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What is a craniopharyngioma?

craniopharyngioma
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What is a craniopharyngioma?

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A craniopharyngioma is a tumor of the brain that commonly affects children. It grows in the area of the pituitary gland and the nerves that relay vision from the eyes to the brain (optic nerves), and may grow up into the base of the brain. The tumors arise from cells that in the developing embryo had helped to form the normal pituitary gland. For reasons that are not understood, these cells begin to grow on their own, producing masses that often contain both solid tissue and fluid. In children, portions of the tumor frequently have calcium deposits. The fluid (“cystic”) portions of the tumors can reach very large size, and extend into both sides of the brain.

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A Craniopharyngioma is a benign brain tumour. A tumour is a condition where a part of the body grows more than it would normally. Benign means that bits of the tumour will not normally break off and spread to other parts of the body. It may, with time however grow and get bigger. The part of the brain that is affected is very near to the optic chiasm. The optic chiasm may become damaged by the growth of a Craniopharyngioma. This is why this condition may cause visual impairment. Other parts of the brain may also be damaged leading to many other different types of symptoms. How might a Craniopharyngioma affect a child’s eyes and vision? A Craniopharyngioma may cause: • Visual Field Loss • Blurred Vision • Nystagmus Visual Field Loss The visual field is the medical word for the full area of a child’s visual world (what they see). Visual Field Loss may occur if damage occurs to a part of the visual pathway. The visual pathway that signals travel along includes the eye, optic nerve, optic

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Craniopharyngiomas are intracranial tumors that are typically both cystic and solid in structure. They occur most commonly in childhood and adolescence and in later adult life after age 50 years. They account for 2-4% of primary brain tumors, 10-15% of sellar and suprasellar tumors (tumors that occur in and above the pituitary fossa) and 50-60% of sellar and suprasellar tumors in children. Craniopharyngiomas are usually not discovered until they impinge upon important structures around them, and are frequently quite large (over 3 cm) when detected. They are histologically benign (not malignant) tumors, but they have a tendency to become adherent to structures in and around the pituitary gland and pituitary stalk, including the optic nerves, optic chiasm, intracranial arteries and the brain itself. They are thought to arise from remnants of the craniopharyngeal duct or Rathke’s pouch which are developmental structures related to the primitive gut. Embryonic cells from an incompletely in

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A craniopharyngioma is a tumor that grows near the pituitary gland in the brain. These tumors tend to occur in children more frequently than adults. Craniopharyngiomas frequently contain both solid and cystic parts that usually enlarge over time to compress the neighboring structures in the brain such as the optic nerves, the pituitary gland, and even the ventricular system.

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A craniopharyngioma is a benign tumor in that it does not transform into malignant cancer, but because it is difficult if not impossible to remove and continues to grow in spite of most x-ray treatments its behavior is “malignant” even though it does not metastasize. Who is most likely to have a craniopharyngioma? 2.5- 4% of all brain tumors are craniopharyngiomas. 50% of craniopharyngiomas occur in the pediatric age group with a peak incidence in children between 5 and 10 years old. What is the treatment? The treatment for craniopharyngioma is surgery which can include an attempt to remove part, but rarely all of the tumor, VP shunting may be necessary to preserve the patency of the CSF circulation pathways. Radiation therapy can be administered to the brain or by implants placed directly into the cyst.

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