Craniopharyngioma

1 What is Craniopharyngioma?

Craniopharyngioma is a rare noncancerous (benign) tumor that develops near pituitary gland, an endocrine gland that controls other hormonal glands.

With continued growth of craniopharyngioma, function of pituitary gland as well as that of neighboring structure is altered.

Though carniopharyngioma can develop at any age, it mostly affects young children and older adults.

2 Symptoms

Symptoms of craniopharyngioma occur due to:

  • Increased pressure within the brain, usually from accumulation of fluid inside your skull (hydrocephalus)
  • Improper functioning of the pituitary gland
  • Compression of optic nerve or damage to the optic nerve that disturbs vision

Signs and symptoms of craniopharyngioma include:

  • Headache, nausea, vomiting, and balance problems due to hydrocephalus
  • Increased thirst and urination, impaired growth due to hormonal imbalances.
  • Permanent impaired vision due to optic nerve damage. The condition may worsen after surgical removal of tumor.
  • Behavioral changes and learning difficulties.

During diagnosis, most people with craniopharyngioma show impaired vision and reduced hormone level.

When to see a doctor?

Visit your doctor if you experience:

  • Headache, nausea, vomiting, or loss of balance (indications of increased pressure inside the skull)
  • Excessive thirst and urination
  • Retarded growth in a child
  • Vision problems

3 Causes

It is not clear what causes a craniopharyngioma. 

4 Making a Diagnosis

Following tests are performed for diagnosis of craniopharyngioma:

Physical exam and medical history review

Your doctor will have a thorough review of your medical history and conduct a neurologic examination that includes testing your

  • vision,
  • hearing ability,
  • balance,
  • coordination
  • reflexes.

Blood tests

Blood tests are performed to check if there are any hormonal changes. Changes in hormone level can signal improper pituitary functions caused by the tumor.

Imaging tests

Imaging tests, such as

  • X-rays,
  • magnetic resonance imaging (MRI),
  • a computerized tomography (CT) scan
  • a positron emission tomography (PET) scan,

can be performed to obtain images of your brain.

5 Treatment

Several treatment methods exist for craniopharyngioma, which include:

Surgery

Your doctor usually removes all or part of the tumor if you have craniopharyngioma. The type of surgery opted is based on the location and size of your tumor.

Your surgeon may opt:

Open surgery

The tumor is removed by opening the skull. This process is called craniotomy.

Minimally invasive surgery

This includes reaching the tumor through your nose or small cut above your upper lip. Then special surgical instruments can be used to remove the tumor. This is called transsphenoidal surgery as it involves your sphenoid bone.

Surgeons usually opt for removal of whole tumor which may not be always possible as the surgery can affect nearby delicate and important structures.

In such cases, other treatments may be recommended after surgery.

Radiosurgery

Also called stereotactic radiosurgery, this procedure delivers a high dose of radiation to specific areas. It is opted when entire tumor removal is not possible.

Radiation therapy

High energy beams, such as X-rays, are used to destroy tumors. Craniopharyngioma can be effectively treated by advanced radiation technology, such as intensity-modulated radiation therapy (IMRT) and intraoperative radiation therapy (IORT).

These advanced treatments also minimize radiation exposure to normal tissues, such as

  • the eyes,
  • optic nerves,
  • brain,
  • brainstem
  • spinal cord.

Complications

The complications of craniopharyngioma treatment include:

  • Hormonal imbalance
  • Vision problems
  • Nervous system problems

6 Risks and Complications

There is a risk of craniopharyngioma to develop again if entire tumor is not removed. 

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