer and Leukaemia Group. Craniopharyngioma: Guidelines for the management of children and young people (CYP) aged up to 19 years. Oct 2021 [internet publication].[Full Text]
36. Stripp DC, Maity A, Janss AJ, et al. Surgery with or without radiation therapy in the management of craniopharyngiomas in children and young adults. Int J Radiat Oncol Biol Phys. 2004 Mar 1;58(3):714-20.[Abstract]
37. Komotar RJ, Starke RM, Raper DM, et al. Endoscopic skull base surgery: a comprehensive comparison with open transcranial approaches. Br J Neurosurg. 2012 Oct;26(5):637-48.[Abstract]
38. Hoffman HJ, De Silva M, Humphreys RP, et al. Aggressive surgical management of craniopharyngiomas in children. J Neurosurg. 1992 Jan;76(1):47-52.[Abstract]
39. Fong RP, Babu CS, Schwartz TH. Endoscopic endonasal approach for craniopharyngiomas. J Neurosurg Sci. 2021 Apr;65(2):133-9.[Abstract]
40. Soldozy S, Yeghyayan M, Yağmurlu K, et al. Endoscopic endonasal surgery outcomes for pediatric craniopharyngioma: a systematic review. Neurosurg Focus. 2020 Jan 1;48(1):E6.[Abstract][Full Text]
41. Palmisciano P, Young K, Ogasawara M, et al. Craniopharyngiomas invading the ventricular system: a systematic review. Anticancer Res. 2022 Sep;42(9):4189-97.[Abstract][Full Text]
42. Cossu G, Jouanneau E, Cavallo LM, et al. Surgical management of craniopharyngiomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section. Acta Neurochir (Wien). 2020 May;162(5):1159-77.[Abstract]
43. Tan TSE, Patel L, Gopal-Kothandapani JS, et al. The neuroendocrine sequelae of paediatric craniopharyngioma: a 40-year meta-data analysis of 185 cases from three UK centres. Eur J Endocrinol. 2017 Mar;176(3):359-69.[Abstract]
44. Clark AJ, Cage TA, Aranda D, et al. A systematic review of the results of surgery and radiotherapy on tumor control for pediatric craniopharyngioma. Childs Nerv Syst. 2013 Feb;29(2):231-8.[Abstract]
45. 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.[Abstract]
46. 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.[Abstract]
47. 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.[Abstract]
48. Yang I, Sughrue ME, Rutkowski MJ, et al. Craniopharyngioma: a comparison of tumor control with various treatment strategies. Neurosurg Focus. 2010 Apr;28(4):E5.[Abstract][Full Text]
49. Fouda MA, Karsten M, Staffa SJ, et al. Management strategies for recurrent pediatric craniopharyngioma: new recommendations. J Neurosurg Pediatr. 2021 May 1;27(5):548-55.[Abstract][Full Text]
50. Hill TK, Baine MJ, Verma V, et al. Patterns of care in pediatric craniopharyngioma: outcomes following definitive radiotherapy. Anticancer Res. 2019 Feb;39(2):803-7.[Abstract]
51. Zhang C, Verma V, Lyden ER, et al. The role of definitive radiotherapy in craniopharyngioma: a SEER analysis. Am J Clin Oncol. 2018 Aug;41(8):807-12.[Abstract]
52. Harary PM, Rajaram S, Hori YS, et al. The spectrum of radiation therapy options for craniopharyngioma: a systematic review. J Neurooncol. 2025 Jun;173(2):275-88.[Abstract]
53. Jalali R, Ghosh S, Chatterjee A, et al. High-precision radiotherapy achieves excellent long-term control and preserves function in pediatric craniopharyngioma-Subset analysis of a randomized trial. Neuro Oncol. 2025 Sep 17;27(8):2147-57.[Abstract]
54. Niranjan A, Kano H, Mathieu D, et al. Radiosurgery for craniopharyngioma. Int J Radiat Oncol Biol Phys. 2010 Sep 1;78(1):64-71.[Abstract]
55. Chung WY, Pan DH, Shiau CY, et al. Gamma knife radiosurgery for craniopharyngiomas. J Neurosurg. 2000 Dec;93 (suppl 3):47-56.[Abstract]
56. 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.[Abstract]
57. Minniti G, Saran F, Traish D, et al. Fractionated stereotactic conformal radiotherapy following conservative surgery in the control of craniopharyngiomas. Radiother Oncol. 2007 Jan;82(1):90-5.[Abstract]
58. Müller HL. Consequences of craniopharyngioma surgery in children. J Clin Endocrinol Metab. 2011 Jul;96(7):1981-91.[Abstract]
59. 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.[Abstract][Full Text]
60. 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.[Abstract]
61. 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.[Abstract]
62. Winkfield KM, Tsai HK, Yao X, et al. Long-term clinical outcomes following treatment of childhood craniopharyngioma. Pediatr Blood Cancer. 2011 Jul 1;56(7):1120-6.[Abstract]
63. Liubinas SV, Munshey AS, Kaye AH. Management of recurrent craniopharyngioma. J Clin Neurosci. 2011 Apr;18(4):451-7.[Abstract]
64. Julow J, Backlund E-O, Lanyi F, et al. Long-term results and late complications after intracavitary yttrium-90 colloid irradiation of recurrent cystic craniopharyngiomas. Neurosurgery. 2007 Aug;61(2):288-95.[Abstract]
65. Van den Berg JH, Blaauw G, Breeman WA, et al. Intracavitary brachytherapy of cystic craniopharyngiomas. J Neurosurg. 1992 Oct;77(4):545-50.[Abstract]
66. Pollack IF, Lunsford LD, Slamovits TI, et al. Stereotaxic intracavitary irradiation for cystic craniopharyngiomas. J Neurosurg. 1988 Feb;68(2):227-33.[Abstract]
67. Zhang S, Fang Y, Cai BW, et al. Intracystic bleomycin for cystic craniopharyngiomas in children. Cochrane Database Syst Rev. 2016 Jul 14;7:CD008890.[Abstract][Full Text]
68. Sahakitrungruang T, Klomchan T, Supornsilchai V, et al. Obesity, metabolic syndrome, and insulin dynamics in children after craniopharyngioma surgery. Eur J Pediatr. 2011 Jun;170(6):763-9.[Abstract]
69. Lo AC, Howard AF, Nichol A, et al. Long-term outcomes and complications in patients with craniopharyngioma: the British Columbia Cancer Agency experience. Int J Radiat Oncol Biol Phys. 2014 Apr 1;88(5):1011-8.[Abstract]
70. Müller HL. Childhood craniopharyngioma. Pituitary. 2013 Mar;16(1):56-67.[Abstract]
71. Erfurth EM. Endocrine aspects and sequel in patients with craniopharyngioma. J Pediatr Endocrinol Metab. 2015 Jan;28(1-2):19-26.[Abstract]
72. Agosti E, Zeppieri M, Antonietti S, et al. Advancing craniopharyngioma management: a systematic review of current targeted therapies and future perspectives. Int J Mol Sci. 2024 Jan 5;25(2):723.[Abstract][Full Text]
73. Brastianos PK, Twohy E, Geyer S, et al. BRAF-MEK inhibition in newly diagnosed papillary craniopharyngiomas. N Engl J Med. 2023 Jul 13;389(2):118-26.[Abstract][Full Text]
74. Wu ZP, Wang YL, Wang LC, et al. Case report: successful use of BRAF/MEK inhibitors in aggressive BRAF-mutant craniopharyngioma. World Neurosurg. 2023 Dec;180:e117-26.[Abstract][Full Text]
75. Aylwin SJ, Bodi I, Beaney R. Pronounced response of papillary craniopharyngioma to treatment with vemurafenib, a BRAF inhibitor. Pituitary. 2016 Oct;19(5):544-6.[Abstract][Full Text]
76. Joshi N, Mueller S, Kline C. Current clinical trials for craniopharyngiomas: what's on the horizon? J Neurooncol. 2025 Apr;172(2):281-8.[Abstract][Full Text]
77. Fahlbusch R, Honegger J, Paulus W, et al. Surgical treatment of craniopharyngiomas: experience with 168 patients. J Neurosurg. 1999 Feb;90(2):237-50.[Abstract]
78. Yamada S, Fukuhara N, Yamaguchi-Okada M, et al. Therapeutic outcomes of transsphenoidal surgery in pediatric patients with craniopharyngiomas: a single-center study. J Neurosurg Pediatr. 2018 Jun;21(6):549-62.[Abstract][Full Text]
79. 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.[Abstract]
80. 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.[Abstract]
81. 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.[Abstract]
82. Honegger J, Buchfelder M, Fahlbusch R. Surgical treatment of craniopharyngiomas: endocrinological results. J Neurosurg. 1999 Feb;90(2):251-7.[Abstract]
83. Duff JM, Meyer FB, Ilstrup DM, et al. Long-term outcomes for surgically resected craniopharyngiomas. Neurosurgery. 2000 Feb;46(2):291-302.[Abstract]
84. Roth P, Pace A, Le Rhun E, et al. Neurological and vascular complications of primary and secondary brain tumours: EANO-ESMO clinical practice guidelines for prophylaxis, diagnosis, treatment and follow-up. Ann Oncol. 2021 Feb;32(2):171-82.[Abstract][Full Text]
85. Pereira AM, Schmid EM, Schutte PJ, et al. High prevalence of long-term cardiovascular, neurological and psychosocial morbidity after treatment for craniopharyngioma. Clin Endocrinol (Oxf). 2005 Feb;62(2):197-204.[Abstract]
