1. What is the most likely diagnosis?
2. What test is not indicated in the evaluation of this patient as presented?
3. The most common side effect following surgical resection of these lesions is:
4. Which of the following describes you?
5. I practice in one of the following locations.
Craniopharyngiomas are tumors usually found in the region of the infundibulum, although they can develop anywhere along an axis from the nasophayrnx to the third ventricle. They comprise approximately 5-10% of pediatric brain tumors and present with signs and symptoms referable to their location, including visual loss, hormonal disturbances, hydrocephalus, and headache.
The combination of chronic presentation with the radiographic findings of a cystic lesion with readily identifiable calcification within the mass is near-pathognomic of craniopharyngioma. Regions of calcification are present in the majority of pediatric tumors (up to 90%) and over half of adult lesions.1-3 The use of CT to look for intra-tumor calcification is a helpful tool for both diagnosis and potential surgical planning (in order to assess areas of potential difficulty with resection).
The most common clinical findings include sequelae of increased intracranial pressure (ICP) due to mass effect and hydrocephalus (especially headache, nausea and vomiting), visual loss and/or endocrinologic dysfunction.4-6 These signs and symptoms are directly related to the location of the tumor and variations in the site of origin help to explain variations in presentation. Workup with endocrine, ophthalmology and neurosurgery evaluations are generally indicated.
Craniopharyngiomas are typically treated with surgery or radiation, with substantial variation in recommended approaches reported across centers. Endocrine dysfunction is common following surgical treatment of craniopharyngiomas, with reports of diabetes insipidus occurring in greater than 80% of patients and need for hormone replacement in over 90% of patients who underwent gross total resections.1, 5, 7-11 12 Equally high percentages exist for replacement of thyroid hormone, sex hormones (following puberty) and corticosteroids.12 Over half of all surgically treated children are candidates for growth hormone replacement.12
1. Samii M, Bini W. Surgical treatment of craniopharyngiomas. Zentralbl Neurochir. 1991;52(1):17-23.
2. Samii M, Tatagiba M. Surgical management of craniopharyngiomas: a review. Neurol Med Chir (Tokyo). Feb 1997;37(2):141-149.
3. Zada G, Lin N, Ojerholm E, Ramkissoon S, Laws ER. Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships. Neurosurg Focus. 2010;28(4):E4.
4. Scott RM. Craniopharyngioma: a personal (Boston) experience. Childs Nerv Syst. Aug 2005;21(8-9):773-777.
5. Karavitaki N, Brufani C, Warner JT, et al. Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin Endocrinol (Oxf). Apr 2005;62(4):397-409.
6. Jane JA, Jr., Laws ER. Craniopharyngioma. Pituitary. 2006;9(4):323-326.
7. Yang I, Sughrue ME, Rutkowski MJ, et al. Craniopharyngioma: a comparison of tumor control with various treatment strategies. Neurosurg Focus. 2010;28(4):E5.
8. Sanford RA. Craniopharyngioma: results of survey of the American Society of Pediatric Neurosurgery. Pediatr Neurosurg. 1994;21 Suppl 1:39-43.
9. Sanford RA, Muhlbauer MS. Craniopharyngioma in children. Neurol Clin. May 1991;9(2):453-465.
10. Merchant TE, Kiehna EN, Sanford RA, et al. Craniopharyngioma: the St. Jude Children's Research Hospital experience 1984-2001. Int J Radiat Oncol Biol Phys. Jul 1 2002;53(3):533-542.
11. Campbell PG, McGettigan B, Luginbuhl A, Yadla S, Rosen M, Evans JJ. Endocrinological and ophthalmological consequences of an initial endonasal endoscopic approach for resection of craniopharyngiomas. Neurosurg Focus. 2010;28(4):E8.
12. Curtis J, Daneman D, Hoffman HJ, Ehrlich RM. The endocrine outcome after surgical removal of craniopharyngiomas. Pediatr Neurosurg. 1994;21 Suppl 1:24-27.
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