Treatment for Childhood Craniopharyngioma

Executive Summary

The different types of standard treatments for childhood craniopharyngiomas are explained in detail. Standard treatments are used along with the integration of effective treatment planning. Clinical trials are used as an innovative treatment approach that combines different therapies and drugs for treating colorectal cancer. The treatment for children with craniopharyngioma brain tumors requires high expertise at a specialized pediatric Centre or sometimes at a general cancer Centre having a pediatric treatment Centre. The common treatment of childhood craniopharyngiomas includes surgery, radiation therapy (external-beam radiation therapy, radiosurgery), and intracystic therapy. Palliative care is being integrated to improve the patients’ quality of life. The patients continue to have regular tests, scans, and physical examinations even after successful treatment to look for recurrence in the later phase after the treatment ends. The clinical trials test are conducted for checking the viability of various treatment options for dealing with recurrent craniopharyngioma.

Treatment of Childhood Craniopharyngiomas

Craniopharyngiomas are rare, benign pediatric tumors that primarily develops in the pituitary gland or near the hypothalamus. Doctors use different procedures and techniques to treat childhood craniopharyngiomas. Since treating such brain tumors requires high expertise, children with craniopharyngioma brain tumors are mostly given treatment at a specialized pediatric Centre or sometimes at a general cancer Centre having a pediatrics treatment Centre ^​1​. The doctor specializing in treating brain tumors in children is called a pediatric neuro-oncologist.

For craniopharyngioma, the mode of treatment is individualized based on several factors like:

• The tumour size.
• The tumour location
• Its stage and grade
• Tumour extension
• Risk factors and overall health condition of the patient.

A multidisciplinary team will consist of doctors to carry out the craniopharyngioma treatment. In addition to treatment, the healthcare team will also focus on providing care and support to the children and their family members. Besides oncologists and other disease specialists, the healthcare team will include a child life specialist, nurses, physical and occupational therapists, dieticians, counsellors and social workers.

Most often, craniopharyngioma treatments are successful. Due to the present technology and advancements in treatments, the survival rates of patients with this brain tumour have mainly improved. Below is a list of treatment procedures used to treat and cure craniopharyngioma brain tumors. Your child’s active treatment will also focus on managing the symptoms and side effects that may arise due to the disease or its treatment.

Doctors will decide the best craniopharyngioma treatment plan suitable for your child’s condition. Parents and caretakers are urged to take time and learn in deep about all the available treatment options and ask questions and clear doubt with your child’s doctor or health care team. Talk to the doctor about the aim of each treatment procedure and what your child can expect during and post-treatment. Hence, shared decision-making is required between the patient and their doctors to decide and carry on with treatments effectively.

Caretakers of the child can also enquire about clinical trials that are taking place to formulate better craniopharyngioma treatment options for dealing with craniopharyngioma. Talk with the doctor about finding clinical trials and participating in them.

SURGERY

Surgery is the most common craniopharyngioma treatment procedure used to treat craniopharyngioma. A doctor who specializes in removing such tumours is called a neurosurgeon. 

The surgical procedure aims to confirm the disease diagnosis and eliminate as much tumor growth as possible. A complete resection (complete removal of craniopharyngioma tumor) with or without radiation therapy would be employed to treat the disease ^​2​. This option is possible in the case of about 70% to 85% of children diagnosed with craniopharyngioma tumors. 

Surgery can be effective but can cause a range of side effects. The side effects depend on the location of the tumor growth. The surgical procedure can cause damage to the parts of the brain near the tumor growth, which will eventually affect vital aspects like hormone functions, vision, metabolism, consciousness, movement of body parts etc. In some cases, the tumor may have grown into the optic chiasm, causing vision problems or into any major blood vessels. Removing such tumors are a high-risk job because it can cause life-threatening impacts for the patient. Hence in such situations where the tumor cannot be eliminated via surgery, the healthcare team will employ other possible craniopharyngioma treatment options.

Sometimes after surgery, the doctor will prescribe some additional treatments. This ensures that the tumor is eradicated and help the patient better manage the side effects or impacts of the surgery like hormone variations, obesity, severe bleeding, stroke, damage to the internal parts of the brain like the hypothalamus etc. Parents and caretakers of the child should talk with their doctor and understand the pros and cons of each surgical procedure.

RADIATION THERAPY

Radiation therapy is usually preferred when a tumour cannot be removed entirely via surgery. Generally, radiation therapy refers to high-energy x-rays or other particles to destroy tumour growth. A radiation oncologist is a doctor who specializes in performing this procedure. The most common form of radiation therapy is external-beam radiation therapy ^​3,4​. Here radiation is given from a machine outside of the body. But since radiation therapy can cause drastic side effects to the patients, doctors must use advanced planning techniques to minimize the impact of radiation. Techniques and methods should be employed so that they help lessen the quantity of radiation given to the areas of the brain that are not affected by tumour growth.

Doctors can employ a subtotal resection with radiation therapy or primary cyst drainage with or without radiation procedure. Radiosurgery is a method used to administer high dose radiation into the tumour without disturbing the nearby normal areas of the brain. This technique will require a head frame so the doctors know the exact point where the radiation treatment should be delivered. Radiosurgery is particularly used in the case of recurrent craniopharyngioma tumours.

Before beginning the craniopharyngioma treatment, the healthcare team should consider all the possible side effects of radiation procedures on children. The procedures are known to affect a child’s growth and development. It can cause permanent learning disabilities and memory problems. It can even affect a child’s metabolism and cause hormone imbalances.

Major complications and side effects of radiation therapy are:

1. Fatigue, nausea
2. Mild skin irritations and reactions
3. Loss of hair
4. Obesity
5. Loss of pituitary hormonal function
6. Cognitive dysfunction leads to memory problems and learning difficulties.
7. Chance of vascular malformations and strokes.
8. Visual impairment
9. Occurrence of second tumours
10. The primary tumour turns malignant upon radiation exposure. 

Parents and caretakers should enquire about the possible short term and long-term side effects of radiation therapy to your child’s doctor before the procedure begins.

INTRACYSTIC THERAPY 

Intracystic or intracystic interferon-alpha therapy can be considered a treatment option for craniopharyngioma. The treatment procedure is seen effective against primary cystic lesions or growths ^​5​. The approach ensures the integrity of the cyst wall to delay the need for alternative therapies or treatments ^​6​. 

PHYSICAL, SOCIAL AND EMOTIONAL SIDE EFFECTS OF CRANIOPHARYNGIOMA

Craniopharyngioma can cause many physical, emotional and social effects. Cancer care doesn’t end with active treatment. Post-treatment care is as critical as active treatment. Palliative or supportive care focus on managing and relieving symptoms and side effects associated with cancer treatment. Palliative care is carried out along with the active treatment strategy, which helps in improving the treatment efficiency by slowing the cancer growth or destroying it.

Palliative care focuses on improving a patient’s condition by helping them to manage and relieve symptoms. The care extends support not just to the patients but also to their loved ones. It also focuses on the non-medical needs of cancer patients, like managing finances. Palliative care is independent of the age, cancer stage or type of patient. Palliative care works best when it is begun right after a cancer diagnosis. This helps prevent the cancer condition and its symptoms and side effects from aggravating. Palliative focuses on improving the comfort and quality of a patient’s life. Palliative care enhances the effectiveness of active craniopharyngioma treatment. Sometimes a patient may receive chemotherapy, surgery or radiation therapy as palliative care.  

Parents or guardians of children with craniopharyngioma should talk with their child’s health care team about the aim of each treatment option, about the probable side effects and the available palliative care options before the active treatment begins.

The health care team will enquire about your child’s medical history, symptoms and side effects during treatment. Be sure to communicate whatever your child is feeling to the doctor. This helps them treat any symptoms or side effects your child is experiencing with ease. This will eventually help prevent your condition from becoming worse. 

INCREASED HUNGER AND WEIGHT GAIN

Obesity is a common side effect of craniopharyngioma. Patients may feel the urge to eat every time. The actual cause of this uncontrolled and increased appetite among children/adults with craniopharyngioma remains unknown. However, it is assumed that increased hunger results from damage to the hypothalamus and nearby areas or a side effect of surgery or radiation procedure. For most children with craniopharyngioma, obesity is seen to be the long-term side effect of tumour growth. Parents or caretakers and children should be well aware of this risk and pay attention to their eating habits. Eating healthy foods and exercising regularly is extremely vital. Patients can also consult a registered oncology dietician or any other healthcare member to help chart a diet plan considering all side effects and impacts of disease and craniopharyngioma treatment.

REMISSION AND THE CHANCE OF CANCER RECURRENCE

Remission refers to a stage where there are no signs or symptoms of cancer in the body. The condition can be called NED or “no evidence of disease” in the body. Remission can be permanent or temporary. Cancer patients in remission are constantly worried about a probable cancer recurrence. Patients must continue tests, scans, physical examinations even after successful treatment to look for recurrence. Parents or guardians of children with craniopharyngioma should talk with your child’s doctor about the chance of the tumour condition recurring back in your child’s body. If cancer recurs in the same place as before, it is called local recurrence, and if it happens nearby the origin point, it is called a regional recurrence. If it recurs in some distant place in the body, it is called distant recurrence.

The doctor will check for any signs of tumour recurrence. And if the tumour growth is back in the body, the doctor will list out the possible craniopharyngioma treatment options. In most cases, the treatment procedures for recurrent tumours include surgery, radiation therapy etc., but the same kind of radiation procedure cannot be given more than once.

A lot of clinical trials are testing the viability of various treatment options for dealing with recurrent craniopharyngioma. Parents are encouraged to talk with their child’s doctor about any such possibilities. Whatever the craniopharyngioma treatment plan be, palliative care will always be an essential part that will primarily focus on managing and relieving symptoms and side effects of treatment.

When a tumour recurs back in the body or the disease worsens, it is quite natural and entirely ok for patients and their loved ones to feel upset and be in extreme fear. In such cases, both the patients and their caretakers are encouraged to talk to the healthcare team or counsellors or seek support services or groups to cope with the circumstances.   

WHAT IF THE TREATMENT DOES NOT WORK?

Cancer recovery may not be successful at times. The disease condition is advanced or terminal if it cannot be controlled or cured. Most cases of craniopharyngioma are treated successfully, but there may be some that cannot be fixed or cured entirely, and this condition is called advanced tumor condition.

Any cancer stage can be stressful to both patients and their loved ones. A diagnosis that states an advanced, aggressive tumor can cause even more stress and make people hopeless and uncertain about their lives. Parents or guardians of children suffering from craniopharyngioma should have open, honest communications with their doctor or health care team about their concerns, feelings, anxieties and preferences. Health care team members are skilled and will support the patients and their caregivers. They will focus on improving the comfort and quality of the patient’s life by providing them physical, mental and financial support.

Children diagnosed with advanced stage craniopharyngioma whose expected survival chance is less than six months can consider hospice care. Hospice care focuses on providing the best possible quality care for patients whose survival chance is low. Patients and their families can talk to the health care team about the available hospice options, including a unique hospice Centre, hospice care at home or any other health care location. People can also avail nursing care options.

With proper nursing care and appropriate equipment, we can even make a hospital environment at home. This helps the patient to stay at one’s home comfortably. Children would like to be engaged in activities. They would be happy to attend school on days they feel well or keep up with other social connections and activities. The parents and the healthcare can design an appropriate range of activities for the child. Parents or guardians and the healthcare team should also focus on the patient being extremely comfortable physically and free from any pain as part of end-of-life care.

The death of a person or child is an immense tragedy. Losing a person to cancer can shatter family members and loved ones. At this point, family members may require support to cope with the situation. Family members of children who died due to craniopharyngioma or other grave diseases can seek help at the pediatric treatment centers. Most of these centers have professional support groups and staff to help people cope and face reality with courage.

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