Craniopharyngioma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Craniopharyngioma is a rare, benign tumor of the central nervous system (CNS). It is a partly cystic embryonic malformation that can occur in the sellar/parasellar region and can result in a wide array of symptomatology such as headaches, nausea and vomiting, visual disturbances, and endocrine disturbances.It has an incidence of 0.5 to 2 cases per million persons per year. Half of these cases occur during the first two decades of life. It represents 1.2% to 4% of all childhood intracranial tumors. It has a classical bimodal distribution of incidence with increased incidence rates in patients aged five to 14 years and 50 to 74 years. Craniopharyngioma has a very high recurrence rate, with reported rates as high as 50%. There are two subtypes of craniopharyngioma: adamantinomatous and papillary. It most commonly presents with signs of increased intracranial pressure (ICP) including a headache and nausea and vomiting along with visual and endocrine disturbances. In children, failure to thrive and decreased growth rate can be the initial presentation. Multiple modalities can be implemented in the management of craniopharyngioma, including neurological surgery, radiotherapy, and instillation of sclerosing substances.
Historical perspective
Classification
Pathophysiology
Differentiating craniopharyngioma from other diseases
Epidemiology and demographics
- The overall incidence of craniopharyngioma is approximately 0.5 to 2 per 100,000 per year.
- The Central Brain Tumor Registry of the United States revealed an average of 338 cases diagnosed annually, with 96 occurring in children aged 0 to 4 years in this country.
- No variance by gender or race was found.
- The age distribution is bimodal with a peak in childhood and a second peak among middle-aged and older adults.
- No definite genetic relationship has been found and few familial cases reported.
- Craniopharyngioma has a very high recurrence rate, with reported rates as high as 50%.
- Interesting cases of craniopharyngioma have been reported, including craniopharyngiomas of the cerebellopontine angle, malignant transformation of craniopharyngiomas and familial cases of craniopharyngiomas.