What is a Craniopharyngioma?

A craniopharyngioma is a benign tumor arising from squamous cell rests found along the path of the primitive craniopharyngeal duct and adenohypophysis.  Craniopharyngiomas are located intracranially but outside the brain, near the anterior base of the cerebrum, in a location termed the sella and suprasellar region.  Craniopharyngiomas will frequently grow to involve the third ventricle, hypothalamus, optic chiasm and pituitary gland.  A Craniopharyngioma is classified as a benign tumor, but its impact can be significant due to its location near critical structures.

Craniopharyngioma accounts for 5-10% of pediatric brain tumors.  Craniopharyngiomas have a peak incidence in children 5-14 years of age and another peak incidence in older adults 65-74 years of age.  The cause of craniopharygioma is unknown.

What are the symptoms of a Craniopharyngioma?
The symptoms of craniopharyngioma are related to its location near the 3rd ventricle, optic chiasm, pituitary gland and hypothalmus.  Growth of the craniopharyngioma into the 3rd ventricle results in obstruction of flow of spinal fluid causing signs and symptoms of increased cranial pressure (headache, vomiting, papilledema (swollen eye nerve seen on ophthalmological exam), difficulty with balance, and lethargy).  Pressure on the optic chiasm results in loss of vision described as “blind spots”, typically with loss of peripheral vision (also known as tunnel vision).  Pressure on the pituitary gland can cause failure to produce pituitary hormones which may cause any of the following symptoms: poor growth, decreased energy, weight gain, cold intolerance, lack of energy, pubertal delay or precocious puberty, and increased thirst and urination.  Craniopharyngiomas that cause pressure on the hypothalamus result in failure to thrive or obesity.

How is a Craniopharyngioma diagnosed?
A computerized axial tomography (CAT or CT) scan and an MRI scan are both helpful in the diagnosis of craniopharyngioma.  The MRI precisely defines the location of the tumor in relation to the surrounding brain and vasculature.  A CT scan helps differentiate it from other tumor types because CT scans easily show tumor calcification, which is a unique feature seen in craniopharyngioma.  It should be noted that craniopharyngioma is typically a tumor with both a solid component and a cystic component.

How is a Craniopharyngioma treated?
Craniopharyngioma can be treated with surgical removal or radiation therapy.  Because children are more susceptible to the long-term toxicities of radiation, complete surgical removal of the tumor is the most common approach.  Complete surgical removal can often result in injury to the hypothalamus resulting in lifelong obesity, further injury to the optic chiasm resulting in worsening visual impairment, and further injury to the pituitary resulting in further pituitary insufficiency.   If complete removal of the tumor is not possible without causing unacceptable side effects, radiation therapy can effectively treat craniopharyngioma.  Radiation can also cause further injury to the optic pathway and pituitary resulting in further visual impairment and pituitary dysfunction.  In rare cases where the tumor has a large predominant cyst, aspiration of the cystic fluid or injection of chemotherapy or a radioactive material into the cyst can be useful in managing the tumor.

About treatment for Craniopharyngioma at Children’s Hospitals and Clinics of Minnesota

Our cancer and blood disorders program consistently achieves excellent results ranking it in the top ten programs in the United States. Children’s Hospitals and Clinics of Minnesota treats the majority of children with cancer and blood disorders in Minnesota and provides patients access to a variety of clinical trials using ground-breaking new treatments. Through our renowned program, patients experience unparalleled family support, a nationally recognized pain management team, and compassionate, coordinated care.

If you are a family member looking for a Children’s Hospitals and Clinics of Minnesota neuro-oncologist, please call our clinic at 612-813-5940.

If you are a health professional looking for consultation or referral information, please call Children’s Physician Access at 1-866-755-2121 (toll-free) and ask for the on-call hematologist/oncologist.

Additional information on Craniopharyngioma

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