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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 children with craniopharyngioma undergo surgery as part of their treatment plan. Surgery may be used to help in the diagnosis, relieve symptoms, and remove as much of the tumor as is safely possible. The extent of surgery for craniopharyngioma is based on the size and location of the tumor as well as potential risks. In some cases, long-term control of the tumor is possible with surgery alone.
Surgical techniques continue to improve to allow more precise and complete surgery with less risk to nearby structures. However, surgery is often not able to remove the tumor completely. In addition, there can be serious complications with more extensive surgery (radical surgery) due to the potential effects on important brain and endocrine functions.
Doctors may plan limited or conservative surgery that doesn’t fully remove the tumor. Instead, the primary goal in limited surgery is to treat symptoms or problems. In limited surgery, the surgeon is concerned with confirming the diagnosis, draining fluid from the cyst in the tumor, and relieving pressure on the optic nerve. Limited surgery is usually followed by radiation therapy.
||Avoid complications associated with radiation therapy
Surgery can damage nearby brain structures and cause both immediate and long-term effects:
|Limited Surgery + Radiation
||Less risk to personality
Radiation therapy can cause long-term and late effects:
Different surgical techniques are used to reach the tumor. Surgeons may be able to reach the tumor through transsphenoidal surgery (endonasal surgery). This minimally invasive surgery is performed through the nasal passages and sinus cavity to reach the tumor. Or, surgeons may perform a craniotomy, or surgical opening in the skull. The surgical technique is based on the location of the tumor and goals of surgery. Surgical techniques continue to improve to allow more precise and complete surgery with less risk to nearby structures.
Surgery to Manage Cysts - Craniopharyngioma tumors are usually made up of a solid mass and fluid-filled cyst. In some cases, surgery may also include placement of a catheter into the cystic part of the tumor in order to drain the fluid from the cyst. Removing this fluid can help many of the symptoms associated with the disease. The catheter may be temporary or permanent. This may include placement of an Ommaya reservoir, which allows fluid to be drained into a small, dome-shaped collection device placed under the scalp. After fluid collects into the reservoir, a needle can be used to remove the fluid.
Surgery to Manage Hydrocephalus - Some patients with craniopharyngioma may have surgery to help manage hydrocephalus. This may include creating an opening in the floor of the third ventricle in the brain (third ventriculostomy) to keep fluid from building up. In other cases, doctors may place a shunt, a small tube that drains cerebrospinal fluid (CSF) so that the fluid can be removed from the brain. The shunt may be temporary or permanent.
Radiation therapy may be used after surgery for craniopharyngioma, especially if limited surgery was performed. The type of radiation therapy used depends on the location of the tumor. Age of the child may be an important consideration in the use of radiation. Radiation therapy is also used if the tumor comes back after a previous radical surgery. Late effects due to radiation can occur and need to be monitored.
A newer form of radiation therapy, proton therapy, may have advantages over traditional radiation therapy using X-rays. However, this type of treatment is still being studied and is not available at every medical center.
Radiation therapy is often given in small doses called fractions. In fractionated radiation therapy for craniopharyngioma, patients usually receive treatments Monday – Friday, 5 days per week, for approximately 6 weeks. Radiation therapy is generally considered a one-time treatment. A repeat course of radiation therapy is rarely given.
In special cases, including patients with tumors that progress after radiation therapy, a single dose of radiation therapy (radiosurgery) may be used to treat craniopharyngioma. In radiosurgery, a higher dose of radiation is given to a focused area of the tumor. Tumors that progress after radiation therapy are difficult to cure if surgery cannot remove the tumor entirely.
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