Craniopharyngioma

What is a craniopharyngioma?

Craniopharyngioma is a rare brain tumor found on or near the brain.

This solid tumor may also contain cysts, which are fluid-filled spaces. The low-grade tumor grows slowly but can cause serious illness. 

These tumors often form very close to important parts of the brain, such as the optic pathway, which controls vision. Craniopharyngiomas can also affect hormone levels since they form near the hypothalamus and the pituitary gland. The tumors do not usually spread to other areas of the brain or spinal cord.

How common is craniopharyngioma?

• This tumor accounts for about 5% of all childhood brain tumors.
• About 150 cases occur each year in the United States in children under age 18.
• Diagnosis most often occurs between ages 5 and 14.
• Boys and girls are equally likely to develop this tumor

Craniopharyngiomas can be divided into 2 main types:

1. Adamantinomatous craniopharyngioma is most common in children. These tumors are unevenly shaped. They usually cause cysts and areas of calcium buildup. A change in the CTNNB1 gene in tumor cells is often found.
2. Papillary craniopharyngioma is most common in adults and very rare in children. These tumors usually solid. A change in the BRAF gene in tumor cells is often found.

What are the symptoms of craniopharyngioma?

The symptoms vary widely according to size and location:

• Blindness or partial vision loss—may occur if the optic nerves are affected.
• Slowed growth—may occur if the hypothalamus or pituitary gland is affected.
• Headache—may occur if the tumor presses on the brain or blocks the flow of cerebrospinal fluid from the ventricles.

Other symptoms include:

• Confusion
• Nausea and vomiting
• Delayed puberty
• Extreme thirst and urination (diabetes insipidus)
• Tiredness
• Low blood pressure
• Problems processing minerals (electrolytes)
• Loss of appetite
• Weight gain
• Problems with thinking and learning

How is craniopharyngioma treated?

Craniopharyngioma treatment can include:

• Surgery - is based on the size and location of the tumor as well as its growth into nearby tissues.
  • Transphenoidal surgery - The tumor is removed via the sphenoid sinus. This area is found at the base of skull, at the rear of the nasal passages.
  • Craniotomy - The tumor is removed through an opening made in the skull.
  • Cyst drainage - The surgeon may place a catheter (a tube) into the cyst. This is done by removing part of the bone from the skull (called an open craniotomy) or by drilling a hole in the bone and using image guidance. Sometimes a permanent catheter is placed to drain fluid. This tube is connected to a reservoir under the scalp.
  • Limited surgery - This surgery is done to alleviate symptoms or obtain a diagnosis rather than to fully remove the tumor. Limited surgery is usually followed by radiation therapy.
• Radiation therapy - uses high-energy X-rays or protons to kill cancer cells or stop them from growing. Radiation therapy is used to kill any tumor that remains after surgery.
  • External radiation uses machines outside the body to deliver the X-ray dose.
  • Internal radiation uses liquid sources placed into the parts of the tumor that are cysts.
  • Proton therapy - Proton therapy delivers high radiation doses directly to tumors. This treatment may reduce the side effects of traditional X-ray therapy. When surgeons cannot remove all of the tumor, a child may receive limited surgery and proton therapy.
• Chemotherapy - uses powerful anticancer medicines to kill or stop the growth of the noncancerous craniopharyngioma cells:
  • Intracavitary - places the medicine directly into the cyst cavity
    
    • Intravavitary interferon therapy - puts interferon alpha (IFN-alpha) into the cyst of the tumor

What are the survival rates for craniopharyngioma?

In the United States, the craniopharyngioma survival rate 10 years after treatment is 80%. Long-term issues may occur. These may include the need for hormone replacement or problems related to brain damage.

Why choose St. Jude for your child’s craniopharyngioma treatment?

• St. Jude has cared for more children with craniopharyngioma than any other hospital in the U.S.
• St. Jude has a clinical trial using proton therapy for craniopharyngioma known as RT3CR.
• St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center devoted solely to children.
• St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
• The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
• St. Jude is currently building the world’s only proton therapy center dedicated solely to the treatment of children. Proton therapy can deliver high radiation doses directly to tumors. This treatment spares normal tissues and reduces the side effects of traditional X-ray therapy. Because it is so precise, proton therapy reduces a child’s risk of having serious side effects and of developing other cancers later in life.
• The hospital has one of the largest pediatric brain tumor programs in the United States.
• The St. Jude Brain Tumor Program offers a closely knit group of doctors, scientists, nurses and support staff providing the many types of treatment and support services crucial to the complete care of children with brain tumors.
• The Brain Tumor Program is fully supported by other St. Jude clinical partners in Oncology, Radiation Oncology, Neurology, Pathology, Nursing, Psychology and Rehabilitation Services.
• In addition to the clinical trials developed at St. Jude, the hospital uses protocols developed by the Children's Oncology Group (COG) and the Pediatric Brain Tumor Consortium (PBTC). Many St. Jude faculty members help develop clinical trials, which occur at centers across the United States.