Overview of Craniopharyngioma

A craniopharyngioma refers to the formation of a lump near or above the pituitary gland and at the base of the brain near the optic nerves that connect the eyes to the brain. In that way, it can lead to a variety of hormone and neurologic problems. To treat them, people with craniopharyngioma require surgical and medical expertise.

Additionally, it is more likely to develop in children. If it is caused due to hormonal problems, then its treatment requires expert management by doctors of a different specialty of medicine.

Prevalence of Craniopharyngioma

Globally, craniopharyngioma cases were reported in about 2-5% of all primary brain tumors, and 5-10% of all childhood brain tumors. This tumor is mainly developed in two age groups; one is a group of age up to 14 and the other over age 45. Overall, the Craniopharyngioma recurrence rate is very high, with almost 50% of cases reported with this cancer. It also has high survival rates (83% to 96% five-year survival and 65% to 100% 10-year survival) in the general world's population. 

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Signs and Symptoms of Craniopharyngioma

A growing craniopharyngioma exerts pressure on the nerves, blood vessels, or brain tissue near the pituitary gland. 

If this tumor affects the brain resulting in symptoms such as: 

  • Nausea and vomiting
  • Headaches
  • Balance problems or difficulty in walking

If the craniopharyngioma tumor interferes with the functions of the optic nerves, resulting in the following symptoms:

  • Vision problems such as double vision or narrowed field of vision

If the craniopharyngioma tumor is exerting pressure on the pituitary gland, then symptoms may include:

  • Slowed growth
  • Excessive thirst 
  • Severe urination problem
  • Delayed puberty
  • Low energy, or unusual sleepiness
  • Being overweight
  • Personality changes
  • Changes in behavior
  • Intolerance of cold temperatures

Types of Craniopharyngioma

There are two histological types of craniopharyngioma that differentiate from each other in terms of different characteristics of cells.

  • Adamantinomatous Craniopharyngioma

Adamantinomatous craniopharyngioma forms deep sores on the epithelium of the brain known as “picket fence.”

  • Papillary Craniopharyngioma

On the other hand, papillary craniopharyngioma immature nodules appear on the surface of the epithelium. Its nodules are not well organized and mature as  Adamantinomatous craniopharyngioma.

Apart from this, Adamantinomatous (ordinary) craniopharyngioma develops in children and is less solid than papillary craniopharyngioma. Papillary craniopharyngioma affects adults and has a more solid tumor,

Causes of Craniopharyngioma

There is no clear cause of craniopharyngiomas. For the development of this tumor, it consists of the two following major theories:

  • The embryonic theory 
  • The metaplastic theory

These two theories are related to the subtypes of craniopharyngioma which are the adamantinomatous craniopharyngiomas and the papillary craniopharyngiomas.

The embryonic theory correlates to the formation of adamantinomatous craniopharyngiomas, which is the most common subtype of craniopharyngioma in the children's age group. During embryogenesis, the embryonic cells of the extended cricopharyngeal duct (due to the outpouching of the ectodermal roof), begin to proliferate over there. This leads to the cause of craniopharyngioma.

The metaplastic theory is related to the development of papillary craniopharyngiomas, which is the most common subtype of craniopharyngioma in adult patients. In this theory, the adenohypophyseal cells of the pars tuberalis can cause metaplasia that results in the formation of squamous cell nests. These nests begin to proliferate and lead to a papillary craniopharyngioma.


Risk Factors of Craniopharyngioma

As already discussed there is no known cause of craniopharyngioma. Similarly, we don’t have information regarding any known risk factors of this disease.



Till now we don't know about any way to prevent craniopharyngioma.



Following tests and procedures are performed to diagnose craniopharyngioma. This may include:

  • Physical Exam- Initially, diagnosis usually starts with a medical history and neurological exam for diagnosing the craniopharyngioma. During this procedure, vision, hearing, balance, coordination, reflexes, and growth and development are being tested.

  • Blood Tests- Blood tests may identify any changes in hormone levels that indicate a tumor affects the pituitary gland.

  • Imaging Tests-These tests are used to create scans of the brain at various sides with the help of different techniques including, X-rays, magnetic resonance imaging (MRI), and computerized tomography (CT).

Treatment of Craniopharyngioma | When to Consult a Doctor

There are any one of the following craniopharyngioma treatment options.

  1. Surgery

 It is the surgery to remove all or most of the tumor often recommended for people with craniopharyngioma. The type of surgery performed for the patients depends on the location and size of the tumor.

Firstly the surgery is open craniopharyngioma (craniotomy) in which a doctor opens the skull and removes the tumor through surgical instruments. Another minimally invasive technique of craniopharyngioma surgery is the transsphenoidal procedure. In this method, special surgical tools are inserted through the nose into the tumor. The tumor is removed without damaging the brain.

It is difficult to remove the entire tumor but in some cases, surgeons try to remove it all. Because of the many delicate and important structures nearby, sometimes doctors cannot remove the entire tumor for saving the patient’s life. In these situations, other treatments are used after surgery.

  1. Radiation Therapy

External beam radiation therapy is used after surgery for the treatment of remaining craniopharyngioma. This treatment passes powerful energy beams, such as X-rays and protons, through the affected area to kill tumor cells. 

In proton beam therapy and intensity-modulated radiation therapy (IMRT), doctors target the affected area through radiation carefully to protect the nearby healthy tissue cells. Specialized expert doctors are required. This technique is also known as external beam radiation technology.

Another type of radiation therapy called stereotactic radiosurgery may be recommended in rare cases. It is required when the tumor doesn't grow to the bundle of nerve fibers that transmits visual signals from the eye to the brain (optic nerve). The multiple beam radiations target the precise points of the affected area to kill the tumor cells.

Another type of craniopharyngioma radiation therapy is called brachytherapy in which radioactive material is directly placed into the tumor, where it can treat the tumor from the inside.

  1. Chemotherapy

 Chemotherapy is a drug treatment in which specific medications are used to kill the tumor cells. In this method, chemotherapeutic drugs can be injected directly into the tumor which kills the targeted cells and mostly prevents damage to nearby healthy tissue.

  1. Targeted Therapy for Papillary Craniopharyngioma

This is specifically used for the treatment of papillary craniopharyngioma. Targeted therapy involves drugs for the treatment of specific abnormalities within the tumor cells. Majorly the mutation occurs in BRAF genes of papillary craniopharyngioma cells. Targeted therapy targets this mutated gene for treatment. Specialized laboratory testing can identify the mutated BRAF gene in papillary cells.

  1. Clinical Trials

 Researchers are experimenting with new treatment strategies that are based on the existing standard treatments. For exploring advanced techniques, clinical trials are conducted for testing the patients. But always consult the doctor regarding the eligibility of clinical trials, in some cases, these trials are being tested.

  1. Follow-up Care

After completion of the treatment, lifelong treatment or follow-up is generally needed.

  • If the tumor has been removed completely, follow-up tests and imaging tests are needed so that the tumor will not recur.
  • The patients whose pituitary gland function is damaged by craniopharyngioma tumors then some medications are used for the replacement of hormonal production.

If individuals are feeling pressure on the brain, nerves, and blood vessels or feel frequent headaches then consult an oncologist immediately.

Craniopharyngioma Prognosis

Craniopharyngiomas are more likely to develop in children and adults. For the removal or inhibition of the tumor growth. Ongoing surveillance and lifelong monitoring are needed in children whose treatment becomes successful.