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Craniopharyngioma

Background

Craniopharyngiomas are benign brain tumors that grow in the pituitary fossa. Craniopharyngiomas are usually large by the time they are discovered, and cause no symptoms until they begin to damage adjacent organs.

Although craniopharyngiomas are benign tumors, they can be life threatening and difficult to cure. They are thought to be closely related to Rathke’s Cleft Cysts (RCCs).

Evaluation

If the craniopharyngioma compresses the Pituitary Stalk or Gland, it can cause Pituitary Hormone Deficiency, with growth failure, delayed puberty, loss of normal menstrual function and sexual desire, increased sensitivity to cold, fatigue, constipation, dry skin, nausea, low blood pressure, and depression.

Craniopharyngiomas can also cause Diabetes Insipidus (DI), and can increase Prolactin levels, causing a milky discharge from the breast (galactohhrea).

If the craniopharyngioma compresses the optic chiasm or nerves, then visual loss can result.

Involvement of the hypothalamus, an area at the base of the brain, may cause obesity, increased drowsiness and temperature regulation problems.

Larger tumors may cause personality changes, headache, confusion, and vomiting.

Studies

A pituitary MRI and skull base CT are the best images for evaluating a craniopharyngioma.

Pituitary hormonal evaluation should also be performed.

Other diagnoses to be ruled out when suspecting craniopharyngioma are cystic pituitary adenoma and arachnoid cyst.

 

Treatment

  • Surgery: The best initial treatment for a craniopharyngioma is surgery. The goal of surgery is to maximally remove the tumor while minimizing damage to adjacent structures. Most craniopharyngiomas can be removed by either an endonasal endoscopic approach (through the nose) or an eyebrow craniotomy. Because of their tendency to adhere to the optic pathways and other nerves, complete removal is possible in only about half of patients.
  • Cyberknife Radiosurgery (SRS): After incomplete removal, stereotactic radiosurgery (SRS) can help delay recurrence, sometimes achieving cure.
  • Hormonal Replacement Therapy: Many patients with a craniopharyngioma will develop Pituitary Hormonal Deficits, and will require hormone replacement therapy. Because hormone deficiency can develop years after treatment, patients should have annual hormonal evaluations for decades. Follow-up with an endocrinologist is recommended for all craniopharyngioma patients.