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Craniopharyngiomas are brain tumors that develop near the pituitary gland and hypothalamus. They are typically benign and do not spread to other areas of the brain or body. This slow-growing tumor affects cranial nerves responsible for vision and the endocrine system which regulates hormones in the body. Craniopharyngiomas can affect people of any age, but they are more commonly seen in children and older adults. Symptoms include vision changes, fatigue, frequent urination, and headaches. Children with this condition may experience delayed growth and be smaller than expected.
Treatment usually involves a combination of surgery, radiation therapy, and hormone replacement therapy, depending on the size and location of the tumor and the individual patient’s needs. With appropriate treatment, the prognosis for craniopharyngioma is generally favorable. However, long-term monitoring and follow-up care are usually necessary to manage any ongoing effects of the tumor or its treatment.
Symptoms
Symptoms of craniopharyngioma are associated with problems that occur when there is an impact on the pituitary and hypothalamus glands, as well as the optic nerves and brain. In children, the growth and development may be impacted more significantly than in adults. Both children and adults may experience vision problems, especially with their peripheral vision. However, these symptoms can resemble those of other conditions, which can make it challenging for healthcare providers to promptly identify the cause of the symptoms as being this tumor.
Many hormonal processes are controlled by the child’s pituitary. The following conditions may result from a craniopharyngioma’s pressure:
- Growth hormone (GD) deficiency: Children’s growth rates are affected by this condition. Adults with GH insufficiency may experience low energy, heart problems, osteoporosis, weak muscles, and elevated LDL cholesterol.
- Gonadotropin deficiency: Puberty delays are common in individuals with this condition. Amenorrhea, a condition when women do not have periods.
- Adenocorticotropic hormone (ACTH) deficiency: People with this condition experience fatigue and weakness. Other conditions include hypotension (low blood pressure), weight loss, appetite loss, muscle weakness, nausea, and vomiting.
- Thyroid stimulating hormone (TSH) deficiency: People may experience irregular periods, weak feelings of exhaustion, and increased forgetfulness.
- Central diabetes insipidus (CDI): People who have this condition have polydipsia, or severe thirst, and polyuria, or frequent urination. Dehydration, irregular heartbeats, fever, dry skin and mucous membranes, disorientation, and seizures are symptoms that CDI patients may experience.
This tumor pressures the patient’s or child’s hypothalamus, which is associated to some medical disorders. The patient’s or child’s hypothalamus regulates bodily functions like mood, hunger and thirst, sleep cycles, and sexual function. The following issues are triggered by pressure from a craniopharyngioma on a patient’s or child’s hypothalamus:
- Hypothalamic obesity: This type of obesity persists even if individuals modify their diet and exercise routines by increasing their activity level and cutting back on their calorie intake.
- Froelich’s syndrome: Due to their constant hunger, individuals with this illness frequently struggle with obesity. It’s possible that children with Froehlich’s condition do not develop as quickly as normal kids do. Some kids struggle with intellectual problems and poor vision.
- Non-24-hour sleep wake syndrome: People who have this sleep disorder are unable to adhere to a regular sleep schedule. Some individuals have acute sleep deprivation.
Craniopharyngiomas frequently form quite close to the optic nerves of the patient or the child. If such nerves are under stress, visual problems or issues with the periphery may result.
Causes
The cause of these tumors has not been specifically identified by researchers, but they theorize that the tumors originate from cells that played a role in the formation of either your or your child’s pituitary gland. These cells seem to undergo a transformation into abnormal cells that have the ability to multiply and develop into tumors.
Risk factors
Currently, there are no identified factors that have been proven to cause or increase the risk of developing craniopharyngioma.
Diagnosis
Craniopharyngioma is a rare type of brain tumor that develops near the pituitary gland. The diagnosis of craniopharyngioma typically involves a combination of imaging tests, such as MRI and CT scans, and neurological exams. Here are some tests and procedures that may be used to diagnose craniopharyngioma:
- Physical examination: Healthcare provider will often analyze the medical history and perform a neurological examination before diagnosing the patient with a craniopharyngioma. The hearing, balance, coordination, reflexes, growth, and development are all examined during this examination.
- Imaging test:
- Magnetic Resonance Imaging (MRI): This imaging test uses a powerful magnet and radio waves to produce detailed images of the brain. It can help identify the location, size, and shape of the tumor.
- Computed Tomography (CT) Scan: This imaging test uses X-rays to produce cross-sectional images of the brain. It can help identify the location and size of the tumor.
- Hormone Tests: Since craniopharyngioma often affects the pituitary gland, hormone tests may be performed to check for hormonal imbalances.
- Biopsy: In some cases, a biopsy may be performed to confirm the diagnosis of craniopharyngioma. This involves removing a small sample of the tumor tissue and examining it under a microscope.
It is important to note that the specific tests and procedures used to diagnose craniopharyngioma may vary depending on the individual case and the healthcare provider’s preferences.
Treatment
The treatment options for craniopharyngioma may depend on various factors such as the size and location of the tumor, the patient’s age and overall health, and the extent of the tumor’s invasion into surrounding tissue. Some treatment options for craniopharyngioma include:
- Observation: In some cases, especially in elderly patients or those with small, slow-growing tumors, observation or “watchful waiting” may be an option. This involves monitoring the tumor’s growth over time with regular imaging tests and only treating it if it starts to cause symptoms or show signs of progression.
- Surgery: Craniopharyngioma treatment typically involves surgical intervention to remove the majority, if not all, of the tumor. The surgical approach used depends on the size and location of the tumor. Open craniopharyngioma surgery involves opening the skull to access the tumor, while minimally invasive surgery involves inserting specialized tools through the nose to access the tumor without disturbing the brain. When possible, the goal of surgery is complete tumor removal. However, in some cases, the proximity of vital structures makes complete removal challenging. In such instances, surgeons may leave some of the tumor behind to preserve the patient’s quality of life. Other treatments may be used in conjunction with surgery.
- Radiation therapy: After a craniopharyngioma surgery, external beam radiation therapy may be used to kill remaining tumor cells. X-rays and protons are used to deliver energy beams to the tumor cells, while sparing nearby healthy tissue. Advanced external beam radiation techniques such as proton beam therapy and intensity-modulated radiation therapy (IMRT) allow precise shaping and aiming of the radiation beam. In some cases where the optic nerve is not affected, a type of radiation therapy called stereotactic radiosurgery may be used, which focuses multiple beams of radiation on specific points to kill the tumor cells. Brachytherapy is another type of radiation therapy, where radioactive material is placed directly into the tumor to radiate it from the inside.
- Chemotherapy: Chemotherapy is a medical procedure that involves the use of drugs to eliminate cancerous cells. By administering chemicals directly into the tumor, the treatment can effectively target the affected cells without causing harm to the surrounding healthy tissues.
- Hormone replacement therapy: Since craniopharyngioma can disrupt the normal function of the pituitary gland and hypothalamus, hormone replacement therapy may be necessary to replace hormones that are not being produced adequately by these glands. This may involve taking medications to replace thyroid, adrenal, or growth hormones, among others.
