Craniopharyngioma

What is a craniopharyngioma?

Craniopharyngioma is a tumor found on or near the brain.

These tumors are usually part solid and part fluid-filled. They may grow and press against parts of the brain; optic nerves; and fluid-filled spaces around the brain, including the hypothalamus and pituitary gland.

How common is craniopharyngioma?

  • Childhood craniopharyngioma is rare, occurring in only two or three children per million.
  • There are approximately 100 cases per year in the United States in children under the age of 18 years.
  • Diagnosis most often occurs between ages 5 and 14.
  • Boys and girls are equally likely to develop craniopharyngioma.

What are the symptoms of craniopharyngioma?

The symptoms of craniopharyngioma 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 compresses the brain or blocks the normal flow of cerebrospinal fluid from the ventricles.

Other symptoms of craniopharyngioma include:

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

How is craniopharyngioma treated?

Craniopharyngioma treatment includes several options:

  • Surgery - is based on the size and location of the tumor as well as its growth into nearby tissues:
    • Transphenoidal surgery - The surgeon accesses the tumor by creating a corridor through the sphenoid sinus, which is part of the base of skull located at the rear of the nasal passages.
    • Craniotomy - The surgeon removes the tumor through an opening made in the skull.
    • Cyst drainage - The surgeon may place a catheter (a permanent or temporary tube) into the cyst 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 repeatedly drain the fluid. It will be connected to a reservoir that will lie under the scalp.
    • Limited surgery - Any surgery procedure which 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 remaining 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 are unable to 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:
    • Systemic - taken by mouth or injected, the medicine enters the bloodstream and moves throughout the body
    • Intracavitary - used to place the medicine directly into the cyst cavity

What are the survival rates for craniopharyngioma?

In the United States, the craniopharyngioma survival rate is 80% when measured 10 years after treatment. Long-term complications, such as the need for hormone replacement and problems related to brain damage, may occur.

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 RT2CR.
  • 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 play key roles in developing clinical trials, which are conducted at centers across the United States.

Contact the Brain Tumor Team any time, 24 hours a day, 7 days a week.

Call our Brain Tumor Coordinator, Tabatha E. Doyle, RN:
(901) 595-2544 or
(901) 595-4599

Or email us at braintumors@stjude.org

Associated Clinical Trials

PBTC39: Peginterferon Alfa-2b in Treating Younger Patients With Craniopharyngioma That is Recurrent or Cannot Be Removed By Surgery

Phase II Study of Peginterferon alfa-2b (PEGIntron) for Pediatric Patients with Unresectable or Recurrent Craniopharyngioma (PBTC-039)

Diseases Treated:

Unresectable or recurrent craniopharyngioma

Eligibility:

  • Participant has a histologically verified diagnosis of craniopharyngioma.
  • Participant has recovered from acute toxicities of all prior therapy before entering this study.
  • Participant has not received prior interferon, either systemic or into the tumor cyst.
  • Participant does not have evidence of metastatic tumor.
View Trial

RT2CR: Limited Surgery and Proton Beam Radiation Therapy in Treating Younger Patients with Brain Tumors

A Phase II Trial of Limited Surgery and Proton Therapy for Craniopharyngioma and Observation for Craniopharyngioma after Radical Resection

Diseases Treated:

Craniopharyngioma

Eligibility:

  • 21 years of age or younger
  • Diagnosis of craniopharyngioma 
  • Has not been previously treated with radiation therapy
View Trial

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