Pediatric Craniopharyngioma

What is Pediatric Craniopharyngioma?

Craniopharyngioma in children is considered one of the most complex brain conditions to treat. This is partly because the tumor is located close to vital parts of the brain, making it difficult to handle. In children, these brain parts are still developing and are highly sensitive, not just to the craniopharyngioma itself, but also to any treatments used which could potentially cause more problems. This review focuses on the most significant aspects of this condition in children.

What Causes Pediatric Craniopharyngioma?

A craniopharyngioma is a mostly harmless tumor found in the central nervous system, which is the part of the body that includes the brain and spine. However, there have been cases where these tumors were harmful. Craniopharyngiomas are part cystic, which means they have fluid-filled sacs, and they develop in the sellar/parasellar region, an area near the base of the brain.

Risk Factors and Frequency for Pediatric Craniopharyngioma

Craniopharyngioma is a type of brain tumor that can occur in people of all ages. Although it is often found in children and makes up a small percentage of brain tumors, about half of these tumors are diagnosed in adults. It most commonly appears in people between the ages of 5 to 14 and 50 to 74. It affects people of all genders, races, and places, and it’s not more common in any particular group. Family cases of craniopharyngioma are very rare and have been found only in two families.

This type of tumor has a tendency to come back, with a recurrence rate of about 50%. On the bright side, the chances of surviving for five years after being diagnosed with craniopharyngioma ranges from 83% to 96%, and the chances of surviving for ten years ranges from 65% to 100%. However, it should be noted that almost every patients with craniopharyngioma will experience some long-term effects or complications.

Signs and Symptoms of Pediatric Craniopharyngioma

Craniopharyngiomas are slow-growing tumors and are often diagnosed late due to their gradual symptom progression. These tumors typically lead to symptoms such as headaches and vision changes due to increased pressure in the skull. Additionally, these conditions often cause hormonal imbalances leading to growth delays, obesity, and late puberty. Below are some of the symptoms often reported in craniopharyngiomas patients:

• Headaches: Reported by roughly 50% of patients, these headaches are often associated with increased pressure in the skull or irritation from the fluid within the cyst.
• Visual Symptoms: Between 62% and 84% of patients experience vision changes. Temporal hemianopsia, or partial vision loss, due to compressing the optic chiasm is common. Regrettably, children often fail to recognize these changes, leading to permanent vision loss. Severe cases may also lead to optic nerve damage and visual field deficits.
• Hormonal Deficiency: Around 80-90% of patients will present one hormonal deficit initially, but only 15% of children will have symptoms leading to the diagnosis of craniopharyngioma. Some of these deficiencies include:
• Growth Hormone (GH) Deficiency: Common in 75% of pediatric craniopharyngioma patients, causing short stature.
• Gonadotropin Deficiency: Present in 85% of patients, causing delayed puberty.
• Thyroid-Stimulating Hormone (TSH) Deficiency: Present in 2-25% of patients, causing symptoms of hypothyroidism such as weight gain, fatigue, dry skin, cold intolerance, constipation, and failure to thrive.
• Adrenocortical Hormone (ACTH) Deficiency: Present in 25-70% of patients, often causing weight loss, fatigue, dizziness, anorexia, and hypoglycemia. Vasopressin deficiency, also known as diabetes insipidus (DI), is reported in approximately 9-38% of patients, leading to symptoms of increased thirst and urination.

Testing for Pediatric Craniopharyngioma

When a doctor suspects a patient might have a craniopharyngioma, which is a type of brain tumor, it often involves a team of medical experts for evaluation. These include an endocrinologist, who specialize in hormones, a neuro-opthalmologist, who focuses on vision-related nerve issues, and a neurosurgeon, a doctor who performs surgery on the brain and nerves.

The primary way to diagnose craniopharyngioma is through imaging tests, specifically, computerized tomography (CT) scans and magnetic resonance imaging (MRI). These tests are usually ordered when the patient is experiencing visual problems. Comparatively, an MRI is considered to be higher in quality because it gives a better detailed image of the tumor, its specific location, and its relation to the surrounding areas in the brain. Both CT and MRI scans can detect the cystic, or fluid-filled, areas of the tumor, but only CT scans can effectively see calcification, which is the accumulation of calcium in the tumor. Craniopharyngioma often contains a mix of solid, cystic, and calcified parts, knowing this can help identify the tumor in scans.

In addition to imaging tests, the doctors may request a full visual exam carried out by the neuro-opthalmologist if the patients are having visual issues. This exam tests the patient’s eyesight and field of vision.

Lastly, an endocrinologist will typically perform a series of tests to determine if there is any hormone deficiency, which might occur because craniopharyngioma is located near the pituitary gland, which is essential for hormone production. Tests may look for deficiencies in growth hormone (GH), gonadotropin (a hormone affecting growth and sexual development), thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH, which stimulates cortisol production), and vasopressin (a hormone that helps regulate water levels in the body).

These tests range from blood tests to measure hormone levels, X-rays to assess bone age (in the case of GH deficiency), to tracking fluid intake and output along with specific blood and urine tests (for vasopressin deficiency). In some cases, more specific tests may be ordered.

In summary, diagnosing craniopharyngioma requires a multi-disciplinary approach that includes several imaging tests and a range of hormonal evaluations. If your doctor suspects a craniopharyngioma, they will guide you through these tests to reach the most accurate diagnosis.

Treatment Options for Pediatric Craniopharyngioma

Treatment for a brain tumor known as craniopharyngioma can be complex because it sits close to important nerves and blood vessels inside the brain. There are various ways to tackle this tumor, including surgery, radiotherapy, and a treatment called intracystic therapy. The best treatment approach depends on factors such as the patient’s age, other health conditions they might have, and the specific characteristics of their tumor.

Neurosurgery is often used to confirm a diagnosis, treat tumors causing symptoms or brain fluid buildup, and manage any dysfunction of the pituitary gland, which is the part of your brain that controls growth and body function. The surgical approach can vary and could be done either through the nose (endoscopic endonasal transsphenoidal) or through the skull (transcranial), depending on where the tumor is.

In some cases, a grading system that categorizes the tumors based on their location and their connection to surrounding structures is used to decide the best surgical approach. If the tumor is cystic (filled with fluid), a tube can be inserted to drain the fluid, helping to avoid aggressive surgeries and further radiation therapy, especially in children.

Radiotherapy uses radiation to reduce the size of the tumor while sparing essential brain structures. Although radiotherapy can be a beneficial part of treatment, it has not been proven to reduce the likelihood of the tumor returning, so it is generally used as additional support to surgery.

Intracystic therapy, which is typically used for purely cystic craniopharyngioma, delivers toxic substances into the tumor to cause it to shrink. This treatment can be effective, but there’s a potential risk of severe nerve damage if the treatment leaks out of the cyst.

After the tumor is removed, hormone treatment is often required because the treatments for craniopharyngioma can affect glands that produce hormones. This can affect growth (GH Deficiency), sexual maturity (Gonadotropin Deficiency), thyroid function (TSH Deficiency), stress response, among others (ACTH Deficiency), and the body’s balance of water (Vasopressin Deficiency). The specific kind of hormone treatment given varies from patient to patient, and can include replacement hormones or drugs. It’s essential that patients receiving these treatments are closely monitored to allow adjustment of the therapy as required, and to pick up and manage any side effects.

What else can Pediatric Craniopharyngioma be?

When trying to diagnose craniopharyngioma, a type of brain tumor, doctors would consider ruling out several other conditions. These can be grouped into different categories:

• Inflammatory Conditions: These include pituitary abscess, lymphocytic hypophysitis, infundibulitis, histiocytosis, sarcoidosis, tuberculosis, and syphilis.
• Other Congenital Conditions: Rathke cleft cyst and arachnoid cysts fall under this category.
• Other Tumors: Some possibilities here are pituitary adenomas, primitive neuroectodermal tumors, hypothalamic hamartoma, germ cell tumor, epidermoid or dermoid tumor, meningioma, medulloblastoma, brainstem glioma, and lymphoma.
• Vascular Malformations: Doctors may also want to rule out a giant suprasellar carotid aneurysm, cavernous sinus hemangioma, and a carotid-cavernous fistula.

This is an important step in reaching the correct diagnosis and providing the right treatment.

What to expect with Pediatric Craniopharyngioma

The outcomes for patients with microadenomas, or tiny tumors in the glands, are generally great. But patients with macroadenomas, which are larger tumors, may experience lasting changes in vision, metabolism, or brain functions after treatment.

From data collected between 2009 to 2013, it was found that 89.5% of patients survived at least two years and 83.9% survived five years. Considering different age groups, the 5-year survival rate was 92.7% for children and teenagers between 0 and 14 years old, while for adults over 40 years of age, the survival rate was 77.7%.

Possible Complications When Diagnosed with Pediatric Craniopharyngioma

Craniopharyngioma can lead to several complications, either because of how it presses on nearby structures or because of issues that can arise after surgical removal of the tumor. Here are some of the possible complications:

• Issues with vision, including the loss of vision
• Diabetes insipidus and dangerously high sodium levels (hypernatremia)
• Buildup of fluid in the brain, a condition called hydrocephalus
• Seizures
• Anomalies in hormone levels made by the pituitary gland
• Obesity caused by problems with the part of the brain that controls hunger (hypothalamic obesity)
• Life-threatening situations, potentially leading to death

Preventing Pediatric Craniopharyngioma

If a patient is preparing for surgery, it’s essential that they are fully informed about what might happen. This includes understanding the potential complications that could emerge from this intervention. Through detailed discussions with their doctor, patients can develop a clear picture of the risks involved in the procedure they are planning to undergo.