Definition

Craniopharyngiomas are benign, slow growing cystic tumors that grow from cells on the pituitary stalk. They are related to Rathke’s Cleft Cysts.


Pre-operative image (side view) showing a craniopharygioma inside the area of the dark circle.

Causes

These tumors most commonly affect children between 5 and 10 years of age and adults between 40 – 60, but they can occur in any age group. They affect males and females equally. There is no known cause.

Risks

Risk factors are things associated with an increased chance of developing a disease or condition. There are no known risk factors for craniopharyngioma.

The main risks of a craniopharyngioma relate to problems caused by pituitary gland dysfunction and damage to the optic nerve which can cause visual problems.


Pre-operative MRI showing a craniopharyngioma from the front

Symptoms

There are several ways that a craniopharyngioma can cause symptoms:

  • Direct pressure on the brain as the tumor enlarges or obstructs the 3rd ventricular may cause headaches, nausea/vomiting and balance problems
  • Hormonal imbalances caused by pituitary gland dysfunction can lead to excessive urination and thirst, stunted growth (in children), weight gain, and lethargy, among other symptoms
  • Damage to the optic nerve can lead to loss of visual fields and other visual disturbances

Diagnosis

Craniopharyngiomas are generally diagnosed after patients present with symptoms. Your doctor will take a medical history and order additional tests, such as:

  • MRI and/or CT of the brain
  • Neurological examination
  • Endocrine hormone levels

Treatment

The decision to treat or not to treat depends on the size, the precise location and shape of the lesion as well as its rate of growth, if known. In addition, the presence of symptoms, especially visual loss virtually always indicates the need for removal.

  • Surgery has traditionally been the primary treatment, usually requiring an open cranial procedure. As minimally invasive, transnasal approaches have evolved, many craniopharyngiomas can now be removed through the nose.
  • Radiation
  • Hormone replacement treats the side effects of the tumor and treatments


MRI demonstrating complete resection of the tumor, as seen from the side.


Post-operative MRI showing complete resection of the tumor, as seen from the front.

Prevention

There is no known way to prevent craniopharyngiomas.

Case Study

A teacher in her mid-fifties was seen after noting nausea, vomiting, fatigue, and rapidly deteriorating loss of short term memory over several months. An MRI revealed a suprasellar craniopharygioma. Endocrine studies showed that her pituitary gland was no longer functioning. She started cortisol which helped some of her symptoms but not the memory problems. After a thorough discussion of her surgical options the patient and her spouse decided to schedule an extended transnasal sublabial resection with Dr. Joshua Bederson. The patient underwent an extended transnasal, endoscopically assisted approach and resection of the tumor. This was performed by Dr. Bederson and Dr. Eric Genden. After recovering from surgery, the patient returned home and experienced a gradual improvement in her memory deficits, which have now completely disappeared. Post operative imaging demonstrates no residual tumor. She has since resumed her full time teaching schedule.

If you want to learn more about Craniopharyngiomas call the Mount Sinai Department of Neurosurgery at 212-241-2377. 

Images used with permission from Joshua B. Bederson, MD