Can Craniopharyngioma Be Cured? Survival Rates

When diagnosed early and managed properly, the 5-year survival rate for children under age 15 with craniopharyngioma is over 95%.

Craniopharyngioma is considered a chronic disease by many experts, however, because of the high tumor recurrence rates even with apparent complete resection of the tumor.

Craniopharyngioma usually forms at the base of the brain close to the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands) and does not spread to other areas.

Craniopharyngioma is also known as:

• Rathke’s pouch tumor
• Craniopharyngeal duct tumor

Craniopharyngiomas are typically partly solid mass and partly fluid-filled cyst that can grow and produce pressure on the nearby organs, including the:

• Pituitary gland
• Optic tracts or chiasm (contain the nerve fibers responsible for vision)
• Third ventricle of the brain

• Adamantinomas: Most commonly occur in children and arise from cells from an embryologic structure called craniopharyngeal duct
• Papillary: More common in adults and arise from cells from the anterior part of the pituitary gland

Although the exact cause of craniopharyngioma is unknown, experts speculate that the tumor grows from leftover pieces of tissue that fail to disappear as expected in early pregnancy when the fetal head, face, and brain are forming.

Though it can affect people of any age, craniopharyngioma most commonly affects children between 5-10 years of age and adults between 50-74 years. All genders are equally likely to develop this type of tumor.

There are no known risk factors for childhood craniopharyngioma.

Craniopharyngioma causes symptoms by:

• Compressing the foramen of Monro within the ventricles, which accumulate the cerebrospinal fluid, resulting in increased pressure on the brain (hydrocephalus)
• Disrupting hormone production by the pituitary gland
• Pressuring or damaging the optic nerve

Increased pressure on the brain can cause:

• Headaches, especially in the mornings
• Nausea
• Vomiting
• Vision changes

Other symptoms may include:

• Hearing problems
• Trouble seeing things to the far left and right
• Poor balance or trouble walking
• Increased thirst
• Increased frequency of urination
• Delayed puberty
• Weight gain
• Slow or retarded growth (short stature)
• Delayed menarche
• Excessive tiredness
• Low blood pressure
• Behavioral and learning problems

After analyzing your medical history and current symptoms, the doctor will perform a physical examination and recommend certain tests, which may include:

• Blood tests to measure hormone levels
• Imaging tests, such as computed tomography or magnetic resonance imaging scans of the brain
• Neurological examination to check mental status, coordination, ability to walk normally, as well as the muscles, senses, and reflexes
• Visual field examination to check the field of vision (total area in which objects can be seen) which includes both central vision and peripheral vision

Additional tests may include:

• Lumbar puncture (spinal tap): A needle is inserted into the back to withdraw a small amount of cerebrospinal fluid for lab analysis
• Biopsy of a tissue sample:
  • Open biopsy: A hollow needle is inserted into a hole in the skull
  • Computed tomography (CT)-guided needle biopsy: A CT-guided hollow needle is inserted through a small hole in the skull and into the brain
  • Transsphenoidal biopsy: Instruments are inserted through the nose and sphenoid bone (a butterfly-shaped bone at the base of the skull)

Surgery is typically the primary treatment for craniopharyngioma. However, for some people, radiation therapy may be the better treatment option.

Depending on the size and location of the tumor, it may be removed through a hole in the skull or the nose.

• Surgery: Removal of the entire or most of the tumor is recommended
  • Open craniopharyngioma surgery (craniotomy): Involves opening the skull to gain access to the tumor
  • Transsphenoidal procedure: Minimally invasive craniopharyngioma surgery that uses specialized surgical tools inserted through the nose
• Radiation therapy: Uses powerful energy beams, such as X-rays and protons, to kill tumor cells
  • Specialized external beam radiation technology, such as proton beam and intensity-modulated radiation therapy (IMRT), allows the radiation beam to target the tumor cells exclusively, sparing nearby healthy tissue
  • Stereotactic radiosurgery may be recommended in cases where the tumor is not affecting the optic nerve and the bundle of nerve fibers that transmit visual information from the eyes to the brain
• Chemotherapy: Drug treatment that uses chemicals to kill tumor cells
• Targeted therapy: Drug treatment that primarily focuses on abnormalities within the tumor cells that allows them to survive
• Immunotherapy: Uses the patient’s immune system to fight cancer cells
• Cyst drainage: Involves a surgical procedure to drain tumors that are mostly fluid-filled cysts through a catheter (thin tube)

Craniopharyngioma and its treatment can cause physical symptoms and side effects which can be managed with palliative care (including medication, dietary changes, relaxation techniques, emotional and spiritual support, and other therapies) and supportive care.

Side effects may include:

• Physical problems
  • Seizures
  • Bone and muscle growth and development
• Behavior problems
• Obesity
• Changes in mood, feelings, thinking, learning, or memory
• Secondary cancers

Complications may include:

• Relapse of tumor
• Hormonal issues
• Vision loss
• Nervous system problems
• Metabolic syndrome, including fatty liver disease
• Blood vessel problems or stroke
• In rare cases, craniopharyngiomas can become malignant