Craniopharyngioma: A Comprehensive Review
- sunshine4cancerkid
- Aug 7
- 24 min read
Words: 6764
Thuvaraha Vinothakaran | Researcher/Writer
University of Toronto Scarborough
Kyle Lee | Researcher/Writer
Long Reach High School
Raya Kondakindi | Researcher/Writer
Princeton High School
Muneeba Usman | Researcher/Writer
Pakistan International School, Riyadh
Giada Santos | Researcher/Writer
St. Paul College of Bocaue
Table of Contents
Abstract...................................................................................................................................................2
Introduction......................................................................................................................................... 3
Overview of Brain Tumours..............................................................................................................3
Craniopharyngioma.......................................................................................................................... 3
Pediatric Craniopharyngioma............................................................................................................ 4
Discussion...............................................................................................................................................6
Why Craniopharyngioma? – A Researcher’s Perspective....................................................................6
Diagnosis............................................................................................................................................. 7
Signs and Symptoms............................................................................................................................ 7
Medical Equipment Used for Diagnosis...............................................................................................7
Medical Professions..............................................................................................................................9
Neurosurgeon / Pediatric Neurosurgeon.............................................................................................9
Neuro-oncologist / Pediatric Neuro-oncologist...................................................................................9
Neurologist / Pediatric Neurologist.................................................................................................. 10
Endocrinologist.................................................................................................................................10
Radiation Oncologist........................................................................................................................ 11
Treatments............................................................................................................................................ 12
Re-Introducing Craniopharyngioma..................................................................................................12
Surgical Procedures and Intervention................................................................................................13
Radiation Therapy............................................................................................................................. 14
Chemotherapy....................................................................................................................................14
Post-Treatment...................................................................................................................................15
Statistics................................................................................................................................................ 16
Demographics and Incidence.............................................................................................................16
Clinical Presentations at Diagnoses...................................................................................................16
Treatment and Recurrence.................................................................................................................16
Survival Outcomes............................................................................................................................ 16
Endocrine Dysfunction...................................................................................................................... 16
Visual Complication.......................................................................................................................... 17
Obesity and Metabolic Concerns.......................................................................................................17
Impacts................................................................................................................................................. 18
Overview........................................................................................................................................... 18
Craniopharyngioma in Children........................................................................................................ 18
Growth and Development..................................................................................................................18
Cognitive and Behavioral Changes................................................................................................... 18
Vision Problems.................................................................................................................................19
Metabolic Syndromes........................................................................................................................ 19
Conclusion.............................................................................................................................................21
Works Cited........................................................................................................................................... 22
Abstract
Craniopharyngioma (CP) is a benign brain tumour found near the pituitary gland in the
suprasellar region of the brain. While rare, it is a highly complex condition, particularly when
affecting children. This research paper focuses on pediatric craniopharyngioma, a form of CP
that occurs in children, which was chosen out of curiosity to better understand a condition that,
despite its non-cancerous nature, presents significant challenges due to its critical location and
probable lifelong impact on young individuals. The paper provides an introduction to pediatric
CP, outlining how it differs from presentation in adults, and then goes on to discuss diagnostic
procedures and essential medical equipment, the diverse medical specialists engaged in care,
treatment options, and consequences. This comprehensive review also highlights the survival
outcomes, high-risk demographics, and concerns through statistical measures. Overall, this paper
underscores the importance of taking early steps to diagnose and follow up with long-term plans
to improve the quality of life of CP patients.
Keywords: craniopharyngioma (CP), benign, brain, pituitary gland, Gross Total Removal
(GTR), Magnetic Resonance Imaging (MRI), Computed Tomography (CT)
Introduction
Overview of Brain Tumours
Brain tumours are abnormal mass growths that develop within the brain, disrupting normal
brain function. They are typically classified as either benign (non-cancerous) or malignant
(cancerous), with each type having distinctions in terms of diagnosis, treatment, and prognosis.
Malignant brain tumours such as gliomas or metastatic tumours are often associated with rapid
spread and growth, and the very aggressive treatments that we typically associate with cancer. In
contrast, benign brain tumours grow more slowly and usually do not spread to other areas,
although they can localize or become prevalent where they are.
Despite being classified as non-cancerous, benign tumours can still have significantly
debilitating effects on an individual, especially when they occur near critical brain regions
responsible for motor function, hormone regulation, or cognition, for example.
Another way to classify is by the onset of the tumour, whether it is congenital (present at
birth) or acquired, where the latter is more common. The classifications are numerous, and one
important distinction that should be understood is the differences we see with brain tumours in
adults versus in children. The histological classification of brain tumours differs significantly
between adults and children, as illustrated below in Figure 1 (Pollack 73).
Fig. 1. Histological Classification of Brain Tumours in Adults and Children. Adapted from Pollack, “Pediatric Brain Tumors,” Seminars in Surgical Oncology, vol. 16, no. 2 (1999): 73.
Craniopharyngioma
Craniopharyngioma (CP) is a rare, benign tumour that typically develops near the sellar
region, which includes the pituitary gland, hypothalamus, and optic nerves. These tumours are
most common in children, as seen in Figure 1, but can occur at any age.
Historically, these tumours were classified into two subtypes, grouped as different forms of
the same CP tumour: Adamantinomatous craniopharyngioma (ACP) and Papillary craniopharyngioma (PCP). However, the fifth edition of the WHO Classification of Tumors of
the Central Nervous System (CNS-5), now classifies these ACP and PCP subtypes as distinct
tumour types. This shift reflects a deeper understanding of their distinct molecular and
pathological characteristics, which makes room for improvement in developing more targeted
and personalized therapies and treatments (Louis et al. 1249). It is also an important
classification as ACP seems to be more common in children, whereas PCP is more so in adults.
Regardless of the specific subtype, CPs are generally classified as WHO Grade I tumours,
which establishes again that they are benign and slow-growing, and not as life-threatening in
comparison to the higher level Grade IV tumours that are more aggressive with poor prognosis
(Karsonovich et al.). But the fact that these tumours are benign does not make them any less
complex; for CPs specifically, the location is the most deadly factor. Since these tumours present
in the sellar and suprasellar regions, which are filled with critical structures in vision, endocrine
regulation, and neurological function (Karsonovich et al.). The proximity to areas like the optic
nerves can lead to visual impairment, while any growth touching the hypothalamus can cause
hormonal imbalance and endocrine impairments, which can further cause disruption in
metabolism, growth, and emotional being. On top of that, the delicate anatomical nature of CPs
makes complete resection a challenge, leading to the need for multimodality treatments to
achieve the best outcomes.
Fig. 2. A bisection of the brain depicting the areas most likely to be impacted by the Craniopharyngioma tumour.
Pediatric Craniopharyngioma
Pediatric craniopharyngiomas specifically refer to the occurrence of CP in children, most
commonly present in children between the ages of 5 and 14 years (“Childhood
Craniopharyngioma”).
When it comes to treatment, the standard for pediatric CP is surgical resection. As already
seen with CPs in general, this is a challenging approach due to their location and complexity.
Since these tumours are often closely associated with critical structures like the hypothalamus,
optic chiasm and nerves, and pituitary gland, achieving a complete surgical removal or gross
total resection without causing new neurological or endocrine deficits is very difficult, especially
in a child whose brain is still developing and whose future quality of life is one of the most
important factors. For this reason, neurosurgeons often lean towards subtotal resections,
deliberately leaving a tiny portion of the tumour behind to protect vital functions. As a result,
additional treatments and monitoring are needed to manage any tumour remnants.
Discussion
Why Craniopharyngioma – A Researcher’s Perspective
● Taking into account the rarity of the disease, to delve further and expand others’
knowledge for further advancements is interesting. The unique characteristics of the
disease, along with their significant impacts, which should not be taken for granted, are
matters that must be shed light on. In spite of being benign, their effects highly impact the
overall aspects of an individual, especially on children. Further, this disease is a
challenging surgical and therapeutic target on which more focus and research are required
to tackle and overcome the complexities of this condition. By understanding the disease,
more people, especially children, will be provided with guidance through research
regarding Craniopharyngioma.
● Having experienced being diagnosed and admitted into the hospital for a neurological
disorder, this topic seems very close to my heart. Waiting through long days in a lone
hospital bed was agonizing until I discovered that my condition could be cured; I felt
ecstatic. Spreading awareness of this condition seems to me the best way I can help other
children and people feel confident in their health improvement.
● Having been fascinated with all the things possible employing the brain and the signals it
sends to regulate the entirety of one’s body, studying Craniopharyngioma was the natural
choice, as this tumour is nestled within the pituitary gland and therefore its position and
the pressure it puts on nearby structures completely changes one’s daily life without even
being cancerous.
Diagnosis
Signs and Symptoms
Craniopharyngioma is a tumour that grows in the brain stem by the pituitary gland (the
gland responsible for hormone secretion to aid bodily function); thus, although benign, the
tumour leads to significant effects, especially in children and elderly adults (Mayoclinic).
Common symptoms are as follows:
Headaches: As the tumour grows, it blocks the flow of spinal fluid from the brain, thereby
creating an environment of excess fluid. This buildup heightens the pressure in the area, leading
to headaches and a nauseous feeling (Mayoclinic).
Vision Impairment: The tumour grows in an area that puts pressure on the optic nerves in
one’s eyes, compressing them, leading to vision changes and impairment in either or both eyes
(Boston Children’s Hospital).
Hormone Imbalances: As the pituitary gland is responsible for regulating and secreting
hormones, the tumour’s growth in this area and its general positioning lead to severe hormone
imbalances within the body, which have consequences that change one’s ability to grow (BCH),
such as:
Excessive Thirst & Urination (Diabetes Insipidus): caused by a disruption in the
production of the hormone vasopressin, which regulates water flow in the kidneys
(Healthline).
Imbalance and Coordination Issues: pressure on the brain by the tumour and
especially the cerebellum, which is responsible for controlling one’s balance (Cleveland
Clinic).
Personality Changes: the position and pressure of the tumour lead to poor mental
health, reduced social functioning, and increased risk of mood disorders (Daughters, et.
al).
Other conditions linked to Craniopharyngioma: Hypothalamic obesity, Froehelich’s
syndrome, and Non-24-hour sleep-wake syndrome, among others (Cleveland Clinic).
Medical Equipment Used for Diagnosis
When a healthcare professional initially suspects a patient has Craniopharyngioma, an
imaging-based test in the area surrounding the pituitary gland may be the first means of
proceeding to a diagnosis (Johns Hopkins). A Magnetic Resonance Imaging (MRI) test is a
painless radiographic test that uses a large magnet and radio waves to create a clear picture of the
structure within one’s body. A Computed Tomography (CT) scan is somewhat similar and uses
X-rays to create an image of the bones and soft tissue within a body (Cleveland Clinic). If the
imaging tests prove inconclusive or further investigation is needed, in general, a biopsy is
performed. This test involves the removal of a tissue sample from the pituitary area to detect
damage and hormone level changes (Colombia Neurosurgery).
Medical Professions
The successful treatment and long-term management of pediatric craniopharyngioma (CP)
requires a multidisciplinary medical team. Due to the tumour’s proximity to important and
delicate brain structures like the pituitary gland, hypothalamus, optic nerves, and cranial nerves
(see Figure 2), a wide range of specialists and professionals are needed to ensure safe treatment,
post-treatment care, and follow-up (“Childhood Craniopharyngioma”; “Pediatric Cancer Care
Team”). Below are several key medical professionals involved in the care of children with this
condition.
Neurosurgeon / Pediatric Neurosurgeon
One of the most important specialists in the treatment of pediatric CP is a pediatric
neurosurgeon. Their primary role is to surgically remove the tumour while protecting nearby
structures. They may perform either a full (gross total) or partial (subtotal) resection depending
on tumour location and risk of complications. Neurosurgeons also play a major role in
post-operative monitoring and coordination of other treatments or therapies (“Childhood
Craniopharyngioma”).
The path to becoming a neurosurgeon is long and complex. It begins with a bachelor’s
degree, typically in a science-related field, along with the completion of pre-medical courses.
Afterwards, individuals must attend medical school for four years to obtain their M.D. (Doctor of
Medicine) or also in the United States, a D.O. (Doctor of Osteopathic Medicine) degree. Once
medical school is complete, they enter a neurosurgery residency, which typically lasts six years
in Canada and seven years in the United States. To further specialize in pediatric neurosurgery,
individuals can complete an additional one-year fellowship, where they gain experience in
treating children with neurological conditions. (Torres, “How to Become a Neurosurgeon? - A
Step-By-Step Guide”; Employment and Social Development Canada, “Neurosurgeon in Canada |
Job Requirements”)
Salaries for pediatric neurosurgeons vary significantly based on experience and location. In
Canada, neurosurgeons earn an annual salary ranging from approximately $137,000 to $776,000,
with a median salary of about $423,000 (Employment and Social Development Canada,
“Neurosurgeon in Canada | Wages”).
Neuro-oncologist / Pediatric Neuro-oncologist
A neuro-oncologist is a pediatric subspecialist who oversees the non-surgical management
of brain tumours. They work closely with neurosurgeons and radiation oncologists to create
individualized and personalized treatment plans. Their responsibilities can include coordinating
chemotherapy, managing tumour growth, and monitoring recurrence in the long run (“Childhood
Craniopharyngioma”).
The education pathway to become a neuro-oncologist mirrors that of other pediatric
subspecialties. It begins with a bachelor’s degree, followed by four years of medical school to
obtain an M.D. or D.O. After medical school, individuals complete a pediatrics residency, which
is typically five years in Canada and three in the United States. From there, they complete a 2-3
year fellowship in pediatric hematology-oncology. To further specialize in brain tumours, some
go on to complete an additional 1-2 year fellowship in pediatric neuro-oncology. (Career
Counseling Office; Morra et al.; “Pediatric Hematology-Oncology Fellowship”;
“Hematology/Oncology”; “Pediatric Neuro-Oncology Fellowship Programs”)
The average salary for a pediatric neuro-oncologist in the United States is approximately
$175,055 per year (ZipRecruiter, “Pediatric Neuro Oncology Salary”). In contrast,
neuro-oncologists, in general, earn higher salaries around $288,841 per year in Canada and
$392,661 per year in the U.S. (Glassdoor; ZipRecruiter, “Neuro Oncologist Salary”)
Neurologist / Pediatric Neurologist
Neurologists are often involved in diagnosing pediatric brain tumours like
craniopharyngioma. They help assess any neurological symptoms, work with MRIs, and manage
neurological issues like seizures, adequate brain function, and also hormone imbalances and their
possible neurological consequences. Their primary role is to monitor neurological health (Torres,
“How to Become a Neurologist - Eight Steps after High School to Licensing in Neurology”).
To become a neurologist, individuals complete a bachelor’s degree and medical school.
Afterward, they enter a neurology residency, which is four years in the U.S. or five in Canada.
Those focusing on children complete an additional 1–2 year pediatric neurology fellowship
(Employment and Social Development Canada, “Neurologist in Canada | Job Requirements”;
Torres, “How to Become a Neurologist - Eight Steps after High School to Licensing in
Neurology”; Faculty of Medicine and Health Sciences, McGill University)
Salaries for neurologists in Canada range from approximately $121,470 to $613,031, with
a median salary of about $299,472 (Employment and Social Development Canada, “Neurologist
in Canada | Wages”). In the United States, the median salary is $301,000 (Torres, “How to
Become a Neurologist - Eight Steps after High School to Licensing in Neurology”).
Endocrinologist
An endocrinologist is an important player who monitors and manages any hormonal
imbalances or issues. This is particularly important for CP bc when the hypothalamus or pituitary
gland is affected. Endocrinologists, who may specialize further to become pediatric
endocrinologists, neuroendocrinologists, or even pediatric neuroendocrinologists, play a major
role in the long-term care of individuals with this condition and their quality of life.
The path to becoming a pediatric endocrinologist includes a bachelor’s degree, medical
school, and either a pediatrics or internal medicine residency. This is followed by a fellowship in
endocrinology or a subspecialty of it (Division of Endocrinology; Employment and Social
Development Canada, “Endocrinologist in Canada | Job Requirements”)
Salaries for endocrinologists in Canada range from approximately $121,470 to $613,031,
with a median salary of about $299,472 (Employment and Social Development Canada,
“Endocrinologist in Canada | Wages”). But, this again can differ depending on the level of
specialization, experience, and location of practice.
Radiation Oncologist
A radiation oncologist is responsible for planning and delivering radiation therapy when
surgery cannot fully remove the tumour or in cases of recurrence.
To become a radiation oncologist, one must complete a bachelor’s degree, followed by
medical school, and then enter a residency in radiation oncology, which typically lasts five years
in Canada (University of Toronto). Some may pursue additional fellowship training in advanced
radiation techniques.
Radiation oncologists in Canada earn between CAD $121,470 and $613,031 annually, with
a median salary of approximately CAD $299,472 (Employment and Social Development
Canada, “Radiation Oncologist in Canada | Wages”).
Treatments
Re-Introducing Craniopharyngioma
Case Overview: Craniopharyngioma is a nonmalignant brain tumour that often grows in
the suprasellar area. The suprasellar area of the brain is located near the pituitary gland and optic
nerves. The diagnosis is an epithelial tumour, -Neoplasia originating from epithelial cells that
coat internal organs and cover surfaces- arising through the craniopharyngeal duct. The condition
makes up 2.5% of all major intracranial neoplasms. Craniopharyngioma tends to present in a
bimodal age distribution, with the first peak occurring in childhood, from ages 5 to 10, and the
second peak arising at ages 40 to 60. Gender is not a factor in procuring the condition, as it
affects men and women equally. (Yad et al. 1; “Clinical Pathology Glossary”; St. Jude |
“Craniopharyngioma Treatment”)
Fig. 3. A mechanical scan of a patient with craniopharyngioma and the area it sits in the brain. So
urced from Ditki.
Treatment Options and Conditions: Treatment for Craniopharyngioma tends to be brain
surgery using a scope through the nose or an opening in the head referred to as craniotomy.
When doing surgery, the objective is to remove as much of the tumour as possible, all while
minimizing damage to the surrounding area. After the procedure is done, the patient may still
require hormone medications, and post-surgery radiation therapy can be helpful. It should be
noted that even if the tumour has been successfully removed, it can grow back within 3 years at a
rate of 17%, increasing drastically with a partial removal. Before any surgical operation takes
place, several tests must be completed, including but not limited to Magnetic Resonance Imaging
(MRI), Computed Tomography (CT) Scans, Neurological and Hormonal evaluations, and the
patient’s overall health. All tests must be completed thoroughly in order to have the best possible
outcomes. The tumour must be located and its size measured to know the risks of damaging
other parts of the suprasellar area. Evaluations of the patient’s neurological and hormone levels
are required because the common location of the tumour can create major postoperative issues
and may disrupt hormonal balances orchestrated by the pituitary gland. (“Clinical Pathology
Glossary”)
Early Detection: Identifying Craniopharyngioma early is key to planning and intervention.
The best hope when diagnosed with Craniopharyngioma is that the tumour is in its early stages.
Being fortunate to find an early case means having the ability to remove the tumour before it
causes serious alterations to the hypothalamic and pituitary stalk. Another benefit of early
identification is target-specific surgeries and distinct recovery treatments. All information is
added on top of the reduced risk of the patient receiving any long-term complications and
standard of living conditions. (St. Jude | “Craniopharyngioma Treatment”)
Advanced Scanning Machinery: Through means of mechanical scanning, the size of the
tumour can be measured, giving specialists an idea of how to proceed if surgery is planned. CT
and MRI scans are most commonly used to examine a patient’s brain and are extremely effective
at revealing sellar and suprasellar masses. In contrast, CT scans and MRI serve different
purposes. CT scans evaluate the level of calcification of a tumour if one is found to have it, and
MRI imaging pinpoints cysts and solid materials, along with the craniopharyngioma tumour’s
extent. MRIs can also gauge the tumour’s relation to the chiasm, pituitary stalk and gland, and
hypothalamus. (“Clinical Pathology Glossary”; AANS)
Surgical Procedures and Intervention
Open Craniotomy: The medical specialist, typically a neurosurgeon for craniopharyngioma
cases, creates an incision on the patient’s scalp or the region of the eyebrow. This step provides a
direct opening into the skull of a patient, allowing professionals to remove parts of or the entire
tumour. The procedure utilizes a microscope during the removal process. The hole in the skull is
then patched with plates and screws before the incision is sewn back together. (AANS)
Fig. 4. Endoscopic images are shown of the benign tumour’s removal process. Sourced from National Library of
Medicine.
Endonasal (Transphenoidal): This surgical procedure is done through the patient’s nose.
The neurosurgeon on deck typically works in collaboration with an otolaryngologist, -medical
study and specialty focused on diagnosis, management, and surgical treatment of issues that
involve the ear, nose, throat, and other similar structures of the head, to access the surgical site
through the nasal passage. Endonasal surgery utilizes an endoscope, a camera, and a light source
that can be used to locate the tumour through the usage of the nasal passage. It should be noted
that tumours over 3cm in diameter have lumbar drains placed at the start of the operation.
Another key point is that both nostrils are used to maximize workspace and the usage of multiple
surgical instruments. Without any external incisions, the surgeon removes the tumour and
reestablishes the space in between the brain and the nasal pathway using various body tissues or
medically imitated material. (NIH; AANS)
Terms - Gross Total Removal (GTR): In surgery, GTR refers to the hope of fully removing
the tumour from the patient’s system, although it may not be possible in all cases without causing
severe or long-term damage. (Medscape; National Library of Medicine)
Radiation Therapy
Exterior Beam Radiation: The patient lies on a table while a machine moves around them,
accurately directing radiation to specific points on the body. Advanced radiation technology
allows a health care team to monitor a patient’s condition directly, in other words, by shaping and
aiming the beam of radiation. Doing so can reduce the damage to any healthy cells and tissue.
Radiation therapy is normally done to minimize the chances of craniopharyngioma from
recurring and is done when GTR isn’t feasible. (MAYO Clinic; UFHealth)
Stereotactic Radiation: An intense form of radiation therapy that is normally completed in
one or a small number of sessions. The process of stereotactic radiation involves a multitude of
radiation beams fired simultaneously from various angles at the tumour location. (MAYO Clinic)
Proton Therapy: Closely related to stereotactic radiation, proton therapy is often used for
pediatric brain tumours, using high dosages of radiation. The greatest benefit of proton therapy is
its precision, being able to shoot radiation at the tumour with a lesser chance of damaging
healthy cells and tissue surrounding the area than other common forms of radiation therapy. This
newer form of radiation therapy may reduce the chance of side effects resulting from radiation
therapy. (St.Jude | “Proton Therapy”)
Brachytherapy: Typically, radiation therapy is an external process; however, brachytherapy
is a form of radiation therapy that happens internally. A radioactive material is strategically
placed inside a tumour to eradicate the harmful cells and tissues, inside out. (MAYO Clinic)
Chemotherapy
Craniopharyngioma Specific Chemotherapy: Applying medication as a means to terminate
the tumour, it can be injected directly into the tumour. Medication tested by NCI-funded trials
shows that a combination of vemurafenib (Zelboraf) and cobimetinib (Cotellic) can delay and
has promising signs of eradicating the condition with no need for further treatment. (MAYO
Clinic; National Cancer Institute)
Post-Treatment
Post Surgery: Due to the hazardous spot where the tumour tends to grow in the suprasellar
region of the brain, it can affect the functions of delicate parts. The process of removing a tumour
is connected to extremely sensitive areas of the brain, so many surgeons and professionals
believe that performing surgery to remove the tumour can cause just as much damage.
(Cleveland Clinic; Science Direct)
Post Radiation Therapy: Continuous monitoring must be done to evaluate a patient’s
recovery process and to predict if another case of craniopharyngioma may arise. Through
long-term monitoring, specialists can assess a patient’s cognitive function, optic neuropathy, and
risk of late toxicities. (National Library of Medicine; Science Direct)
Fig. 5. Table of treatment type, description of treatment procedure, and advantages and disadvantages of the
treatment type. Sourced from National Library of Medicine.
Statistics
Demographics and Incidence
Craniopharyngiomas (CPs) are rare, benign tumours arising from remnants of Rathke’s
pouch, accounting for 1.2% to 18.4% of paediatric primary brain tumours globally (Mohammed
et al.)(Oliver et al.)(Mareina et al.). Their annual incidence ranges between 0.5 and 2.5 cases per
million, with about 338 new diagnoses annually in the U.S., 96 of which are in children under 14
(Greta et al.). CBTRUS (2020) reports a national incidence of 0.13 per 100,000 person-years,
with a bimodal age distribution peaking in children aged 5–14 and older adults aged 50+.
Clinical Presentations at Diagnoses
CPs are equally likely in boys and girls, though some cohorts show slight male
predominance (57–58%) (Magerman et al.); (Lim et al.). The mean age at symptom onset is
around 7 years, with an average diagnostic delay of 6 months, often due to vague symptoms and
the tumour’s slow-growing nature (Magerman et al.).
Headaches and vomiting linked to raised intracranial pressure are the most common
presenting symptoms (63–75%), followed by visual deficits (47–79%), growth failure (26–61%),
polyuria/polydipsia (16%), and obesity (10–13%) (Magerman et al.); (Puget et al.); (Lim et al.).
Treatment and Recurrence
Treatment is almost always surgical. In multiple studies, only 41–42% of patients had
gross total resection, with the rest undergoing subtotal resection followed by radiotherapy
(Magerman et al.), (Lim et al.). Despite this, tumour recurrence rates remain high, occurring in
58–83% of patients, typically within 6–22 months post-surgery. Even after gross total resection,
recurrence rates can range from 0 to 50%, while subtotal resections may reach 25–100%
recurrence (Lim et al.).
Survival Outcomes
Survival outcomes remain generally favourable, though not without challenges. Five-year
survival rates hover around 83–96%, 10-year survival rates range from 65–100%, and long-term
survival stabilises near 62% (Puget et al.); (Lim et al.). In the Singapore cohort, overall survival
was 75%, with median progression-free survival at 4.16 years and median time to recurrence just
5.87 months (Lim et al.).
Endocrine Dysfunction
Endocrine dysfunction is the most consistent long-term complication. At diagnosis, 53% of
children already had pituitary deficiencies, which increased to 100% over 10 years (Magerman et al.). Post-treatment, 40–87% of children experience hypothalamic-pituitary dysfunction,
primarily impacting growth hormone, gonadotropins, and ACTH/TSH levels (Lim et al.).
Visual Complication
Visual complications are also common. At diagnosis, 27% had papilledema, and 79% had
visual impairment, though only 47% noticed symptoms (Magerman et al.). Postoperative
changes include worsening vision in some cases and persistent impairment in most cases. Visual
decline is often associated with larger tumour volumes and recurrence (Puget et al.).
Obesity and Metabolic Concerns
Obesity is another major concern. While only 13% of patients were obese at diagnosis, this
increased to 40% six months after surgery, with weight gain continuing long-term despite no
significant correlation with tumour size or surgical approach (Magerman et al.). These findings
reinforce the need for early intervention and holistic long-term follow-up.
Impacts
Overview
Craniopharyngioma is a non-cancerous brain tumour found near the pituitary gland. It can
occur at any age, but is most often diagnosed in children between the ages of 5 to 14 and older
adults. They do not spread to other parts of the brain or the body. They are usually part solid
mass and part fluid-filled cyst. In spite of being benign, they can press on nearby parts of the
brain, such as the optic chiasm, optic nerves, and fluid-filled spaces in the brain. They can still
have significant impacts due to their location near vital brain structures. The effects depend on
the size of the tumour, location, and the degree to which it compresses surrounding tissues.
Craniopharyngioma can affect both children and adults, causing long-term and major effects.
Craniopharyngioma in Children
Craniopharyngioma can significantly impact a child’s health and development. The tumour
can grow or spread to other areas of the brain, which causes major effects. Even after treatment
ends, children may still experience some complications. The tumour can be fully removed with
surgery in 9 out of 10 children. Although there are some cases wherein the tumour may grow
back. Most cases involve the tumour growing back 2 years after surgery. Craniopharyngioma can
cause certain changes in how the brain cells function, particularly how they divide and grow new
cells. The effects of disease before and after treatment are equally significant. The impacts of
craniopharyngioma in children include:
Growth and Development
A child’s pituitary gland manages many hormonal functions. The pressure caused by
craniopharyngioma may affect a child’s growth rate, leading to growth hormone (GH) deficiency
(Brinkman et al.). This is a condition wherein the pituitary gland is unable to release enough
growth hormones. Those affected by GH deficiency are shorter than expected with normal body
proportions. Craniopharyngioma can also delay puberty as there is a deficiency in gonadotropins,
which are hormones that stimulate the reproductive system. Both boys and girls may have a
delay in puberty. Because of the location of these tumours near the hypothalamus and pituitary
gland, hormonal imbalances occur. The hypothalamus is small yet does a lot of control in the
brain, especially when influencing the pituitary gland to release hormones. Also, girls who are
already 15 or older may experience amenorrhea, which is the delay of one’s menstrual cycle
(Pinkerton).
Cognitive and Behavioral Changes
A study conducted by Goodnight-Fournier et al. showcases how children with
craniopharyngioma show weaker neurocognitive performance in comparison with the normal
population before treatment. They showed poor performance in aspects of executive functions, learning/memory, motor abilities, adaptive skills, psychosocial adjustment, and academic skills.
Further, before radiation therapy, participants in this study showed reduced performance in
working memory in the naturalistic setting and verbal fluency. An even weaker performance was
found in visual-spatial learning and memory. Short-term visual-spatial memory was weak, but
long-term visual-spatial memory was intact. After treatment, neurobehavioral, social, and
emotional dysfunction were still quite prevalent. According to Zada et al., between one-third and
one-half of patients with treated childhood-onset craniopharyngioma have impairments in social
function, school, and maintaining relationships. Beyond neurocognitive changes, patients seem
to be more affected in the aspects of social, behavioral, and emotional function than they are by
neurocognitive or physical impairments. Some studies that focus on neurocognitive abilities have
noted that some children with treated craniopharyngioma have a normal IQ. Impairments in
cognitive and behavioural aspects were seen with depression and somatic concerns, which have
an overall impact on the mood, attention, familial and other interpersonal aspects, and school
function. Common effects of these behavioral impacts include social isolation, internalizing
behavior, and withdrawal, which vastly affect the child’s social life and mental health. Emotional
outbursts, impulsivity, and aggressiveness are also neuro-behavioral effects, which define
impaired social-behavioral abilities and emotional dysfunction.
Vision Problems
The tumour can cause pressure that affects the nerves of the eyes. This can lead to vision
loss in one or both eyes. The tumour’s location near the optic nerves and chiasm highly impacts
the vision of a child with craniopharyngioma. These tumours may compress or infiltrate the
visual pathway, which leads to a range of visual impairments. Children may develop a decrease
in visual acuity (VA), visual field defects (VF), particularly bitemporal hemianopia or abnormal
pupillary responses (Garnett et al.). The increased intracranial pressure can lead to papilledema
with subsequent optic atrophy and permanent vision loss as potential complications (Edmond et
al.). Hypothalamic-pituitary dysfunction, a decrease in one or more hormones, is another
impairment a child may experience when the disease is not treated properly or adequately.
Moreover, therapeutic interventions such as gross total tumour resection can lead to further
vision loss. This form of therapy causes a high risk of vision impairment or vision loss because
of the direct damage it causes to visual structures or disruption of their vascularisation.
Metabolic Syndromes
The spreading of these tumours in parts of the brain that ensure hormone regulation causes
risks to a child’s health, especially when it comes to their metabolism. The pressure caused by
the tumour can lead to endocrine and metabolic consequences. Diabetes insipidus, a condition
characterized by large amounts of dilute urine and increased thirst, may take effect on a child
with craniopharyngioma when not treated properly. The tumour may damage the hypothalamus,
a part of the brain that is able to control one’s thirst. Thus, causing DI. Further, there is a
deficiency in ADH, which usually helps the kidneys retain water better. Craniopharyngiom
disrupts ADH production, which leads to an inappropriate phase of ADH secretion. Obesity,
especially in a youth with craniopharyngioma, is prevalent. As for hormone regulation in body
weight, patients in a study by Chakrabarti et al. show higher serum leptin levels as compared to
BMI-matched healthy controls. O’Gorman et al. also found a delay in ghrelin response after an
oral glucose tolerance test.
Conclusion
Craniopharyngioma is a rare, benign tumour that, despite not being cancerous, can cause
long-lasting effects due to its proximity to vital brain structures such as the pituitary gland, optic
nerves, and the hypothalamus. Treatment options are complicated, often involving complex
surgery to remove the tumour without damaging brain functions, and there’s always a risk of
recurrence. Pediatric craniopharyngioma specifically needs even more attention as patients are
affected by symptoms like hormonal imbalances during crucial developmental periods and
therefore often need lifelong medical support.
Overall, our research shows the necessity of early detection and commitment to long-term
treatment plans to improve the living conditions of those affected. Real-world application of our
findings lies in raising awareness of this rare condition and providing information that may lead
to the development of safer and more direct treatment options. Future research should continue
to make efforts in refining surgical practices, tackling recurrence, and improving the quality of
living for all those affected, especially pediatric patients.
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