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Craniopharyngiomas are rare brain tumors that usually develop near the pituitary gland and hypothalamus. They are benign, slow growing tumors and rarely spread to other parts of the brain. However, the tumor can be very harmful due to the effect on nearby brain structures. Because of their location, craniopharyngiomas affect endocrine function and hormone levels. If the tumor compresses the optic nerve, vision may also be affected.
Childhood craniopharyngioma is most common in children ages 5-14 years. The tumor rarely occurs in children younger than 2 years old. Craniopharyngiomas account for about 6% of childhood brain tumors. There are about 100-120 cases of pediatric craniopharyngioma in the United States each year.
Treatments for craniopharyngioma include surgery, radiation therapy, or both. The goal of surgery is to completely remove the tumor or to remove a portion of the tumor to relieve symptoms prior to radiation therapy. Radiation therapy can be used alone or in combination with surgery.
The 10-year survival rate for craniopharyngioma is 80-90%. Because of possible long-term health problems, it is important for families to work closely with a multidisciplinary care team to support quality of life after craniopharyngioma.
Signs and symptoms of craniopharyngioma depend on the tumor size and location. Symptoms may include:
As the tumor grows, it may block the normal flow of cerebrospinal fluid. This causes a build-up of fluid within the brain known as hydrocephalus. The fluid causes the fluid-filled pathways (ventricles) in the brain to widen and increases pressure on the brain. Many of the symptoms of craniopharyngioma are due to increased intracranial pressure.
A diagnosis of craniopharyngioma starts with a physical exam, medical history, evaluation of symptoms, and sometimes blood tests. Doctors may then order an MRI or CT scan. Craniopharyngioma may be diagnosed based on imaging tests or analysis of tumor tissue or cyst fluid.
Specific medical tests to assist the diagnosis of craniopharyngioma may include:
Craniopharyngiomas can be divided into 2 main types:
There is no standard staging system for craniopharyngioma. The tumor is described as newly diagnosed or recurrent.
The 10-year survival rate for craniopharyngioma is about 80 to 90%. Craniopharyngiomas are benign and very rarely spread. However, these tumors can cause serious problems including changes in endocrine function and damage to the optic nerve. Therefore, it is important to consider long-term quality of life when planning treatments for craniopharyngioma.
Treatments for craniopharyngioma include surgery and radiation therapy. Currently, there is no effective chemotherapy, and chemotherapy is rarely used to treat craniopharyngioma. Targeted therapy is being studied for some types of craniopharyngioma.
In most cases, the treatment approach for pediatric craniopharyngioma is less a decision of survival and more of quality of life. It is important that children be treated by an experienced medical team including specialists in radiation oncology, neurosurgery, endocrinology, neurology, and ophthalmology. Doctors should discuss the risks and benefits of each treatment so that families can make informed decisions.
Most survivors of craniopharyngioma live with complications that affect quality of life due to the tumor or its treatment. These problems may include:
Life after craniopharyngioma may include problems with the endocrine system. The endocrine system is a group of glands that make hormones to control many of the body’s functions such as growth, puberty, reproduction, metabolism, urine production, sleep, body temperature, emotions, and responses to stress.
Most problems after craniopharyngioma are related to abnormal hormone levels that are caused by damage to the pituitary gland or hypothalamus. Hormones that may be affected include growth hormone, thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin, and antidiuretic hormone (ADH). Hormone replacement therapy is often needed.
Craniopharyngioma often causes hypothalamic obesity, extreme weight gain caused by damage to the hypothalamus. This is likely due to a complex interaction of factors including changes in metabolism (fewer calories burned at rest), increased appetite, and low physical activity levels. Obesity can occur even when patients don’t eat more than normal. It is hard to treat hypothalamic obesity, and it can be frustrating for patients, families, and doctors. Obesity increases risk for heart disease, diabetes, stroke, and metabolic syndrome as well as poor physical function, joint problems, and low fitness levels. This makes healthy habits like good nutrition and physical activity even more important for quality of life in craniopharyngioma survivors.
Metabolic syndrome is a common health issue that affects quality of life and longevity for survivors of childhood craniopharyngioma. This syndrome is a group of conditions that include obesity, high blood pressure, high blood sugar levels, high triglyceride levels, and low HDL levels (good cholesterol). Metabolic syndrome is linked to heart disease, stroke, and diabetes.
Sleep disorders such as excessive daytime sleepiness are common among craniopharyngioma survivors. This is often due to damage to the hypothalamus. A sleep disorder can contribute to symptoms such as fatigue, emotional and behavioral problems, and problems with learning or memory. Feeling tired also makes it hard to eat healthy or be physically active, which can lead to further health problems. It is important for patients to have healthy sleep habits and seek help if a sleep disorder is suspected.
Treatments for craniopharyngioma, including surgery and radiation therapy, can affect long-term brain health. Craniopharyngioma survivors are often at higher risk of developing problems in blood vessels of the brain. These changes can sometimes lead to ischemia or stroke. Patients are also at increased risk for seizures. Cognitive problems (cognitive late effects) may occur in children with craniopharyngioma. Classroom accommodations and school support services can help students and families cope with any educational challenges.
Although most children with craniopharyngioma survive their disease, long-term effects are common. There are things patients and families can do to help promote quality of life:
Reviewed: February 2020