Symptoms of Craniopharyngioma

Craniopharyngiomas are non-cancerous brain tumors that grow near the pituitary gland and the hypothalamus. The tumor is slow growing and typically does not spread to other parts of the brain or the body. 

Craniopharyngiomas are extremely rare, occurring in approximately 2 per 1 million people and accounting for about 2% to 3% of all brain tumors. 

Craniopharyngiomas can affect the surrounding nerves that transmit visual information from the eyes to the brain (optic nerves), pituitary gland, and hypothalamus. The degree to which this can happen depends on the tumor’s exact location and size. A large craniopharyngioma can compress and disrupt the function of a number of neighboring structures.

Disruption of the optic nerves can cause vision impairment. Because the pituitary gland and hypothalamus are involved in the production and secretion of hormones that regulate a wide variety of bodily processes, craniopharyngiomas can affect many different parts of the body and aspects of life.

There are 2 types of craniopharyngiomas based on the features observed under the microscope.

This is the more common type of craniopharyngioma that presents as cystic masses with calcifications. There can be multiple cysts filled with a thick oily fluid rich in protein and blood products. Children are more often affected with this type.

Papillary craniopharyngioma is the rarer type of craniopharyngioma and more commonly affects adults. In some instances, cysts might form, but they are not a prominent feature. The tumor is typically more solid than cystic.

Symptoms of craniopharyngiomas can present for years before diagnosis because they can be vague or develop so gradually that changes go unnoticed. Additionally, if the tumor is small, symptoms may be completely absent. Early symptoms of craniopharyngiomas vary depending on the tumor's location and size. Tumors produce symptoms by compression of adjacent structures like optic pathways, the hypothalamus, and the pituitary stalk. 

Some early signs of craniopharyngiomas may include visual changes and headaches caused by increased intracranial pressure from the enlarging tumor. However, “early” is a slight misnomer, as the tumor may have existed for a long time before it grew to a sufficient size to cause symptoms.

In general, regular visits to your primary care physician provide the best opportunity for both you and your physician to monitor changes in your body. Your physician may be able to detect changes that developed too gradually for you to notice, and order additional tests or refer you to a neurosurgeon or endocrinologist if appropriate.  

Although craniopharyngiomas can also occur in adults, there are several symptoms unique to children due to the importance of hormones during normal development.

Growth-related abnormalities can occur in children with craniopharyngiomas affecting the pituitary gland. The pituitary gland produces growth hormone that promotes healthy development in children by acting on bones, muscles, and many other tissues in the body. A lack of growth hormone can lead to poor growth and symptoms such as short stature or delayed puberty. Rarely, some children with craniopharyngioma can develop puberty too early instead.

Symptoms of craniopharyngiomas vary depending on the location of the tumor and the surrounding structures impacted. We expand on important symptoms below.

The pituitary gland controls the release of multiple hormones in the body, hence its common nickname, the “master gland.” Craniopharyngiomas can affect the performance of the pituitary gland causing hormonal deficits because of compression from and invasion of the growing tumor. Commonly affected pituitary gland hormones and the results of their deficiencies include the following: 

• Growth Hormone (GH) deficiency can decrease a child’s growth rate and delay puberty.
• Luteinizing Hormone (LH) and Follicle-Stimulating Hormone (FSH) deficiency can cause sexual dysfunction and amenorrhea (absence of menses) in women.  
• Adrenocorticotrophic Hormone (ACTH) deficiency can cause symptoms like weakness, fatigue, and loss of appetite.  
• Thyroid-Stimulating Hormone (TSH) deficiency has many health implications, including generalized weakness, fatigue, menstrual irregularities, weight gain, and depressed mood.

The hypothalamus is another critical structure that has an important role in hormone production and helps to manage a wide variety of processes and feelings such as hunger and thirst, body temperature, sex drive, sleep, and emotions. The hypothalamus is located above the pituitary gland but can be affected by a large craniopharyngioma. Damage to the hypothalamus can cause symptoms such as:

• Weight gain that occurs even with calorie restriction and lifestyle changes
• Difficulty sleeping at night
• Delayed sexual development or sexual dysfunction
• High or low blood pressure
• Fluctuations in body temperature

Compression of the hypothalamus or pituitary gland can also cause increased thirst and urination in a condition known as central diabetes insipidus. 

The optic nerves carry visual information to the brain. Above the pituitary gland is the optic chiasm, where the right and left optic nerves intersect. When the craniopharyngioma compresses the optic nerves and the optic chiasm, this can lead to 2 main symptoms: blurry vision and loss of peripheral vision. Visual symptoms are among the more common symptoms in patients suffering from a craniopharyngioma. 

Many patients diagnosed with craniopharyngiomas experience headaches. Headaches can occur due to increased pressure in the brain from a large craniopharyngioma. In some cases, the tumor may obstruct passage of cerebrospinal fluid in a condition called hydrocephalus.

Craniopharyngiomas are typically benign tumors that grow at the base of the brain behind the nose. Symptoms can vary depending on a patient’s age and the size and location of the tumor. In many cases, the symptoms of the tumor present years before diagnosis and can vary depending on the structures the tumor is pressing against. 

• National Brain Tumor Society 
• Pituitary Network Association
• American Brain Tumor Association 
• Pediatric Brain Tumor Foundation 
• Children’s Brain Tumor Foundation 
• Child Growth Foundation 
• Endocrine Society 
• Pituitary Foundation