A small tumor inside the head? Let's learn about craniopharyngioma in simple terms!

Have you ever heard of a strange tumor that grows inside the head, or rather in the brain, near a very small but very important gland – the pituitary gland ? This is called a craniopharyngioma. It's actually a bit rare, meaning it's not a disease that affects everyone. But this small tumor can have such a big impact on your vision and your endocrine system . So let's talk about this in a little more detail today, okay?

What is craniopharyngioma?

Simply put, a craniopharyngioma is a very rare, non-cancerous (benign) tumor. It develops near your pituitary gland. The tumor grows slowly. However, it can affect your cranial nerves , especially the nerves that connect to your vision. It can also affect your endocrine system, which controls the hormones in your body.

The main treatments for this are surgery and radiation therapy . Sometimes, chemotherapy is even used for a specific type of tumor, the papillary subtype.

The good news is that more than 90% of people with this tumor live five years after diagnosis. However, this tumor is considered a chronic condition . Because treatment does not always cure the other conditions caused by this tumor, and this type of tumor is more likely to recur.

Who gets craniopharyngioma?

Each year, approximately two people per million develop one of two types of craniopharyngioma: adamantinomatous and papillary . They are most common in two age groups:

• Children between 5 and 14 years old
• Adults between 50 and 74 years old

While the adamantinomatous type can develop in people of any age, the papillary subtype is most often seen in adults.

How serious is this craniopharyngioma?

In fact, craniopharyngioma is a serious medical condition. It can require lifelong medical treatment. About half of the tumors that are completely removed with surgery will come back after a while. Some of the health conditions caused by these tumors can persist even after the tumor is removed.

What is the difference between Craniopharyngioma and Pituitary Adenoma?

Both can affect hormone function. A pituitary adenoma is a tumor that develops in your pituitary gland. A craniopharyngioma develops near the gland. While both tumors are benign, craniopharyngioma tends to be more aggressive than a pituitary adenoma.

What are the symptoms of Craniopharyngioma?

Craniopharyngioma symptoms are related to conditions that occur when something affects your or your child's pituitary gland, hypothalamus gland, optic nerves , and brain.

• For children: This can affect the growth and development of children. This is more evident in children than in adults with a craniopharyngioma.
• For both children and adults: Vision may be affected, especially peripheral vision .

However, because these symptoms can be similar to those of other diseases, it can be difficult for doctors to quickly conclude that these symptoms are caused by this tumor.

Conditions caused by pressure on the pituitary gland

Your or your child's pituitary gland controls many of the body's hormonal functions. When a craniopharyngioma presses on this gland, the following conditions can occur:

• Growth Hormone (GH) deficiency: This can cause stunted growth in children. In adults, GH deficiency can lead to frailty, osteoporosis ( thinning of the bones), decreased muscle strength, increased LDL cholesterol, and heart disease.
• Gonadotropin deficiency: People with this condition may experience delayed puberty. Women may stop menstruating, a condition called amenorrhea .
• Adrenocorticotropic hormone (ACTH) deficiency: This can cause weakness and fatigue. It can also cause weight loss, loss of appetite, muscle weakness, nausea and vomiting, and low blood pressure ( hypotension ).
• Thyroid Stimulating Hormone (TSH) deficiency: People with low TSH may experience symptoms such as fatigue, irregular periods, and forgetfulness.
• Central Diabetes Insipidus (CDI): This condition causes excessive thirst ( polydipsia ) and frequent urination ( polyuria ). People with CDI may experience dehydration , irregular heartbeat, fever, dry skin and mucous membranes, confusion, and seizures.

Conditions caused by pressure on the hypothalamus

Your or your child's hypothalamus controls body processes such as mood, hunger and thirst, sleep patterns, and sexual activity. When a craniopharyngioma tumor presses on the hypothalamus, the following conditions can occur:

• Hypothalamic obesity: This refers to obesity that occurs despite calorie restriction, exercise, and diet control.
• Froehlich's syndrome: People with this condition feel hungry no matter how much they eat, leading to obesity. Children with Froehlich's syndrome may develop more slowly than other children. Some children may also have poor vision and intellectual disabilities.
• Non-24-hour sleep-wake syndrome: This is a sleep disorder that causes people to be unable to follow a normal sleep schedule. Some people can develop severe sleep deprivation .

How does this tumor affect your or your child's vision?

Craniopharyngioma tumors often develop very close to your or your child's optic nerves . When these nerves are compressed, they can cause blurred vision or problems with peripheral vision.

What causes craniopharyngioma?

Researchers still don't know the exact cause, but they believe that these tumors develop from cells that help form your or your child's pituitary gland. For some reason, these cells become abnormal, multiply, and grow.

How do doctors diagnose Craniopharyngioma?

If you are being examined for this type of tumor, your doctor may ask about your medical history and physical development. If your child is being examined, they may ask about his physical development, intellectual development, vision, and whether he feels unusually thirsty or needs to urinate frequently.

What kind of tests are done?

Doctors may perform tests like these:

• Blood tests: These measure certain hormone levels.
• Urinalysis: Checks for kidney problems.
• CT scan (Computed Tomography - CT scan): A series of X-rays and a computer are used to create three-dimensional (3D) images of soft tissues and bones.
• MRI Scan (Magnetic Resonance Imaging - MRI): This is a painless test that uses a large magnet, radio waves, and a computer to produce very clear images of the body's organs and structures.
• Biopsy: Doctors perform a biopsy (take a tissue sample) and examine the cells, fluid, tissue, or growths under a microscope.

Can Craniopharyngioma be treated?

Craniopharyngioma is usually treated with brain surgery . This can be done using a small camera (scope) inserted through the nose, or by making a small hole in the head ( craniotomy ). The goal of the surgery is to remove as much of the tumor as possible while minimizing damage to the pituitary gland, hypothalamus, or optic nerves. However, some people may need to take hormone medications after surgery. In addition, radiation therapy may be recommended after surgery if the tumor cannot be completely removed safely.

What are the common side effects of surgery?

Craniopharyngioma surgery is very challenging . First, because these tumors grow near very delicate nerves and the pituitary gland, there is a high risk of damaging them. Second, removing a craniopharyngioma is like removing a sticker from an envelope without damaging the envelope. This is because these tumors are so tightly attached to the pituitary gland and the nerves that connect to vision. Some doctors believe that surgery to remove these tumors can cause as much harm as the tumor itself. So, it is important to understand in advance what the purpose of the surgery is and what side effects to expect.

Can Craniopharyngioma be prevented?

Unfortunately, these tumors cannot be prevented. They form because of changes in cells that occur during the development of your or your child's body.

How long can you live with Craniopharyngioma?

Many people live for years after treatment. But these tumors can often recur. Overall, about 17% of tumors that are completely removed with surgery will come back. This number increases to 25% to 63% for tumors that are only partially removed. Most craniopharyngiomas that do come back will come back within three years of surgery.

Some doctors consider craniopharyngioma to be a chronic condition that requires long-term care and monitoring. There are several reasons for this:

• You or your child may need another surgery to remove a tumor that recurs.
• If you or your child's pituitary gland or hypothalamus is affected by a tumor or surgery, hormone replacement therapy may be necessary.
• Many children with hypothalamic obesity caused by the tumor are at increased risk of other health problems. If your child has hypothalamic obesity, you will need support and guidance to help your child make good food choices and exercise regularly.

When should I/my child see a doctor?

You or your child will likely have to live with a craniopharyngioma for many years. These tumors can grow back, and treatment may not completely eliminate all symptoms. Consider these things when planning your/your child's care:

• You should see your doctor annually to monitor your or your child's overall health.
• Your or your child's doctor will likely order annual scans, usually MRI scans , to check for recurrence of the tumors.
• You or your child may need help managing conditions such as hormone deficiencies or vision problems that were not corrected by surgery.

What questions should I ask my/my child's doctor?

Although craniopharyngioma is a rare, noncancerous tumor, it can cause several challenging medical conditions that require long-term care. If you or your child has this tumor, you may be concerned about managing this lifelong condition. Here are some questions that can help you talk to your or your child's doctor:

• I've never heard of this fruit. Can you explain what it is?
• Could this tumor cause me/my child to have other serious medical problems?
• Why did I/my child develop this tumor?
• What treatment options are there?
• What are the chances of treatment being successful?
• Will the treatment make my/my child's symptoms go away?
• What are the short-term and long-term side effects of treatment?
• What happens if the tumor grows back?

Even if you have surgery to remove a craniopharyngioma, these rare, noncancerous tumors can have long-term effects on you or your child. In addition, you or your child may need ongoing medical care for conditions caused by the tumor. In a way, craniopharyngioma is a chronic disease that you or your child will have to manage for the rest of your life. While doctors cannot cure it completely, they can do their best to help you and your child cope with the challenges of living with this rare tumor.

Finally, take-home message:

Craniopharyngioma is a serious and rare condition, but it can be managed with proper medical treatment and long-term planning.

• Seek regular medical advice: It is very important to stay in touch with your medical team and attend scheduled tests and appointments.
• Stay hopeful: Although this is a challenging journey, you are not alone. With the support of doctors, family, and friends, you will find the strength to get through this.
• Be informed: Being well informed about this condition, its treatments, and possible complications will help you make informed decisions.

Remember, early detection and proper management are the best ways to live successfully with this condition. If you or your child has any of these symptoms, don't hesitate to see a doctor.

` Craniopharyngioma, brain tumors, pituitary gland, hormones, vision, child health, non-cancerous tumors