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Craniopharyngioma is a type of brain tumor derived from pituitary gland embryonic tissue that occurs most commonly in children but also in men and women in their 50s and 60s. People may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopia, as the tumor may compress the optic chiasm. Craniopharyngiomas are typically very slow growing tumors. They arise from the cells along the pituitary stalk, specifically from nests of odontogenic (tooth-forming) epithelium within the suprasellar/diencephalic region and, therefore, contain deposits of calcium, which are evident on an x-ray. Balance disorder, Dry skin, Fatigue, Fever, Headache, Hypersomnia, Lethargy, Myxedema, Nausea etc. include is symptoms.
Childhood craniopharyngioma is rare, occurring in only two or three children per million. There are approximately 100 cases per year in the United States in children under the age of 18 years. Diagnosis most often occurs between ages 5 and 14. Boys and girls are equally likely to develop craniopharyngioma. The survival rate is 80% when measured 10 years after treatment. Long-term complications, such as the need for hormone replacement and problems related to brain damage, may occur.
Treatment generally consists of subfrontal or transsphenoidal excision. Surgery using the transsphenoidal route is often performed by a joint team of ENT and neurosurgeons. Because of the location of the craniopharyngioma near the brain and skullbase, a surgical navigation system might be used to verify the position of surgical tools during the operation. Additional radiotherapy is also used if total removal is not possible.
St. Jude Hospital in USA has a clinical trial using proton therapy for craniopharyngioma known as RT2CR. St. Jude is currently building the world’s only proton therapy center dedicated solely to the treatment of children. Proton therapy can deliver high radiation doses directly to tumors. This treatment spares normal tissues and reduces the side effects of traditional X-ray therapy. Because it is so precise, proton therapy reduces a child’s risk of having serious side effects and of developing other cancers later in life. The hospital has one of the largest pediatric brain tumor programs in the United States.
