Craniopharyngiomas (CPs) are rare (2.5 - 4% of all brain tumors; 6 to 9% of all pediatric intracranial tumors) histologically benign tumors that originate from the remnants of Rathke’s pouch (cisternal surface of the hypothalamic region) and can arise from the anterior superior margin of the pituitary. Some CPs can originate primarily within the third ventricle, with displacement of the floor (5.9% of all intraventricular tumors). Almost all CPs have solid and cystic components. The cyst contains cholesterol crystals. Macroscopically, CPs present two types of calcification. There are two types of CPs: adamantinomatous and papillary. The former occurs in childhood (peak incidence: age 5-10yrs) and the latter in the 6th decade.
Best imaging tool: MR, particularly thin sagittal and coronal sequences.
MR findings: T1WI - classic adamantinomatous type is a hyperintense cyst and heterogeneous nodule, the less common papillary type is isointense solid component; T2WI - cyst is hyperintense, solid component heterogeneous (Ca2+ portions are hypointense); FLAIR - Cyst contents hyperintense; T1 C+ - solid portions enhance heterogeneously, the cyst walls enhance strongly. The most common clinical presentation is in childhood with visual defects, headache, short stature (endocrine disturbances), hydrocephalus.
The treatment is radical surgery (total resection). Great care is essential in surgical procedures in order to avoid damage to the pituitary stalk. Recurrences are related to a limited surgery and to tumor size. A tumor > 5 cm has a 83% recurrence rate. The treatment for residual or recurrent tumor is surgery, radiation therapy, or cyst aspiration. In pediatric cases, it may be best to postpone radiation therapy to minimize deleterious effect on IQ. There is a ten year survival rate of 64 - 96%.
Best imaging tool: MR, particularly thin sagittal and coronal sequences.
MR findings: T1WI - classic adamantinomatous type is a hyperintense cyst and heterogeneous nodule, the less common papillary type is isointense solid component; T2WI - cyst is hyperintense, solid component heterogeneous (Ca2+ portions are hypointense); FLAIR - Cyst contents hyperintense; T1 C+ - solid portions enhance heterogeneously, the cyst walls enhance strongly. The most common clinical presentation is in childhood with visual defects, headache, short stature (endocrine disturbances), hydrocephalus.
The treatment is radical surgery (total resection). Great care is essential in surgical procedures in order to avoid damage to the pituitary stalk. Recurrences are related to a limited surgery and to tumor size. A tumor > 5 cm has a 83% recurrence rate. The treatment for residual or recurrent tumor is surgery, radiation therapy, or cyst aspiration. In pediatric cases, it may be best to postpone radiation therapy to minimize deleterious effect on IQ. There is a ten year survival rate of 64 - 96%.
References
- Ciric IS: Surgery of the third ventricle: Regional embryology, in Apuzzo MLJ (ed): Surgery of the Third Ventricle. Baltimore, Williams & Wilkins, 1987, pp167-174.
- Maira G, Anile C, Colosimo C, Cabezas D: Craniopharyngiomas of the third ventricle: Trans-lamina terminalis approach. Neurosurgery 47:857-863, 2000.
- Konovalov AN, Gorelyshev SK: Surgical treatment of anterior third ventricle tumors. Acta Neurochir (Wien) 118:33-39, 1992.
- Villani R, Papagno C, Tomei D, Grimoldi N, Spagnoli D, Bello B:Transcallosal approach to tumors of the third ventricle: Surgical results and neuropsychological evaluation. J Neurosurg Sci 41:41-50, 1997.
- Handbook of Neurosurgery - fifth edition - Mark S. Greenberg - ed. Thieme.
- Ross JS, Brant-Zawadzki M, Moore KR, et al.. Diagnostic Imaging Spine. First edition - Amirsys - Elsevier Saunders - 2004.
