Life After Craniopharyngioma Treatment

Receiving a craniopharyngioma diagnosis can be a cause for concern for many patients. As with any medical condition, most people want to know what the diagnosis will entail and what treatments are available. This information helps to alleviate concerns and allows for informed decision making.

It is also important to understand what might happen after craniopharyngioma treatment and what life will look like during recovery and beyond. Although survival rates are excellent, the tumor and its treatment can cause hormonal, emotional, social, and behavioral impairments that can lead to a decreased quality of life. Developing skills to cope with these potential challenges is important.

This article will provide an overview of life after being treated for a craniopharyngioma and equip patients and their caregivers with information about what to expect.

A craniopharyngioma is a slow-growing, relatively benign (non-cancerous) brain tumor. This tumor tends to grow close to the hypothalamus and pituitary gland, and as the tumor enlarges, it can place pressure on these essential structures, leading to symptoms such as:

• Headaches
• Balance issues
• Vomiting and nausea
• Fatigue
• Vision problems
• Cognitive difficulties

Craniopharyngiomas have no risk factors, and experts have not pinpointed a specific trigger for the condition. Craniopharyngiomas develop at any age but are most often seen in children and older adults.

There are three options available for treating a patient with a craniopharyngioma:

• Surgery: The traditional surgical method is a craniotomy, a procedure in which the skull is opened to access the tumor for removal. Alternatively, the minimally invasive transsphenoidal procedure involves using special tools to reach the tumor through the nasal cavity.
• Radiotherapy: External beam radiation is used to destroy the tumor cells. Doctors can deliver radiation with enough precision to avoid affecting other normal tissues surrounding the craniopharyngioma using advanced procedures like proton beam radiation therapy, intensity-modulated radiation therapy, and stereotactic radiosurgery (gamma knife).
• Chemotherapy: Although craniopharyngiomas are benign, chemotherapy may be used in certain cases. The medications are typically injected directly into the tumor to shrink it or prevent growth.

Thanks to medical advances, the survival rate for childhood-onset craniopharyngiomas has grown. However, treatment of the tumor is not the final step in dealing with the condition. Patients and their caregivers may need to manage health issues that arise after treatment, and be aware of visual, neurocognitive, behavioral, and emotional changes that may affect quality of life.

Because of the tumor's proximity to nerves involved in visual processing, vision problems may occur. These issues can persist in 50% to 80% of patients even after treating the tumor. In addition, younger patients are more likely to experience persistent visual difficulties.

Additionally, a craniopharyngioma can adversely affect hormone production. Your hypothalamus and pituitary gland are responsible for producing and releasing many of the hormones needed for essential functioning and growth. An enlarging craniopharyngioma can place pressure on or invade these hormonal structures, causing dysfunction and hormone deficiencies. Often, this persists after treatment and requires lifelong hormone supplementation. 

These same adverse effects can also be caused by surgical removal or radiation therapy due to the close proximity of the tumor to surrounding nerves and hormonal structures. In some cases, the treatment plan may involve intentionally removing parts of the nearby structures to achieve complete tumor removal.

For patients living with craniopharyngiomas, it will be crucial to view this as a long-term chronic condition. Although complete tumor removal may provide a “cure” by significantly reducing the chances of the tumor growing back, there is no perfect treatment that will eliminate both the tumor and the effects it has on the body.

Visual, hormonal, neurocognitive, and behavioral impairments can lead to issues in school performance and the ability to maintain relationships. Once tumor treatment has concluded, the patient will require continued care, monitoring, medication, and therapy to maintain a favorable prognosis.

Although medical therapies can address the physical impairments associated with the disease, families and caregivers will often have to deal with the emotional and social impacts that arise, especially in a growing child. Patience, kindness, and encouragement can help to support the patient during these challenging times. Patients or caregivers may also find it beneficial to participate in craniopharyngioma support groups to share and gain information about the condition from different perspectives.

Even with complete tumor removal, craniopharyngiomas can grow back. In fact, 20% to 40% end up recurring, though the chances are minimized if the tumor was completely excised. Thus, patients will undergo regular imaging tests to monitor for any signs of recurrence.

After surgical treatment, patients will typically face a recovery period between several weeks and months. During this time, the patient must follow up with his or her surgeon and physicians. There is a possibility that hormone therapy will become a long-term necessity, and the patient must undergo regular MRIs and CT scans to monitor for tumor recurrence. Attending follow-up appointments will also be important to address any neurological deficits stemming from the tumor or its treatment.

Survivors of craniopharyngiomas have a life expectancy of up to 90% at 10 years. This means that 90% of patients will survive for at least 10 years, though many go on to live much longer. Although patients often experience hormonal and/or visual deficits, many patients suffering from craniopharyngioma can go on to live with few significant changes to their quality of life. A multidisciplinary medical team and supportive caregivers will be essential to providing the best possible outcomes for the patient.

• A craniopharyngioma is a relatively benign tumor that grows near the hypothalamus and pituitary gland.
• Treatments for craniopharyngiomas include surgery, radiation therapy, and chemotherapy.
• Even with treatment, a craniopharyngioma is a long-term condition that causes hormonal and neurological changes that may affect the patient's quality of life.
• The outlook for patients suffering from craniopharyngioma depends on the mode of treatment and the severity of the condition.
• It is possible to survive a craniopharyngioma and the chances of a favorable prognosis from this condition increase with the proper care.

• 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