Introduction to Childhood Craniopharyngioma

Executive Summary

Childhood craniopharyngiomas is a brain tumour type, usually found near the pituitary gland in the brain accounting for 6-9% of brain tumours in children. It is generally cystic, and the tumors are benign and usually not life-threatening. The CNS tumour craniopharyngioma usually develops in the part of the brain called the suprasellar region. It is considered a chronic disease due to its aggressive, locally invasive behaviour and its ability to affect vital structures such as the pituitary gland, hypothalamus, optic nerves, cavernous sinus, third ventricle, brainstem, frontal and temporal lobes. Craniopharyngioma and its treatment can cause many problems and abnormalities in children. Childhood craniopharyngioma affects a child’s growth and development and can cause vision loss, endocrinological, neurocognitive and metabolic issues. A multidisciplinary approach is necessary to manage patients diagnosed with craniopharyngioma tumors and their family members. Most cases of craniopharyngioma allow treatment and cure (accompanied by life-long side effects and treatment impacts), but some cases could be highly challenging and life-threatening.

What is Childhood Craniopharyngioma?

Craniopharyngioma is a brain tumor type, usually found near the pituitary gland in the brain. It is a common type of central nervous system (CNS) tumor. Craniopharyngioma accounts for 6-9% of brain tumors in children ​1​.

It is considered a benign tumour, which grows at a languid pace and is unlikely to metastasize and affect other body parts. But the disease should be regarded as chronic due to its aggressive, locally invasive behavior and its ability to affect some vital structures like the pituitary gland, hypothalamus, optic nerves, cavernous sinus, third ventricle, brainstem, frontal and temporal lobes.

Craniopharyngioma can be solid or cystic. A cystic tumor possesses a sac or closed pouch that contains the tumor fluid. Cystic parts have high amounts of protein in them. At the same time, the solid part contains calcium, which imaging tests like CT scans can detect. Childhood craniopharyngiomas are usually cystic. These tumors are benign and generally not life-threatening. But they can have a life-long impact on a person’s well-being. Also, there is no specific cure or treatment for this CNS tumor. The treatment strategies should be tailored as per the patient’s disease gravity. 

CENTRAL NERVOUS SYSTEM (CNS)

The central nervous system (CNS) refers to the part of the nervous system that contains the brain and the spinal cord. It is the vital spot where all the body’s essential functions are controlled and coordinated. The brain integrates the information received in the form of signals and controls and influences all parts of the body. The brain is the centre of memory, thought and emotion. It controls the five body senses like smell, taste, touch, sight and hearing. It also controls and coordinates other essential body functions like heartbeat, consciousness, breathing, circulation etc. the spinal cord is a long tube-like structure made up of nerves that carry messages from the body to the brain and vice-versa.

The CNS tumor craniopharyngioma usually develops in the part of the brain called the suprasellar region. This is the region just above the pituitary gland. The pituitary gland is vital in the body and is called the “master endocrine gland”, as it produces some essential hormones that regulate essential body functions. Nearby to the pituitary gland are the hypothalamus and optic nerves. Hypothalamus handles aspects like body temperature, hunger, sleep, thirst, fatigue etc.

Craniopharyngioma and its treatment can cause many problems and abnormalities in children or adults. Childhood craniopharyngioma affects a child’s growth and development and can cause vision loss, endocrinological, neurocognitive and metabolic issues ​2​. It can cause various psychosocial problems, memory loss, hypothalamic and pituitary dysfunctions. Hence even though benign, the disease can have severe, lasting impacts in children and adults.

A multidisciplinary approach is necessary to manage patients diagnosed with craniopharyngioma tumors and their family members. Most cases of craniopharyngioma allow treatment and cure (but accompanied by life-long side effects and treatment impacts), but some cases could be highly challenging and life-threatening ​3​. Mostly, the treatment will include the cooperation of experts from fields like oncology, ophthalmology, endocrinology, psychology and neurosurgery. Along with physical treatment, the healthcare team must also address the patients’ emotional, mental, and social issues and dilemmas. 

References

  1. 1.
    Müller HL. Childhood craniopharyngioma. Pituitary. Published online June 8, 2012:56-67. doi:10.1007/s11102-012-0401-0
  2. 2.
    Otte A, Müller HL. Childhood-onset Craniopharyngioma. The Journal of Clinical Endocrinology & Metabolism. Published online June 4, 2021:e3820-e3836. doi:10.1210/clinem/dgab397
  3. 3.
    Müller HL. Childhood craniopharyngioma: treatment strategies and outcomes. Expert Review of Neurotherapeutics. Published online January 13, 2014:187-197. doi:10.1586/14737175.2014.875470