Craniopharyngiomas may involve the pituitary gland. They account for 6-9% of all CNS tumors in children. The peak incidence is 5-10 years of age. Patients present with symptoms of increased ICP, visual loss, and endocrine deficiencies. They typically require replacement therapy with cortisone, thyroxine, growth hormone, and sex hormones. These tumors are slow-growing benign lesions amidst vital anatomic structures.
Craniopharyngiomas should be completely removed if possible without producing untoward neurologic sequelae. Complete excision is possible in 60-90% of cases. If radical excision can be accomplished without significant postoperative morbidity, a primary surgical approach is warranted. However, controversy exists over whether subtotal resection followed by radiation will produce less morbidity and be an equally effective strategy. Complete tumor removal is most easily accomplished in cystic tumors and is most difficult in solid or mixed tumors larger than 3 cm in size.
In tumors in which conservative surgery consisting only of drainage or subtotal removal is performed, the addition of radiotherapy reduces the local recurrence rate and improves long-term survival. For children older than 5 years, 5000-5500 cGy are given. The dose is reduced to 4000-4500 cGy in children less than 5 years of age.
At present there is no established role for chemotherapy in craniopharyngioma. A recent study demonstrated that 25% of patients (primarily those with cystic lesions) treated with interferon-alpha 2a responded.
The long-term survival of patients treated with radical and total removal is 80-90% at 5 years and 81% at 10 years. The 5-year recurrence-free survival after subtotal removal is approximately 50%. Survival after partial removal and radiation therapy is 62-84%.
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