Craniopharyngioma

Craniopharyngiomas are benign tumors that occur at the base of the brain, above the pituitary gland. The term comes from:

• Cranio, meaning head
• Pharyng, meaning throat
• Oma, meaning condition

Craniopharyngiomas nearly always involve the pituitary stalk, the connection between the pituitary gland and the brain.

Carniopharyngioma: Expert Care in Southern California

The UCLA Pituitary Tumor Program offers comprehensive management of carniopharyngiomas. Our physicians have years of experience in diagnosing, treating and managing pituitary conditions. Our sophisticated diagnostic equipment, team approach and minimally invasive surgical procedures mark our program as one of the best in the country.

Learn more:

• Physiology
• Symptoms
• Diagnosis
• Treatment
• Contact Us

Craniopharyngioma: Physiology

Craniopharyngiomas occur in both children and adults. Most children are diagnosed between five and 10 years of age. Craniopharyngiomas are relatively rare.

Craniopharyngiomas can grow to large sizes, even bigger than a golf ball, and may not cause any symptoms in the beginning.

There are two types of craniopharyngiomas:

• Adamantinomatous craniopharyngioma. Under the microscope, these cells look very similar to cells that produce tooth enamel. This type commonly calcifies, which is visible on computed tomography (CT) scanning and helpful in making the diagnosis.
• Papillary craniopharyngioma. This type rarely calcifies.

Craniopharyngioma: Symptoms

Symptoms produced by a craniopharyngioma vary depending upon the tumor's location and the age of the patient. The symptoms also depend on which specific hormone is involved. Learn more about normal pituitary function.

Symptoms include:

• Personality changes
• Headache
• Confusion
• Vomiting
• Pituitary insufficiency. This often occurs to some degree because craniopharyngiomas develop in the area of the pituitary stalk, which can affect the function of the pituitary gland.
• In children, hormone deficiency can result in:
  • Growth failure
  • Delayed puberty

Other symptoms include:

• Diabetes insipidus. This occurs due to the absence of a posterior pituitary hormone called antidiuretic hormone (ADH). Symptoms include:
  • Excessive thirst
  • Excessive urination
• Adrenal insufficiency. This occurs because of a reduction in ACTH production, a reduction in cortisol. In severe cases, it can be fatal. Symptoms include:
  • Fatigue
  • Low blood pressure
  • Electrolyte abnormalities
• Growth hormone (GH) insufficiency, caused by the reduction in growth hormone (GH) production. Symptoms include:
  • Stunted growth and delayed puberty (in children)
  • General fatigue, loss of muscle mass and tone (in adults)
• Hypothyroidism, caused by a reduction in TSH production. Symptoms include:
  • Loss of appetite
  • Weight gain
  • Fatigue
  • Decreased mental function
• Reduction in prolactin production. This is very uncommon and occurs with severe pituitary insufficiency.
  • Large pituitary tumors can paradoxically elevate blood prolactin levels due to the "stalk effect." This elevation occurs as a result of the compression of the pituitary stalk, which interferes with the brain's control of prolactin production.
  • In premenopausal women, elevated prolactin can lead to reduction or loss of menstrual periods and/or breast milk production (galactorrhea).
  • With stalk effect, prolactin levels are usually only slightly elevated, as opposed to prolactinomas in which the prolactin level is usually very high.
• Reduction of sex hormones, luteinizing hormone (LH) and follicle-stimulating hormone (FSH).
  • In men, this can lead to a low testosterone level, causing decreased sexual drive and impotence.
  • In some cases, there can be loss of body and facial hair.
  • In women, this can lead to infertility

If the tumor involves the optic tracts, chiasm, or nerves, blindness can occur.

If the hypothalamus, an area at the base of the brain, is affected, symptoms may include:

• Obesity
• Increased drowsiness
• Temperature regulation abnormalities
• Diabetes insipidus

Craniopharyngioma: Diagnosis

Your doctor will conduct a physical examination and ask you about your symptoms and medical history. Diagnostic procedures include:

• Imaging scans
• Pituitary function testing

Imaging Scans for Craniopharyngioma

Your doctor may order a high-resolution magnetic resonance imaging (MRI) scan. This test is valuable because it allows the neuroradiologist to view the tumor from different angles.

In some cases, a powerful 3T (Tesla) MRI scanner can help define the location of critical brain structures affected by the tumor.

A computed tomography (CT) scan is also a good diagnostic tool as it detects calcification in the tumor

Pituitary Function Testing

Pituitary hormone function testing is necessary for every patient with a craniopharyngioma. An endocrinologist who specializes in pituitary tumors will help evaluate the results.
 

Craniopharyngioma: Treatment Options

Typically, a medical team, combining specialists from many disciplines, will manage your treatment. The UCLA Pituitary Tumor Program has experts in each of the specialties, working closely together to provide you with the most comprehensive, state-of-the-art surgical and non-surgical treatments.

Treatment options include:

• Surgery
• Radiation Therapy
• Medication

Surgery for Craniopharyngioma

If you are diagnosed with craniopharyngioma, you may require surgery to:

• Establish or confirm the diagnosis by removing a sample of tumor tissue for analysis
• Decompress the optic chiasm (the part of the brain where the optic nerves partially cross) to improve vision
• Remove as much of the tumor as safely possible

There are several surgical approaches to craniopharyngioma tumors. Your medical team will decide which approach to use based on:

• Location of the tumor relative to key brain structures
• Size of the tumor and whether it is cystic (has fluid-filled cavities)
• How extensively the tumor invaded surrounding structures, particularly the hypothalamus
• Your age and overall health

The goal of surgery also differs for children and adults:

• In children, the surgeon will attempt to remove the entire tumor if safely possible. Your doctor may recommend a minimally invasive endoscopic endonasal approach, which may cause less hypothalamic injury. It also means you will have no visible scars after surgery.
• In adults, doctors often use a more conservative approach, aiming to establish a diagnosis and decompress the optic nerves, but not necessary attempt full removal of the tumor. Because the tumor almost always involves the pituitary stalk, attempting to remove the entire tumor often results in complete pituitary insufficiency.

Craniopharyngioma: Surgical Approaches

The two basic approaches are:

• Endonasal
• Craniotomy

Endoscopic Endonasal Surgery

The endoscopic endonasal approach is a minimally invasive approach, using your natural nasal passageway. It does not require a head incision. Our neurosurgeons have the advanced training and extensive experience necessary for performing this complex procedure.

An endoscopic technique can be very effective in safely removing tumor, while at the same time minimizing hospitalization time and discomfort.

However, not every craniopharyngioma tumor can be removed using an endonasal endoscopic approach. If you wish to find out whether you are a candidate for an endoscopic approach, please arrange a film review at [email protected].

Open Craniotomy

During an open craniotomy, your surgeon:

1. Makes an incision into your scalp
2. Temporarily removes a piece of your skull
3. Uses a surgical microscope to provide high magnification of the area
4. Access and remove the tumor; the most common approach involves gently elevating the base of the frontal lobe of the brain, above the eye.

Your surgeon may be able to use a minimally invasive eyebrow incision keyhole approach.

Possible consequences and complication of surgery

• Children:
  • When surgeons attempt a complete resection of the craniopharyngioma tumor, it may result in complete pituitary insufficiency, requiring lifelong hormone replacement therapy.
  • Damage to the hypothalamus can cause many problems, including severe obesity.
• 

  Adults and children

  • Diabetes insipidus may develop.
  • Damage to the optic nerves can cause blindness.
  • If your surgeon used an endonasal approach, it may lead to leakage of cerebrospinal fluid (CSF) through the nose, requiring repeat surgery.

Radiation Therapy for Craniopharyngiomas

Your doctor may recommend radiation therapy following surgery. Stereotactic radiosurgery is a technique that uses a highly focused dose of radiation delivered directly to the tumor. Because the radiation beam is sculpted to target only the tumor, the surrounding brain structures receive only a fraction of the radiation dose and may remain unharmed.

At the UCLA Pituitary Tumor Program, we use the advanced Novalis® Radiosurgery delivery system, which allows us to treat irregularly shaped tumors that are close to critical brain structures.

One drawback of radiation treatment is that it leads to delayed pituitary failure. This typically occurs several years after treatment, necessitating complete hormone replacement.

Medical Therapy for Craniopharyngiomas

Pituitary hormonal dysfunction is common after craniopharyngioma surgery and/or radiation therapy. Problems with hypothalamic function can be particularly challenging to treat. You will require careful monitoring and follow-up visits with an endocrinologist.

Contact Us

To schedule an appointment with one of our physicians at the Pituitary Tumor Program, please call (310) 825 5111.