Craniopharyngioma is a rare brain tumor that is usually found near the pituitary gland, a small gland situated at the base of the brain. The tumors usually manifest in the sellar and parasellar regions, the parts of the brain where the pituitary gland is located. Craniopharyngiomas are usually benign; they are not cancerous, but they can cause serious health problems in the brain and the pressure they apply to the surrounding structures. 

The specialty of craniopharyngiomas is that they histologically resemble the tissues found in the developing embryo’s or fetus’s nasopharynx (upper part of the throat) and nearby structures. This is the reason for naming this tumor a craniopharyngioma.  

Craniopharyngiomas estimated incidence is 0.5 to 2 cases per million people every year. 

Age: Craniopharyngioma has a bimodal age distribution. The first peak is experienced by children between the ages of 5 and 14, and the second peak is experienced by adults between the ages of 50 and 74. 

Embryonic Origin: Craniopharyngiomas are believed to be formed from the remains of Rathke’s pouch, which is a part of the embryonic structure that is responsible for the formation of the anterior pituitary gland. These are transformed into tumors during the later stages of life. 

Location: Craniopharyngiomas mostly occur within or close to the sella turcica, the bony structure at the base of the skull where the pituitary gland is located.  

Compression and Displacement: When these craniopharyngiomas become larger, they can press and interfere with the nearby structures. The pressure on the optic nerves, hormonal imbalances due to the pituitary gland, and neurological deficits due to the pressure on the adjacent brain tissue are some of the symptoms of the compression that can come from the tumor. 

Cyst Formation: Craniopharyngiomas have solid components, and the cystic ones usually worsen the symptoms. These cysts can expand the tumor size and at the same time, exert more pressure on the adjacent structures. 

Hormonal Dysfunction: Craniopharyngiomas can be the cause of the disturbance of the normal functioning of the pituitary gland, and thus, the hormonal imbalances occur. The extent and the location of the tumor will determine the type of hormone deficiencies a person will have.  

Craniopharyngiomas are of embryonic tissue that is near the pituitary gland that undergone abnormal development. There are two main types with distinct origins: 

Adamantinomatous craniopharyngioma: This type, most of the time found in children, grows from the remains of the craniopharyngeal duct, a structure that is connected to the pituitary gland during the embryonic development. The exact cause for their formation is still unknown, but studies have discovered the presence of mutations in the CTNNB1 or APC genes in more than 70% of these tumors. The genes are involved in the embryonic development, and mutations may cause abnormal cell growth. 

Papillary craniopharyngioma: The latter, which is more common in adults, is produced by a transformation of the cell type (metaplasia) of the anterior pituitary gland itself. Squamous cells that are usually seen on the skin surface, are found inside the pituitary gland. 

The prognosis for craniopharyngioma patients can be different from one to another depending on the variables like the size, location and the age and health of the patient. 

Age group: 
Craniopharyngiomas typically occur in two age groups: Children and adults. In children, they are usually diagnosed between the ages of 5 and 14, whereas in adults, they are typically diagnosed between the ages of 40 and 60. Nevertheless, they can be present at any age. 

• Visual assessment 
• Endocrine assessment 
• Imaging studies 
• Cognitive Assessment 

• Hypopituitarism 
• Hydrocephalus 
• Cognitive Impairment 
• Hypothalamic Dysfunction 
• Diabetes Insipidus 
• Endocrine Dysfunction 

Acute Presentation: Occasionally, craniopharyngiomas can cause acute symptoms because of the sudden increase of intracranial pressure (ICP) from the obstruction of cerebrospinal fluid (CSF) flow or the haemorrhage into the tumor. The primary acute symptoms which can be observed are the severe headache, vomiting, altered consciousness, and neurological deficits. 

Subacute Presentation: Some of the patients may have subacute symptoms that come up gradually from days to weeks. These symptoms may consist of headache that are increasingly becoming worse, the visual problems (such as double vision or the blurred vision), the hormonal imbalance (causing the symptoms like the thirst or urination, the weight gain or loss, or the menstrual irregularities. 

Surgery: Surgical procedure of removing the tumor is usually the main treatment. The aim is to get as much of the tumor as possible out of the patient’s body while preserving neural function. Nevertheless, total surgical removal of the tumor is difficult because of the tumor’s location near the vital structures in the brain. 

Radiation Therapy: In some situations when the surgical removal of the tumor is not possible or when the tumor again arises, radiation treatment may be suggested. Radiation may be helpful in the fight against tumor growth and the relief of symptoms. 

Combination Therapy: Occasionally, the combination of surgery and radiation therapy is carried out to get the highest possible outcome. This method can be represented as a surgery to take out as much of the tumor as it is feasible, after that the radiation therapy would be used to destroy the remaining tumor cells. 

Medical Management:  Sometimes, the surgery or radiation therapy is not possible or when the tumor is not growing rapid and is not making any symptoms, some medications like antineoplastics agent can be prescribed. 

Neurology

Vision Support: If the tumor or the treatment of the tumor has caused vision impairment, in that case the lighting in the home should be adjusted to reduce the glare and shadows, the sliding doors should be removed, and the pathways should be clear. Handrails and grab bars are the ones that offer the stability. 

Temperature Control: The hypothalamus is the organ that regulates the body temperature, and, therefore, the home temperature should be kept as comfortable as possible. Probably, this can be done by using fans, air conditioning, or heating systems, which will be used according to the needs of the time. 

Cognitive Support: The variations in the cognitive disabilities may lead to some difficult tasks. Therefore, the modifications might be the reduction of distractions in the environment, the use of memory aids such as whiteboards or digital reminders, and the splitting of tasks into smaller, more manageable steps. 

Nutritional Support: Hormonal imbalances can even lead to changes in the way a person eats and the way their body processes food. The accessibility to the right foods and the regularity of the eating schedule are the pillars that support the treatment of the obesity problem. 

Neurology

A class of chemotherapy drugs that might be taken in the cure of craniopharyngioma is antineoplastic agents. 

The chemotherapy drugs used for craniopharyngioma can be differ due to the factors like the size, location, and the patient’s overall health. 

Neurology

Transcranial surgery (craniotomy): This conventional procedure involves making a craniotomy (opening of the skull) to reach the tumor. Thus, the surgeon can see the tumor directly and then remove it. Although craniotomy allows one to see the tumor very well, it is a more intrusive surgery that requires a long recovery time and a higher chance of complications. 

Endoscopic endonasal transsphenoidal surgery (EET): This range of minimal invasive procedure that is easy to access the tumor through the nose as well as the sphenoid sinus. The surgeon uses the endoscope to see the tumor and to remove it. EET is a less traumatic method that has a quicker recovery time than craniotomy. Yet, it can be applied only to some tumors, especially the larger ones and the ones outside the sellar region. 

Neurology

Diagnosis: 

Symptoms: Among the various symptoms of the craniopharyngioma are headaches, visual problems, and hormonal imbalances. 

Neuroimaging: MRIs or CT scan show the tumor’s location and the effect it has. 

Endocrine evaluation: The hormone levels are evaluated to check for the disruption of the pituitary gland. 

Treatment Planning: 

Multi-disciplinary consultation: The practitioners are working as a team to draw up the right plan for the person. 

Tumor characteristics: The factors that shape treatment decisions are the size, location, and the patient’s ease or difficulty of treatment. 

Surgical Management: 

Transsphenoidal surgery: The method of tumor removal by entering the nasal passage. 

Craniotomy: Any intrusion on the skull for the hard to access tumors. 

Adjuvant Therapy: 

Radiation: Third line treatment targets the remaining tumor cells that were not removed by surgery or are the initial treatment. 

The Radiotherapy, for the recurring tumors, is combined with radiation, and, hence, the physicians recommend this therapy for the recurring tumors because it is the radiation. 

Long-Term Follow-Up: 

Periodic imaging: MRI or CT scans are the methods that determine the existence of tumor recurrence or growth. 

Endocrine surveillance: The controlling of the hormonal level on a periodical basis and the therapy modification are the primary step that should be done.