Craniopharyngioma - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

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 to 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 growth failure and adults with diabetes insipidus and sexual dysfunction.
Primary treatment consists of surgery. Following surgery, radiation therapy may be required.

Craniopharyngioma: coronal postcontrast MRI

From the collection of Dr Marc C. Chamberlain

Quick Reference

History & Exam
- Key Factors
- Other Factors
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Diagnostics Tests
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Treatment Options
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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: 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

Key Articles

Garre ML, Cama A. Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. 2007 Aug;19(4):471-9.[Abstract]
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]
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]
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][Full Text]

Referenced Articles

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46. Chung WY, Pan DH, Shiau CY, et al. Gamma knife radiosurgery for craniopharyngiomas. J Neurosurg. 2000 Dec;93 (suppl 3):47-56.[Abstract]
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.[Abstract]
48. 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]
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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.[Abstract][Full Text]
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.[Abstract][Full Text]
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59. 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]
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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.[Abstract]
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.[Abstract][Full Text]
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.[Abstract]
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.[Abstract]
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