The craniopharyngioma is a benign brain tumor that is located at the base of the skull in the region of the pituitary gland. Due to this location, there can sometimes be difficulty in distinguishing the craniopharyngioma from a pituitary tumor on CT or MRI scans. These brain tumors are benign and cause symptoms by local pressure on the brain or blockage of spinal fluid, which can result in hydrocephalus (build-up of spinal fluid within the brain). Craniopharyngiomas represent between 2 and 3 percent of all primary brain tumors, and between 5 to 13 percent of brain tumors in children. The peak age of discovery ranges between 5 and 10 years.
SYMPTOMS
The symptoms of craniopharyngioma relate to its location and size. These symptoms include those related to increased intracranial pressure (pressure within the skull) caused by blockage of spinal fluid pathways causing hydrocephalus (Build-up of spinal fluid within the brain). Additionally, decreased vision, particularly peripheral vision may be noted. Also, pressure on the hypothalamus and pituitary gland may cause obesity and delayed development.
DIAGNOSIS
A careful history and physical examination are important. The imaging test that best shows these tumors is an MRI performed both without and with intravenous contrast material. An ophthalmological consultation and an endocrinological consultation should also be obtained.
TREATMENT
The most effective treatment for craniopharyngioma is surgery. An attempt should be made to completely resect the brain tumor. If there is residual tumor, consideration is given to radiation therapy or stereotactic radiosurgery. If possible, radiation is withheld in younger children to prevent developmental delay. Unfortunately, tumor recurrence is not unusual.