Craniopharyngioma is a type of tumor that comprises 9% of all pediatric brain tumors and usually occur in children between 5 and 10 years of age. They are very slow growing tumors. They arise from the cells along the pituitary stalk.
They are also known as Rathke pouch tumors, hypophyseal duct tumors, or adamantinomas.
Survival rates for brain tumor are about 50%. Most of those who die as the result of the effects of a brain tumor are either very old or are young children with ages ranging from 2 months to 4 years. The majority of those who die are believed to have a metastatic brain tumor that originates from cancer cells imported from other parts of the body, or the deadliest type of brain tumor known as a glioblastoma multiforme (primary malignant brain tumor.)
Brain tumors run a spectrum from those which grow slowly and behave in a benign fashion, and those which grow quickly and behave in a malignant fashion. Astrocytoma is the most common tumor arising from brain tissue. A grade I astrocytoma grows slowly, whereas a grade IV astrocytoma (glioblastoma) grows quickly.
The symptoms of a brain tumor do not begin until months after the tumor starts to grow into a large enough size to put pressure on the vital brain areas. The symptoms of a brain tumor depend on the location with in the size of the tumor. Since different brain functions are controlled by each side of the brain, the location of the tumor may cause more severe symptoms than the size of the tumor. Size still is an important factor, because the skull is made of hard bone, making it unable to accommodate a growing tumor (Brain Disorder source book). Symptoms can also vary depending on how fast the tumor grows. If the tumor grows slowly, then sometimes the brain can adjust to the increased mass and size in the skull. If the tumor grows at a quick rate, the symptoms can appear suddenly. Symptoms of a brain tumor usually start with constant headaches, because of the increased pressure on the brain. Research shows that twenty percent of all people who have brain tumors suffer from headaches as their first symptoms (Neurology Channel page 1 link 1). Most people who suffer from headaches as the first sign of their brain tumor have their tumor in the frontal areas of the skull.
The next stages of symptoms include nausea, vomiting, and change in cognitive status. After the manifestation of these symptoms, patients may seek medical assistance. A doctor may not realize this is a brain tumor, since these are also the symptoms of insomnia and migraine headaches. The doctors may not diagnose a brain tumor immediately, because they will investigate and rule out more common causes of the same symptoms first. (Cicala, Brain Disorder Source Book Page 169).
The next stage in the life of a tumor may cause permanent damage to the brain. The symptoms include seizures of various intensity, depending on the location of the tumor. At this stage, the tumor pressing on the optic nerve would crush that optical nerve, causing visual problems ranging from blurriness in vision to blindness. If medical intervention does not occur, the tumor will continue to grow until it shuts down the motor and sensory abilities of the body, as the result of increased pressure on the part of the brain that controls those functions.
When sensory and motor abilities are damaged because of the increased pressure on the brain, a person can initially experience numbness in the hands and feet, tingling in the arms and legs, and eventually the loss of control of motor functions such as bowel movements and bladder control. Other affected areas can be the five senses of the body, which includes loss of hearing and having earaches, slowed speech, loss of clear vision, inability to feel, and loss of smell and taste.
In the case where the tumor is at the intersection of the optical nerves (the optic chiasm) the patient will most likely develop a loss of the temporal visual fields from both eyes, due to the crossing of nasal optic nerve fibers in that area. This would result in a loss of peripheral vision, known as bitemporal hemianopsia, also described as "tunnel vision". As the tumor grows, the increasing adverse effect on the functioning of the body can be progressively felt by the patient, until the body completely shuts down. This can be avoided by not ignoring the symptoms and seeing a doctor right away when the patient notices a continued pattern of worsening headaches.
Craniopharyngioma not only causes vision problems due to the location of the tumor at the intersection of two optic nerves (optic chiasm), but it also causes hormonal problems due to pressure inside the stem of the pituitary gland where the tumor is located and continues to grow. The pituitary gland controls hormones that regulate various endocrine functions (Cicala, Brain Disorder Source Book pages 173-174).
The cause of occurrence of brain tumors is unknown, but researchers have discovered that people that work with rubber and certain chemicals have a greater than average risk of developing brain tumors. Researchers are now looking for a relationship between brain tumors and viruses, family history, and long-term exposure to electromagnetic fields (Gale Encyclopedia of Medicine Pg 512). Other researchers believe that a primary benign brain tumor starts at the development of the fetus, when a mutation occurs causing abnormal cells to develop inside the cranium. Although not enough evidence is known to prove this theory, some researchers believe this to be a possible cause, since this theory supports the common occurrence of brain tumors in young children.
* Balance disorder
* Dry skin
* Headache (obstructive hydrocephalus)
* Short stature
* Polyuria (diabetes insipidus)
* Vision loss (bitemporal hemianopsia)
* Weight gain
To diagnose a person’s brain tumor and to find out the correct treatment plan for the person with a brain tumor, the patient needs to go through tests known as diagnostics. Diagnostics for brain tumors include magnetic resonance imaging (MRI), computerized axial tomography (CAT scan), and positron emission tomography (PET). The tests listed are neuro-imaging techniques used to evaluate the cause of the problem resulting from the brain tumor (Neurology Channel link 3, page 1). Other tests includes various neurological examinations, including eye movement, reflexes, hearing, balance and coordination, body movement, and various other sensations.
The neuro-imaging tests include MRI, CT scans, and PET scans. The MRI is a test that uses a magnetic field fed into a computer to translate the magnetic electronic images of specific atoms and molecular structures into images of a person’s brain. The MRI scans are clearer than all the other scans, but may not be used as the initial imaging test. In situations when doctors need results immediately, they use CT scans. CT scans take less time to obtain but are less clear images than MRIs. The CT scan is an X-ray machine coupled with a computer translates picture of a person’s body. A PET scan is a way of measuring brain activity by injecting substances into the brain that can be monitored and used to evaluate tumor growth (Neurology Channel link 3, page 1). These scans are used to give a tumor, a grade. The grades are determined by the World Health Organization (WHO). The WHO system clarifies all cancers on a grading level of 1-4. Grades 1-2 are slow growing benign brain tumors, and grades 3-4 are faster growing malignant tumors (Brain tumors guide for the newly diagnosed). Craniopharyngioma will get a grade between 1 and 2 depending on how fast it grows.
Based on a patients diagnosis, using techniques such as PET, doctors can determine what type of a treatment they must put their patients on, including the possible removal of the tumor. The most common and effective way to treat craniopharyngioma is by surgery. Even though surgery is the most effective treatment, it is also the most dangerous. The reason surgery is the most common or popular form of treatment is the quick results and the fact that a lesser percentage of tumors reoccur in the brain as opposed to radiation and chemotherapy.
The treatments are divided into three categories. The categories are surgery, radiation, and chemotherapy. Depending on the diagnostics of the brain tumor the doctor will decide what the patient’s treatment should be. Various treatments can be combined for a stronger effect. If a tumor is very large, radiation and chemotherapy may be used to reduce the size of the brain tumor, in preparation for a follow up surgery.
The first category is surgery and the most common kind is a craniotomy, which is surgical opening through the skull. During this surgery the patient is put under anesthesia. For craniopharyngioma it is impossible to remove the tumor without cutting the stem of the pituitary gland due to the tumor growing inside of the stem. The second type of treatment is radiation therapy. High powered x-rays or gamma rays are aimed at the tumor (Brain Disorder Source Book Page 200). The third type of treatment is chemotherapy which is usually only used in malignant tumors. The malignant tumors containing cancer cells can be treated by chemotherapy, a cancer killing chemical which is taken by mouth or by injection (The Gale Encyclopedia of Medicine Volume 1 A-B pages 514).
The common and standard treatment for craniopharyngioma is surgery. The negative effects of surgery can be higher than radiation and chemotherapy, but it is usually chosen over the other treatments because it has an immediate impact on the body. For craniopharyingioma tumors, having surgery might mean cutting out the whole pituitary gland depending on the size of the tumor, which would cause the patients to take hormone medications for the rest of their lives. After surgery, patient will spend time in the Intensive Care Unit (ICU), until they are stable. After spending time in the ICU the patient will move to a regular hospital room, until they are stable to go onto rehabilitation (rehab) and exercise sensory and motor abilities. After going through rehab, a person may still not return to normal. One thing that might not change after surgery on a craniopharyingioma is vision, which may remain impaired because of the damage sustained by the optical nerves. Other things that may occur after surgery are a change in patient’s personality and memory loss.