Craniopharyngiomas are benign suprasellar
tumors that arise from epithelial remnants of the Rathke'
s pouch. The two standard treatment options are primary total resection or limited surgery followed by external beam radiation. The 10- and 20-year progression-free survival rates following limited surgery and
radiation therapy are superior to those achieved by primary surgery alone. The side effect profiles for these two treatment approaches are different. Following total resection there is a very high incidence of
panhypopituitarism requiring lifelong multiple
hormone replacement therapy. The other side effects include potential damage to adjacent structures such as optic chiasm, vasculature and hypothalamus. Following limited surgery and
radiation therapy the incidence of endocrine deficits is significantly lower compared to radical surgery, as is the risk of neurovascular and hypothalamic injury.
Optic neuropathy and brain
necrosis are rare in modern
radiation therapy series. Second
malignant neoplasms, although rare, can occur. In children with recurrent
craniopharyngiomas following radical surgery, the recommended
salvage treatment is
radiation therapy, as further surgical attempts at salvage are associated with high relapse rates and increased morbidity and mortality. There have been significant technological advances in the field of
radiation treatment planning and delivery that have great potential for reducing the incidence of long-term irradiation sequelae in the developing brain. The general availability of megavoltage
linear accelerators and modern
radiotherapy innovations such as three-dimensional conformal
radiation treatment (3D CRT), stereotactic radiosurgery (SRS), stereotactic
radiotherapy (SRT), and intensity modulated
radiation therapy (IMRT) should further limit the rate of complications and improve cure rates in children with primary or recurrent
craniopharyngioma.