The mainstay of treatment for craniopharyngioma remains surgery. If severe hydrocephalus is present, an emergency ventriculostomy may be necessary at first. Controversy exists as to the extent of surgical resection that should be attempted. The surgical approach often depends on the exact location of the tumor with respect to the chiasm and its suprasellar extension. The principles of resection involve identification of vital structures, early decompression of the cyst and dissection of the tumor from the surrounding brain. The tumor capsule itself can be densely adherent to the structures such as the pituitary stalk, optic nerves and hypothalamus. Radical surgery, while offering the greatest possibility of long term tumor control, is also associated with higher morbidity.
In the event that a subtotal resection is achieved, adjunctive therapy usually consists of conventional fractionated external beam radiation therapy up to a dose of 55 Gy. Regardless of whether radical surgery or radiation is used, endocrine replacement, partial or complete, is often the rule. Diabetes insipidus is often unavoidable in the majority of these cases. The long term survival for these patients is uncertain. Successful radical resection or limited surgery and radiation therapy appear to have similar 10-year survival rates (approximately 80-90%). Other adjunctive therapies include Gamma Knife® radiosurgery, intracavitary bleomycin and intracavity radiation.
Was this article helpful?