CRANIOPHARYNGIOMA IS A SLOW growing usually benign, grade I brain tumor. Located in the central region of the brain, these cystic tumors form near the pituitary gland, and as it grows, it can disrupt the surrounding structures including vision and the level of hormone secreted from the pituitary gland. A change in hormone levels leads to a variety of symptoms so it is best to consult with a doctor if you have any unexplained changes that you are experiencing. If you have been diagnosed with a brain tumor or craniopharyngioma or are concerned about the symptoms you or a loved one is experiencing, consider contacting Centers for Neurosurgery, Spine & Orthopedics in New Jersey for a thorough evaluation.
There are two subtypes of craniopharyngioma:
- Adamantinomatous most commonly occurs within the first two decades of life
- Papillary is predominantly seen in older adults.
Some studies have reported a lower recurrence rate of the papillary subtype, while others have found no significant differences in outcome or recurrence between both subtypes.
There are many different clinical manifestations of a craniopharyngioma. Depending on where within the stellar region of the brain it originates and then its growth rate and size, a variety of symptom may begin to occur. The most common symptoms experienced by children and adults include:
- Confusion, mood swings, or other behavioral changes
- Loss of balance
- Increased thirst or urination
- Impaired peripheral vision (bitemporal hemianopia)
- Horizontal double vision
Due to the proximity to the pituitary gland, craniopharyngioma may disturb hormones vital to childhood development. These disturbances may present themselves as growth failure and delayed puberty in pediatric cases. In adults, craniopharyngioma may cause hypogonadism, which may lead to sexual dysfunction, irregular menstrual cycles in women, and infertility. Thyroid-stimulating hormone deficit may also occur, which can cause weight gain, fatigue, cold intolerance, and constipation.
Another pituitary hormone that may be affected by the growth of a craniopharyngioma is ACTH. Changes in the levels of ACTH result in reflective changes in cortisol production and release from the adrenal glands. A decrease in ACTH will lead to a lack of blood pressure responsiveness to positional changes known as orthostatic hypotension. Symptoms of dizziness, light-headedness, and syncope can be expected when moving quickly from a sitting or supine position to a standing position. Decreases in ACTH can also cause, high potassium (hyperkalemia), irregular heartbeats (cardiac arrhythmias), lethargy, and weight loss.
Causes & Risk Factors
While the exact cause of craniopharyngioma is not yet fully known, its development is best understood by the following two theories:
- Embryo-genetic: Pertains to the adamantinomatous subtype, which most commonly occurs during childhood, adolescence, or young adulthood. In this instance, it is believed that cell remnants from a particular stage in embryonic development continue to proliferate in the craniopharyngeal duct thus develop into a tumor.
- Metaplastic: This theory relates to the papillary subtype of craniopharyngiomas, which is more prevalent among older adults. In this case, adenohypophyseal cells in the pituitary gland or stalk undergo abnormal changes (metaplasia). These changes then multiply and become papillary craniopharyngioma.
Other risk factors for a craniopharyngioma include:
- Age: Craniopharyngioma is most commonly diagnosed in children between the ages of 1 to 14 years old, and adults between the ages of 50 to 74 years old.
- Family history and genetics: A family history of craniopharyngioma is a small, considerable risk factor. Particular genetic mutations (CTNNB1 or APC genes) are present in over 70 percent of these tumors, too. CTNNB1 and APC produce the protein beta-catenin (β-catenin), which plays a role in embryo development. Thus, mutations in these genes may contribute to adamantinomatous craniopharyngioma.
The symptoms of craniopharyngioma are broad and highly variable. Furthermore, other neurological and non-neurological health conditions can cause very similar symptoms. Therefore, it’s important to be as descriptive and specific as possible when consulting with a doctor about symptoms. If there is an index of suspicion that a brain tumor may have developed, the doctor or neurosurgeon will order a CAT scan or an MRI.
The diagnostic process for craniopharyngioma utilizes both pre-contrast and post-contrast CAT scans and MRIs. Cerebral angiography may also be performed, along with complete endocrinological, neuro-ophthalmologic, and neuropsychological evaluations in order to determine the exact location and origin of the brain tumor so it can be correctly classified. Only thereafter can the most effective treatment be determined.
- CAT & MRI Scans: A CAT (computerized tomography) scan uses rotating x-ray machines and computers to take cross-sectional images inside the body. These images will enable the physician to evaluate the bony anatomy and possible calcifications of the tumor. Likewise, an MRI (magnetic resonance imaging) scan uses strong magnets and radio waves to create more detailed images of the tissues and organs inside of the body. MRI scans are particularly useful for analyzing the structure of the tumor. Both of these diagnostic tools are common and painless. MRI scans are often preferred when evaluating the location and impact of a tumor on the surrounding tissue and organs.
- Cerebral Angiography: Cerebral angiography is a procedure that uses contrast dye and x-rays to view the blood vessels in the brain. This diagnostic tool allows the specialist to monitor how blood flows through the brain, which can detect or rule out any potential abnormalities including tumors within the blood vessels or new blood vessels forming within a tumor.
- Endocrine Evaluations: Blood work and urine will help verify whether the craniopharyngioma has disrupted the function of the pituitary gland. An endocrinologist is a medical doctor with expertise is the study of hormones. An endocrinologist will evaluate e the results of blood tests which check your levels of various hormones such as thyroid-stimulating hormone, cortisol levels, ACTH, growth hormone, luteinizing hormone, and follicle-stimulating hormone. Based on the endocrinologist’s analysis, the impact of the brain tumor can be further specified. Treatment can bridge the consequences of abnormal hormones from before the tumor is removed through the perioperative period and thereafter if necessary.
- Neuro-ophthalmology Evaluations: A neuro-ophthalmologist is a specialist who evaluates and treats visual defects that originate in the brain as opposed to within the eye. If the patient has been experiencing a loss in visual fields, a neuro-ophthalmologist will conduct a full eye exam, including a visual field test. There will also be a corresponding neurological examination to test strength, sensory perceptions, and coordination. Thereafter, the neuro-ophthalmologist will determine how severely the craniopharyngioma has impacted vision or whether the visual disturbances have a different origin.
- Neuropsychology Evaluations: A neuropsychologist is a physician who specializes in how the neural network of the brain impacts mental performance and behavior. A neuropsychological evaluation can determine if there are defects in mental performance or concerning behavior patterns. During an appointment, the physician will evaluate an array of cognitive abilities, including visual reasoning, verbal reasoning, language, memory, fine motor, and sensory-motor skills. The neuropsychologist will also assess attention span, executive functioning, reading, writing, math, social perception, emotional functioning, and adaptive functioning. These tests will determine how severely brain function as it relates to performance and behavior has been impacted because of the craniopharyngioma as well as providing a baseline for cognitive brain function throughout other areas of the brain.
After interpreting the results of a patient’s laboratory tests and imaging, removal of the craniopharyngioma by a neurosurgeon is the primary treatment. The goal of the neurosurgeon is to remove the brain tumor entirely. If the tumor is adherent to critical brain structures, the neurosurgeon will remove the bulk of the tumor then use other techniques to remove the now smaller remaining portions adjacent to the critical structures. The smaller remaining portions of the tumor can safely be more safely removed with less exposure via radiation therapy.
Radiotherapy is only administered when it’s necessary to the success of the surgery. It may be determined best to have a radiation oncologist administer radiotherapy prior to surgery in order to shrink the tumor.
Depending on whether the pituitary gland or hypothalamus has been affected by the craniopharyngioma, the surgery, or radiation, an endocrinologist will provide the necessary hormone treatment or replacement therapy.
Three months after the surgical procedure, your doctor will order a postoperative MRI to verify the tumor is gone. Postoperatively, there will need to be a regular check-up MRI as per treatment guidelines given the national incidence of recurrence is 10% within five years of surgery. Over 10 years after treatment, the incidence of recurrence is 19%.
Let CNSO’s New Jersey Team Help
Craniopharyngioma is just one of the many types of brain tumors treated by the neurosurgical team at Centers for Neurosurgery, Spine, and Orthopedics in six locations throughout Northern New Jersey. Each office in Paramus, Jersey City, Wayne, Morristown, and Clifton has CNSO surgeons each with over 10 years of experience, and routinely using the most advanced minimally invasive endoscopic surgical techniques to remove brain tumors such as the transsphenoidal approach plus comprehensive care as necessary in partnership with endocrinologists, radiation oncologists, and pain management physicians experts. Contact Us today to schedule your appointment.