Craniopharyngioma

What Causes Craniopharyngioma?

Scientists don’t know what causes craniopharyngiomas. The root cause of a tumor is a mutation or damage in the genes that control the growth of affected cells. The specific cause of the gene damage that triggers a tumor’s formation is usually not identifiable. In a healthy cell, these genes prevent the cell from growing or reproducing too rapidly, and the genes can also determine the cell’s normal lifespan. In a tumor’s cells, the damage to the genes causes the cells to grow and reproduce rapidly, and the cells may live longer than usual. As this rapid growth and reproduction continue, the cells grow into an abnormal mass.

Is Craniopharyngioma Hereditary?

Craniopharyngiomas do not appear to be linked to inherited traits. Instead, researchers believe the tumors result from external environmental factors or changes within cells that occur randomly and with no external trigger. 

How Is Craniopharyngioma Detected?

Slow-growing craniopharyngiomas may develop for an extended time without producing symptoms. However, as they grow larger, they are likely to result in noticeable symptoms. Craniopharyngiomas in children are most often diagnosed between 5 and 14.

Some warning signs of a craniopharyngioma may include:

• Headaches
• Nausea or vomiting
• Vision problems
• Problems walking
• Slow growth in children

How Is Craniopharyngioma Diagnosed?

Doctors may take several different diagnostic steps when they suspect a patient may have a craniopharyngioma.

• Neurological exam. A basic neurological exam will test a patient’s reflexes, balance, coordination, strength, vision, and hearing. This exam may prompt a doctor to look further for a tumor’s presence, giving a clue about the affected part of the brain.
• Imaging. Imaging technologies are non-invasive ways to look at brain tissue and possibly detect a tumor’s presence. They may also be used to judge the tumor’s size, location, and growth. Magnetic resonance imaging (MRI) uses a strong magnetic field to produce images of the brain and central nervous system. Computerized tomography (CT) scan may also be used to look for tumors.
• Laboratory tests. Blood tests and urine tests may be able to detect abnormal hormone levels that could indicate a tumor affecting the function of the pituitary gland.

How Is Craniopharyngioma Treated?

Treatment of a craniopharyngioma varies depending on the tumor’s growth rate, location, and size.

Surgery

Surgical removal of a tumor is usually the first step in treating a craniopharyngioma. Some small tumors may be removed using a technique called endoscopic nasal surgery. In this procedure, a small incision is made inside the nasal cavity, and the tumor is removed via the nose and sinuses.

Some tumors may require a procedure called a craniotomy. In this case, the surgeon makes an incision in the scalp and removes part of the skull to reach the tumor. In some cases, surgeons may be able to remove the tumor using a technique called orbitozygomatic craniotomy. This procedure is less invasive than some other types of surgery.

Craniopharyngiomas tend to recur, and many people have to undergo multiple surgeries over their lifetime to remove regrowing tumors.

Radiation Therapy

Radiation therapies involve using high-energy x-rays to target and kill tumor cells directly. Radiation is typically focused on the tumor to avoid damaging healthy cells. Radiation therapy is often used when the tumor can’t be entirely removed with surgery, or if the tumor begins to grow again after surgery.

Side effects of radiation therapy may include headaches, memory loss, fatigue, and scalp reactions. The therapy may also affect the function of the pituitary gland.

Chemotherapy

Chemotherapy uses chemicals that intentionally damage the body’s cells with the expectation of healthy cells recovering from the damage more easily than tumor cells. This type of therapy may be used to treat recurring craniopharyngiomas.

Medications

Hormone-replacement treatment may be necessary if the tumor impairs the normal function of the pituitary gland or if other treatments lead to pituitary impairment.

How Does Craniopharyngioma Progress?

With treatment, the outlook for most people with craniopharyngiomas is good. The 20-year survival rate is more than 90%. However, the tumors tend to regrow after removal, and the need for lifetime monitoring and treatment is common.

Some craniopharyngiomas and associated treatment procedures can produce long-term complications, including:

• Hormone deficiencies
• Weight gain
• Growth problems
• Diabetes
• Liver problems
• Heart problems
• Respiratory problems
• Stroke
• Behavior or mood changes

How Is Craniopharyngioma Prevented?

There is no known way to prevent craniopharyngiomas from occurring.

Craniopharyngioma Caregiver Tips

Caring for someone with a brain tumor can be even more challenging than the already high demands of caring for someone with any other type of severe and progressive illness. Along with the physical changes that make other cancers and serious illnesses so physically and emotionally exhausting to deal with, brain tumors also often produce psychological and cognitive changes in the patient that can threaten the caregiver’s well-being.

As you care for your loved one through the progressive stages of their illness, keep these tips in mind:

• Learn as much as possible about the potential effects of your loved one’s specific type of brain tumor. This will allow you to understand how the illness affects the sufferer’s behavior.
• Get help from your friends and family. Caring for a brain tumor patient is a huge task, and you shouldn’t try to do it alone.
• Take time whenever possible to step away from the patient and the illness and find time for yourself. Acknowledge that it is normal and acceptable to need occasional relief from caregiving burdens.
• Find a support group. It can be beneficial to learn that you are not alone, and other people understand what you are going through.

Some people with pituitary tumors also suffer from other brain and mental health-related issues, a condition called co-morbidity. Here are a few of the disorders commonly associated with these tumors:

• People with brain tumors often experience depression or anxiety.
• Personality changes resembling bipolar disorder are sometimes an indication of a brain tumor.

Craniopharyngioma Brain Science

Although craniopharyngiomas are rarely cancerous, they cause problems when their growth interferes with the function of critical surrounding tissues. The areas most often affected by these tumors are the pituitary gland, the optic chiasm (where the optic nerves cross en route to the brain), and a part of the brain called the hypothalamus.

The pea-sized pituitary is sometimes called the “master gland” because it helps control the action of other glands throughout the body. Glands are organs that produce chemicals called hormones which regulate bodily functions.

The pituitary works in conjunction with a part of the brain called the hypothalamus, to which the pituitary is directly connected. The hypothalamus produces several different hormones and passes them on to the pituitary, where they’re stored, released, or used to control the function of other glands. Together, the hypothalamus and pituitary monitor and regulate some of the body’s most basic and essential systems.

Some of the functions controlled or impacted by the hypothalamus and pituitary include:

• Growth
• Reproduction
• Metabolism
• Regulation of water and sodium in the body
• Blood pressure
• Labor, childbirth, and lactation
• Stress responses

Craniopharyngioma Research

Title: A Phase II Trial of Intensity-Modulated Proton Therapy for Incompletely Resected Craniopharyngioma and Observation for Craniopharyngioma After Radical Resection

Stage: Recruiting

Principal investigator: Thomas E. Merchant, DO, PhD

St. Jude Children’s Research Hospital

Memphis, TN

Craniopharyngioma is a rare brain tumor that affects both children and adults. It arises in a region of the brain near the pituitary gland, visual pathways, and central blood vessels. Patients often present with headaches, loss of vision, or delayed growth. In some instances, they may present with an imbalance of water and salts in the body.

The treatment for craniopharyngioma may be radical surgery or a combination of surgery and radiation therapy. In some instances, surgery is not required. However, if the tumor cannot be completely removed, radiation therapy may be necessary. In this study, researchers will use the most advanced form of proton therapy called intensity-modulated proton therapy. This is a newer form of radiation therapy having numerous advantages over older forms of proton therapy and conventional radiation therapy using x-rays.

The main goal of this study is to learn if proton therapy will effectively treat patients with craniopharyngioma brain tumors and reduce side effects compared to more traditional forms of radiation therapy.

Title: MEKTOVI® for the Treatment of Pediatric Adamantinomatous Craniopharyngioma

Stage: Recruiting

Study Chair: Kathleen H. Dorris

Children’s Hospital Colorado

Aurora, CO  

Adamantinomatous Craniopharyngioma (ACP) is a highly debilitating pediatric brain tumor lacking medical anti-tumor therapies. Current therapy, which depends mainly on surgery and radiation, is associated with poor quality of life and becomes more challenging and risky in the setting of recurrent disease. However, recent discoveries regarding the biological characteristics of ACP indicate that available agents, including Mitogen-activated protein kinase/extracellular signal-regulated kinase (MAPK/ERK) pathway inhibitors, may have efficacy in the control of ACP. Binimetinib is one such agent.

In this study, up to 38 patients will receive oral binimetinib at the recommended phase 2 pediatric dose (RP2D) of 32 mg/m2 PO every 12 hours for four weeks, representing one cycle. Cycles will last 28 days, and treatment may continue for up to two years (26 cycles).

It will be a multi-center Phase 2 trial with two strata for patients aged >1 year and <25 years with measurable ACP who may have been previously treated with radiation (Stratum 1, 18 patients) or without radiation (Stratum 2, 18 patients).

Title: Memantine for Prevention of Cognitive Late Effects in Pediatric Patients Receiving Cranial Radiation Therapy for Localized Brain Tumors

Stage: Recruiting

Principal investigator: Heather M. Conklin, PhD

St. Jude Children’s Research Hospital

Memphis, TN

Children with brain tumors who have had radiation therapy are at risk for problems with attention, memory, and problem-solving. Such issues may cause difficulty in school and daily life. Memantine, the drug being used for this study, is not yet approved for use in children by the U.S. Food and Drug Administration. However, studies have shown some improvements in memory for patients with dementia, Attention Deficit Hyperactivity Disorder, and autism. Scientists have also used this medication for adult cancer patients receiving radiation therapy, with results showing less cognitive decline over time compared to patients taking a placebo (inactive pill). These studies have also shown few side effects.

This pilot/feasibility study is the first known study involving children with a cancer diagnosis or brain tumor.

PRIMARY OBJECTIVES:

To estimate the participation rate in a study of memantine used as a neuro-protective agent in children undergoing radiotherapy for localized brain tumors (low-grade glioma, craniopharyngioma, ependymoma, or germ cell tumor)

To estimate the rate of memantine medication adherence

To estimate the rate of completion of cognitive assessments

SECONDARY OBJECTIVES:

To estimate the effect in the size of change in neurobehavioral outcomes (cognitive, social, quality of life, neurologic) associated with memantine

To evaluate the frequency and nature of memantine side effects as measured by the Systematic Assessment for Treatment Emergent Events (SAFTEE)