Craniopharyngiomas are benign (noncancerous) tumors that grow at the base of the brain near the pituitary gland. These tumors can put pressure on different parts of the brain, which can cause additional symptoms in the body. While treatment options can be complex given the tumor’s size and location, surgery is known to be successful.
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What Is Craniopharyngioma?
Craniopharyngioma is a benign brain tumor that originates in the pituitary gland. This pea-sized gland is responsible for secreting hormones into the body.
Though benign and unable to spread, craniopharyngioma tumors can grow and place excessive pressure on the brain and cause severe headaches, vision problems, and vomiting. Damage to the pituitary gland can lead to hormonal problems, ranging from excessive thirst to stunted growth.
What are the types of craniopharyngioma?
There are two key types of craniopharyngiomas:
- Adamantinomatous (ordinary) craniopharyngioma — Occurs in children and is more common.
- Papillary craniopharyngioma — Occurs in adults and is less frequently diagnosed.
How common is craniopharyngioma?
Craniopharyngioma is rare — fewer than 1,000 new cases are diagnosed in children and adults each year in the United States.
What causes craniopharyngioma?
The exact cause of craniopharyngioma is not known, but research suggests that certain changes to brain cell function can trigger the development of craniopharyngiomas.
Craniopharyngioma risk factors
You are at a higher risk for craniopharyngioma if you are:
- Between the ages of 5 and 10.
Complications of craniopharyngioma
Other conditions may result from damage to the pituitary gland caused by the craniopharyngioma, including:
- Adrenal failure (dizziness, hypoglycemia, cardiac arrhythmias, lethargy, confusion, anorexia, nausea, and vomiting).
- Decreased sexual drive, including impotence in men and amenorrhea in women.
- Delayed puberty and stunted growth in pediatric patients.
- Diabetes insipidus (excessive thirst and urination).
- Hypothyroidism (weight gain, fatigue, cold intolerance, and constipation).
- Overeating, obesity, and sleepiness.
How can I prevent craniopharyngioma?
There is no way to prevent craniopharyngiomas, as these cell changes happened while you or your child were still being developed in utero.
What Are the Signs and Symptoms of Craniopharyngioma?
Depending on the hormones or area(s) of the brain the tumor is affecting, craniopharyngioma symptoms can include:
- Balance problems.
- Delayed puberty in children.
- Disruption of hormone production by the pituitary gland.
- Excessive thirst.
- Excessive urination.
- Extreme tiredness and fatigue.
- Headaches due to increasing pressure on the brain.
- Hearing loss.
- Nausea and vomiting.
- Personality changes.
- Progressive loss of vision.
- Slow growth in children.
- Vision problems due to the tumor pressing on or damaging the optic nerve.
- Weight gain.
When should I see a doctor about my craniopharyngioma symptoms?
If you or your child are experiencing any of the above symptoms, make an appointment with your doctor or child’s pediatrician. Early diagnosis of craniopharyngioma can lead to better treatment outcomes.
How Do You Diagnose Craniopharyngioma?
To make the diagnosis of craniopharyngioma, your doctor may:
- Ask you about your symptoms and medical history.
- Order diagnostic tests, such as MRI and CT scans.
- Perform a physical exam.
Depending on your other symptoms, such as hormonal and visual problems, your doctor may refer you to an endocrinologist or ophthalmologist to further diagnose your craniopharyngioma.
What to expect during your visit
First, your doctor or your child’s pediatrician will ask about symptoms, health history, and family history. Because some symptoms may indicate other conditions, you can expect diagnostic tests to be ordered.
These tests may include:
- Blood hormone study — Studies a blood sample to determine the amount of certain hormones in the blood. Higher or lower than normal results of certain hormones may indicate craniopharyngioma.
- Imaging tests — Such as CT scans or MRIs help doctors get a better image inside the body.
- Visual field exam — Checks a person’s field of vision, or total area of vision. They will test central and peripheral vision, as loss of eyesight is a symptom of a benign brain tumor pressing on the optic nerve.
- Biopsy for craniopharyngioma — Takes a small tumor or tissue sample to be examined under a microscope for cancer cells. During this procedure, a surgeon will either remove the tissue from a needle in the skull in an open or minimally invasive surgery, or remove the biopsy sample through the nose. The tissue sample is then sent to a lab for a pathologist to view and diagnose.
Craniopharyngioma prognosis
While most people who have craniopharyngioma live for many years after diagnosis, there is a significant risk for recurring (returning) tumors. Most recurring tumors come back within three years of surgical removal.
How Do You Treat Craniopharyngioma?
Most craniopharyngioma cases can be treated with minimally invasive surgery, although surgery can vary depending on the size, shape, and location of the tumor. For some people, radiation therapy after surgery may be recommended.
Minimally invasive surgery
Surgery is the primary treatment for craniopharyngiomas.
Craniopharyngiomas of the skull base may be approached directly using the endoscopic endonasal approach (EEA). This state-of-the-art, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. Surgeons then remove the craniopharyngioma through the nose and nasal cavities.
EEA offers the benefits of no incisions to heal, no disfigurement, and a faster recovery time. If you need complementary treatments, such as radiation, those therapies can begin soon after EEA surgery.
Although the goal of the surgery is total tumor removal with pituitary function preservation, in some cases the craniopharyngioma's proximity to critical brain structures (the hypothalamus, optic chiasm, and blood vessels) makes complete removal impossible.
The expanded endonasal approach gives surgeons the best view of the undersurface of the optic nerves, chiasm, and pituitary stalk, and helps them decide whether to leave residual tumor attached to these important structures.
In cases where the tumor has clearly invaded the pituitary gland, surgeons may also remove the pituitary gland.
Gamma Knife® radiosurgery
Gamma Knife radiosurgery is a painless treatment that uses hundreds of highly focused radiation beams to target tumors and lesions within the brain, with no surgical incision.
Surgeons may use Gamma Knife radiosurgery:
- After surgery to treat residual craniopharyngioma tumors.
- At the time of tumor recurrence.
Doctors at UPMC — the nation's leading provider of Gamma Knife procedures — have used this technology to effectively treat 12,000 patients with tumors, vascular malformations, pain, and other functional problems.
Radiation therapy
In instances where it's not safe to remove a craniopharyngioma tumor completely, radiation may follow surgery as the next preferred treatment.
Radiation therapy for residual craniopharyngioma tumors:
- Eradicates the remaining tumor cells.
- Has success rates on par with those of complete tumor removal.
