Cancer and Craniopharyngioma: Is There a Link?

A craniopharyngioma is a brain tumor that more commonly affects children aged 5 to 14 and older adults over 50. Craniopharyngiomas are rare, making up about 6% to 13% and 1% to 3% of brain tumors in children and adults, respectively. 

Fortunately, craniopharyngiomas are benign and not cancerous. However, these tumors also pose many serious health risks. Here is a comprehensive overview of craniopharyngiomas, their overall effects on a patient's quality of life, and the available treatment options. 

Craniopharyngiomas form and grow near the pituitary gland. These tumors also grow close to other important structures, including the optic nerves and hypothalamus. 

Most craniopharyngiomas have a gradual, steady growth curve. They usually develop as cysts (hollow sacs filled with protein-rich fluid) or solid masses but may have both solid and cystic components. 

Interestingly, craniopharyngiomas affect young children and older adults. Most cases are diagnosed among children aged 5 to 14 and adults over 50. It is also worth noting that craniopharyngiomas are more common among children than adults. 

Any brain tumor is a serious matter, as it grows within a confined space in our body amidst essential structures. Fortunately, craniopharyngiomas typically remain where they are and do not spread to other areas of the body. However, a large craniopharyngioma can cause troublesome symptoms due to its proximity to the pituitary gland, hypothalamus, and optic nerves (responsible for vision).

Craniopharyngiomas grow near the pituitary gland, which synthesizes and produces major hormones that regulate many of the body's internal functions. These tumors can also affect other important structures in the brain as they grow bigger, including the optic nerve and hypothalamus.

Craniopharyngiomas can cause many symptoms, including the following:

• Slow growth in children
• Poor appetite in children 
• Irritability and mood swings 
• Behavior changes
• Loss of balance
• Hearing loss
• Nausea and vomiting
• Headaches and migraines
• Seizures
• Confusion and forgetfulness
• Sleeping disorders
• Increased thirst and excessive urination
• Weight gain
• Vision impairment

Patients may experience some or most of these symptoms. Please note many of these symptoms are nonspecific and may be caused by different tumors and other conditions. Some of these symptoms may have adverse effects on the patients' overall quality of life. For example, vision impairment can complicate navigation and other everyday tasks. 

It is worth noting that these symptoms start mildly and get worse over time as the tumor grows bigger. Patients may ignore some of the mild symptoms as temporary issues that will go away. Still, it is advisable to consult a doctor if the symptoms persist—an early diagnosis means earlier management and treatment of the tumor.  

Craniopharyngiomas' benign (non-cancerous) nature makes them easy to treat, and most patients experience relatively high survival rates. Children and adults diagnosed with this condition experience more than a 90% survival rate over a 10-year period after diagnosis. This means that 9 out of 10 people survive at least 10 years following the diagnosis. Many people likely live long after the study cutoff time of 10 years.

Although survival times may be relatively long, patients may experience decreased quality of life if they need to undergo repeat or multiple treatments. Unfortunately, craniopharyngiomas have high recurrence rates. Even craniopharyngiomas that were wholly removed through surgery can grow back in the same spot. This may necessitate a repeat operation or discussion of other treatment options, including radiation.  

Many patients live with a craniopharyngioma for years without realizing it until the tumor grows big enough to cause problems. Because there are a wide range of possible symptoms, it may be difficult to diagnose a craniopharyngioma in a timely fashion.

Diagnosis typically begins with a comprehensive history and physical examination performed by your physician, followed by laboratory (e.g., blood, urine) and imaging tests. Blood and urine tests can help to determine any abnormalities in hormone levels.

Imaging tests such as a CT scan or MRI test are often used to pinpoint an intracranial cause for your symptoms. These tests provide a detailed image of the brain, including characteristic features of the tumor and its proximity to surrounding structures.

Fortunately, craniopharyngiomas are treatable and have high recovery and survival rates. The most effective treatment options include: 

Surgery 

Surgery is the most effective treatment option for craniopharyngiomas. It can be performed either through a craniotomy to access the tumor through the skull, or a transsphenoidal approach to access the tumor through the nose.

The transsphenoidal approach is a more minimally invasive option that may be more favorable for smaller, midline tumors. Meanwhile, the open craniotomy approach may be more suited for larger, complex tumors that extend beyond the midline.

Radiation Therapy 

Although the goal of surgery is to remove the entire tumor, some pieces may be left behind to avoid damaging the surrounding healthy tissues. In this case, the radiation oncologist may recommend radiation therapy to keep the remaining tumor cells under control, thus preventing and inhibiting recurrence. Radiation therapy is often recommended as a follow-up treatment option after surgery.

Radiation therapy involves exposing the remaining tumor cells to beams of highly concentrated beams of energy, effectively killing them. The process is non-invasive and painless. Depending on the tumor’s size and position, there are various types of radiation therapy that may be offered.

Stereotactic radiosurgery is recommended for craniopharyngiomas that are not contacting the optic nerve. Stereotactic radiosurgery is also recommended for small tumors that are not close to delicate nerves and tissues. 

Chemotherapy 

Craniopharyngiomas can also be treated using chemicals and medication through chemotherapy. The drugs and chemicals are usually injected directly into the tumor, killing it from the inside without affecting the surrounding healthy tissues. 

However, chemotherapy is slower and less efficient than surgery and is not usually considered as the first option. Chemotherapy can also have adverse side effects. 

Hormone Replacement 

A craniopharyngioma can hinder the pituitary gland's ability to produce hormones essential for regulating many bodily functions. Abnormal hormonal levels can cause many health problems, necessitating hormone-replacement therapy. This treatment option essentially replenishes insufficient hormones using supplements, which can alleviate certain symptoms. This would also be required following treatment, if the pituitary gland was sacrificed to perform a complete tumor removal.   

Craniopharyngiomas are not cancerous. However, their proximity to critical structures such as the pituitary gland, hypothalamus and the optic nerves can cause troublesome symptoms when the tumor is large. Fortunately, craniopharyngiomas are treatable, and most patients experience high survival rates. Recurrence is relatively common and may necessitate repeated treatment and regular followup.

• National Brain Tumor Society 
• Pituitary Network Association
• American Brain Tumor Association 
• Pediatric Brain Tumor Foundation 
• Children’s Brain Tumor Foundation 
• Child Growth Foundation 
• Endocrine Society 
• Pituitary Foundation