What are the other Names for this Condition? (Also known as/Synonyms)

• ACP (Adamantinomatous Craniopharyngioma)
• adaCP (Adamantinomatous Craniopharyngioma)
• Adamantinomatous Craniopharyngeal Duct Tumor

What is Adamantinomatous Craniopharyngioma? (Definition/Background Information)

• A craniopharyngioma is a type of brain tumor. A brain tumor may be described as a mass of abnormally growing cells arising from the brain tissue (brain parenchyma and meninges) or the spinal cord (central spine). The brain along-with the spinal cord constitute the central nervous system (CNS) of the body
• Broadly, brain tumors are classified as benign (non-cancerous) and malignant. Malignant tumors are cancerous and can spread or metastasize to other regions of the body. They generally exhibit rapid growth and, in some cases, may present symptoms during the early stages of development
• The World Health Organization (WHO) classifies central nervous system tumors according to their grade and histological subtypes. A tumor subtype is determined by a pathologist after examining a tissue biopsy of the tumor under the microscope. This WHO classification system is used by healthcare professionals all over the world in diagnosing and treating these tumors
• Adamantinomatous Craniopharyngioma (adaCP) is a histological subtype of craniopharyngioma, a relatively benign and slow-growing tumor that arises from the sellar and suprasellar region, at the base of the brain. There are two subtypes of craniopharyngiomas, namely the adamantinomatous type and the papillary type, or papillary craniopharyngioma (papCP). Both the craniopharyngioma variants are classified as WHO grade I brain tumors (low-grade tumors)
• Grade I tumors are the most benign of the tumors. They are slow-growing and are not known to infiltrate into the surrounding tissues. They offer a very high chance for surgery to be curative; and thus, have the best prognosis among all brain tumors with long-term survival being noted. Per WHO, craniopharyngioma forms part of a group of tumors designated as “tumors of the sellar region”
• The sellar region includes the sellar turcica and the pituitary gland. It is located just below the center of the brain and bounded by other vital structures such as the hypothalamus, optic nerve, internal carotid artery, and the brainstem. The pituitary gland is the “master gland” of the body that regulates all the other endocrine glands
• A large majority of Adamantinomatous Craniopharyngiomas are diagnosed in childhood, although middle-aged and older adults are also known to be affected. Even though these slow-growing tumors are rare, adaCP is the more common histological type, constituting between 86% to 89% of all craniopharyngiomas
• The cause of formation of Adamantinomatous Craniopharyngioma is not well-established. It is believed that an abnormal development of the embryonic tissue during early pregnancy may be causative. In general, brain tumors may form from a combination of several factors including genetic, environmental, and lifestyle-related. The risk factors may include the presence of certain genetic disorders, exposure to ionized radiation, viral infections, and head trauma
• Apart from headaches, seizures, muscle weakness, and dizziness, the signs and symptoms of Adamantinomatous Craniopharyngioma are typically associated with hormonal deficiencies in the body. Often, the tumor causes an obstruction of cerebrospinal fluid flow leading to hydrocephalus. The tumor is also associated with vision defects and neurological abnormalities including memory loss and personality changes. Severe complications may develop during/from treatment too
• The treatments for Adamantinomatous Craniopharyngioma is usually challenging but may primarily involve surgery and/or radiation therapy. Hormonal therapy is an important component of treatment, depending on the specific combination of signs and symptoms noted for each individual. Sometimes, removing the tumor can worsen the signs and symptoms in some individuals
• The prognosis of Adamantinomatous Craniopharyngioma depends on a wide variety of factors, including the size of the tumor, and overall health status of the individual. In a majority, the long-term prognosis may be unpredictable; however, continued vigilance, medical screening, and follow-up treatments are mandatory for prolonged periods

Who gets Adamantinomatous Craniopharyngioma? (Age and Sex Distribution)

• Craniopharyngiomas are rare tumors that represent less than 5% of all primary intracranial tumors (brain tumors)
• Individuals of a wide age group may be affected by Adamantinomatous Craniopharyngiomas. A bimodal age distribution with two peaks is noted:
  • Children in the 5 to 10 years’ age group - most cases are noted in this subset
  • Middle-aged and older adults in the 50 to 60 years’ age group - some cases are noted in this subset
• Both males and females are affected, and no gender preference is observed
• Worldwide, individuals of all racial and ethnic groups may be affected. The tumor is noted in higher incidences among Africans and Far East Asians

What are the Risk Factors for Adamantinomatous Craniopharyngioma? (Predisposing Factors)

The risk factors for craniopharyngioma are not well defined. Children and adolescents are at a high risk for the development of Adamantinomatous Craniopharyngioma, which is a form of brain tumor.

In general, the following factors may increase one’s risk for brain tumors:

• Advancing age is an important risk factor; although, some tumors are common among children
• In general, males are at a higher risk for brain tumors than females
• Positive family history: It is reported that certain genetic (hereditary) factors are responsible for the formation of certain brain tumors. Such hereditary conditions include:
  • Li-Fraumeni syndrome
  • Neurofibromatosis types 1 or 2 (NF1 or NF2)
  • Nevoid basal cell carcinoma syndrome
  • Tuberous sclerosis
  • Turcot syndrome
  • Von Hippel-Lindau disease
• Environmental factors such as ionized radiation exposure (both high-dose and low-dose)
• Individuals who have undergone radiation therapy for other cancers involving the head and neck region are at an increased risk
• Viral infections involving pathogens, such as Epstein-Barr virus (EBV) and cytomegalovirus (CMV), have been implicated
• Exposure to certain chemicals, pesticides, or products, either at work or at home
• Some reports indicate that the use of mobile phones may increase one’s risk due to exposure to electromagnetic waves. However, the scientific research community is presently divided on this factor
• Head injuries
• History of seizures
• Hormonal factors: Studies have shown a correlation between hormones and some types of brain tumors
• Intake of N-nitroso compounds, either in one’s diet (such as cured meat) or via vitamin supplements may increase one’s risk

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Adamantinomatous Craniopharyngioma? (Etiology)

The exact cause of development of Adamantinomatous Craniopharyngioma (adaCP) is not well-understood. It is an epithelial neoplasm that resembles an ameloblastoma or odontogenic cyst (types of jaw tumor).

• It is hypothesized that the origin of the tumor is from the epithelial remnants of Rathke’s pouch (a precedent structure to the anterior pituitary during embryogenesis); or, they are considered to be “misplaced odontogenic rests along the pituitary stalk”
• According to research, these tumors are known to be caused by an abnormal development of embryonic cells of the craniopharyngeal duct
• It is believed that craniopharyngioma does not form a part of any hereditary condition (i.e., it is not deemed to be inherited from one’s parents)

adaCP is associated with the following molecular mutations:

• In nearly all cases, gain-of-function mutations (or activating mutations) of the WNT signaling pathway CTNNB1 gene is noted. The CTNNB1 gene is needed for encoding the dual function protein catenin beta-1 (or β-catenin)
• In over 70% of the cases, the tumor exhibits mutations in either the CTNNB1 or APC genes

In general, it is known that tumors form when normal, healthy cells begin transforming into abnormal cells - these cancer cells grow and divide uncontrollably (and lose their ability to die), resulting in the formation of a mass or a tumor.

What are the Signs and Symptoms of Adamantinomatous Craniopharyngioma?

The signs and symptoms of Adamantinomatous Craniopharyngiomas may be significantly different from one individual to another. It may be mild or severe, depending on several factors. The onset of symptoms and speed of progression of the tumor can vary. In many individuals, tumors that grow slowly and remain asymptomatic, are only diagnosed during certain radiological imaging studies of the head and neck region, which are performed for unrelated health conditions. Since the tumors are slow-growing, it is reported that the time from recognition of symptoms to establishing a diagnosis may take a couple of years.

The signs and symptoms of Adamantinomatous Craniopharyngioma primarily depend on the location and size of the tumor. Most of the tumors are large and usually over 5 cm in size at the time of diagnosis. Many occur in the suprasellar region; some are found in the sellar region. They can also be present intranasally (involving the nasopharynx), at the sphenoid sinus, in the region of the cerebellopontine angle (CPA), and near the pineal gland.

Due to obstruction in the flow of cerebrospinal fluid (CSF), increased intracranial pressure is noted in almost all cases. Apart from this, dysfunction of the pituitary gland causing endocrinal associated systemic symptoms, and optic nerve damage causing vision problems may be noted.

A variety of signs and symptoms are observed. Among these, the commonly noted ones include:

• Increased intracranial pressure is observed due to obstruction of the cerebrospinal fluid (CSF) drainage, since the ventricular system is affected. This may result in nausea, vomiting, headaches, and seizures
• Abnormally functioning (or enlarged) pituitary gland can result in hormonal imbalances and lead to:
  • Pituitary insufficiency causing reduced production of hormones such as luteinizing hormone (LH), adrenocorticotropic hormone (ACTH), follicle-stimulating hormone (or FSH, such as gonadotropin), growth hormone (GH), and thyroid-stimulating hormone (TSH)
  • Sleep disturbances; sleep apnea
  • In children and teenagers, delayed onset of puberty and growth failure due to hormonal disturbances
  • Developmental abnormalities 
  • Diabetes insipidus presenting increased thirst and frequency of urination, especially in adults
  • Reduced mental ability
  • Changes in one’s behavior; personality changes
  • Intolerance to cold
• Endocrine abnormalities due to endocrine (hormone) deficiencies can cause severe associated signs and symptoms
• Visual impairment, such as blurred vision, double vision, poor eyesight, visual field defects, and swollen optic nerve (papilledema); visual symptoms are more common in adults than in children

Other associated signs and symptoms may include:

• Persistent headaches
• Numbness and tingling sensation
• Muscle weakness; loss of strength in the arms of legs
• Neck pain
• Hearing impairment or hearing loss
• Speech problems
• Tremors
• Seizures or convulsions
• Lack of coordination; unsteadiness and loss of balance (vertigo)
• Dizziness and fainting
• Confusion
• Memory loss

Large tumors can cause significant signs and symptoms. Most of the cases represent single tumors; but occasionally, multiple tumors can be detected growing simultaneously in the brain or spinal cord region, particularly in individuals with genetic disorders. The associated symptoms of the underlying condition, if any present, may be noted.

How is Adamantinomatous Craniopharyngioma Diagnosed?

Adamantinomatous Craniopharyngiomas may remain undiagnosed for prolonged periods, if they are asymptomatic and slow-growing. The slow development of symptoms may contribute to a delayed detection and diagnosis of these tumors. The tumors are detected when there is a sudden worsening of symptoms prompting the healthcare provider to perform radiological studies of the brain.

The diagnosis of Adamantinomatous Craniopharyngiomas may involve the following tests and examinations:

• Complete physical examination and a thorough medical history
• Assessment of the presenting signs and symptoms
• Blood tests to assess hormonal imbalances and serum electrolytes
• Visual field tests; tests to assess visual acuity
• Neurological, motor skills, and cognitive assessment:
  • Checking intellectual ability, memory, mental health and function, language skills, judgment and reasoning, coordination and balance, reflexes, sensory perceptions (space, sight, hearing, touch)
  • The healthcare provider/neurologist may use the Karnofsky Performance Scale in order to assess the neurological functioning of the individual’s central nervous system (CNS)
• Electroencephalography (EEG)
• Electromyography with nerve conductivity tests
• Imaging studies that may be performed include:
  • X-ray of the head and neck region
  • Computerized tomography (CT) scan of the head and neck region; CT scan with contrast agent
  • Magnetic resonance imaging (MRI) scan of the central nervous system; MRI with contrast agents such as gadolinium
  • Cerebral angiographic studies or MR angiography: An angiogram involves injecting dye into the bloodstream, which makes the blood vessels to appear visually on X-rays. The X-ray may show a tumor in the brain or the blood vessels leading into the tumor
  • Magnetic resonance spectroscopy: This radiological technique is used to study the chemical profile of the tumor. It is often performed with and compared to corresponding MRI scan images of the affected region
  • Positron emission tomography (PET scan): A PET scan is a nuclear medicine imaging technique that uses three-dimensional images to show how tissue and organs are functioning. It may be performed to detect any malignancy, and if there is a metastasis (spread) of the tumor to other regions
• Cerebrospinal fluid analysis, where a spinal tap or lumbar puncture procedure may be performed: This diagnostic test is used to remove a sample of cerebrospinal fluid (CSF) from the spaces in and around the brain and spinal cord. The sample is removed from the lower spinal cord using a thin needle, and it is then checked for the presence of cancer cells
• Molecular testing to identify any genetic mutations or abnormalities

Tissue biopsy: A biopsy of the affected region (brain or spinal cord) is performed and sent to a laboratory for a pathological examination. A pathologist examines the biopsy sample under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis. Examination of the biopsy sample under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis.

There are two ways to perform a biopsy:

• First, a biopsy can be performed as part of a surgical procedure to remove the brain tumor
• Second, a stereotactic needle biopsy can be performed when the tumor is deep within the brain or located in a sensitive area. In this procedure, the surgeon drills a small hole (called a burr hole) into the skull. A thin needle, guided by CT or MRI scanning, is then inserted through the hole to help in the removal of tissue

A differential diagnosis to eliminate other conditions or tumor types may be considered, before arriving at a definitive diagnosis. This may include:

• Epidermoid cyst of brain
• Metastatic tumors to the brain
• Papillary craniopharyngioma
• Pilocytic astrocytoma
• Rathke’s cleft cyst
• Xanthogranuloma, originating in the sellar region

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Adamantinomatous Craniopharyngioma?

The possible complications associated with Adamantinomatous Craniopharyngioma include:

• Emotional and mental stress for both the patient and the caretakers, due to the diagnosis and during treatment of a brain tumor
• Hydrocephalus: When the tumor obstructs the free flow of cerebrospinal fluid in the brain, it can cause a condition known as hydrocephalus. This can result in enlarged head in babies, lethargy, irritability, nausea and vomiting, blurring of vision, loss of balance, seizures, urinary incontinence, and growth delays, among other symptoms
• Imbalance of hormones in the body due to hypothalamic-pituitary dysfunction can lead to complications such as:
  • Generalized weakness and fatigue
  • Stunted growth in children; short stature
  • Weak bones causing easy fractures
  • Hypothyroidism resulting in dry skin, weight gain, tiredness, hoarse voice, and increased cold sensitivity
  • Central diabetes insipidus, from pituitary gland dysfunction
  • Adrenal failure that may result in low blood sugar, low blood pressure, confusion, and even cardiac arrhythmias
  • Precocious puberty or early-onset puberty
  • Impotence and reduced sex drive in men
  • Amenorrhea in women (absence of menstruation); menstrual irregularities
• Disrupted blood (oxygen) supply to the brain causing cerebral ischemia
• Recurrent infections in children and adults
• Optic atrophy resulting in partial or complete blindness (usually unilateral)
• Hypothalamic obesity is a significant issue in children; uncontrolled weight gain occurs due to damage to the hypothalamus
• Dysfunction of the hypothalamus can also present sleep disorders, abnormal heart rate and blood pressure, and body temperature variations
• Severe intellectual disability; dementia including personality changes causing a reduced quality of life
• In some cases, the condition can result in a coma
• Severely affected physical and mental health may lead to the overall quality of life being greatly reduced
• Large undetected tumors can severely affect brain function and be disabling or even life-threatening, due to mass effect/compression of surrounding tissue
• In the brain, large tumors may also press against the skull (or other) bones causing it to expand, causing severe complications
• Chemical meningitis from rupture of the tumor, in rare cases
• Peritumoral brain edema (PTBE): Presence of fluid around the tumor region in the brain resulting in severe symptoms and complications
• In rare instances, the “mostly benign” tumor can transform into a malignancy. A malignant Adamantinomatous Craniopharyngioma may take several years to develop. Such malignant transformations have been reported in both children and adults. Due to the increased instances of radiation therapy following frequent tumor recurrences, the risk for radiation-induced malignant transformation is reportedly high
• Complications due to an underlying genetic disorder, if any present

Complications may arise from surgery, radiation therapy, or other treatment modalities.

Surgical complications:

• Damage to the muscles, vital nerves, and blood vessels, during surgery to remove the tumor; injury to unaffected brain tissue during surgery
• Post-surgical infection at the wound site is a potential complication
• In many cases, the symptoms are known to significantly worsen following surgical removal of the tumor and become irreversible
• Recurrence of the tumor after surgery may be observed; malignant tumors often have very high recurrence rates. Local recurrences following (partial) surgery is generally high with Adamantinomatous Craniopharyngioma. The rate of recurrence of the adamantinomatous type is higher than that of the papillary type

It is important to note that tumor recurrences are reported even when the tumor is “apparently” completely removed via invasive procedures.

Chemotherapy side effects depend on the type of chemotherapy medication used and may include:

• Nausea and vomiting
• Hair loss
• Mouth sores
• Loss of appetite
• Diarrhea
• Fatigue
• Increased risk for infections
• Easy bruising

Radiation therapy can cause short-term side effects. It depends on the type used and may include:

• Nausea and vomiting
• Hair loss
• Dryness of mouth; loss of taste
• Headaches
• Fatigue
• Skin color changes; usually darkening of skin on the face and neck may be noted
• Speech and hearing difficulties
• Memory issues
• Seizures

Long-term side effects of radiation therapy (seen after 6 months or beyond) may include loss of memory, impaired brain function, and appearance of symptoms that mimic stroke.

How is Adamantinomatous Craniopharyngioma Treated?

The treatment modality for craniopharyngioma is chosen, depending on the size, location, and stage of the tumor, tumor growth rate, age and health status of the individual. Often, a multidisciplinary team of specialists including ophthalmologists, endocrinologists, neurologists, oncologists, surgeons, radiation therapy experts, and other healthcare professionals are involved in managing the condition.

The treatment of Adamantinomatous Craniopharyngioma is often difficult and challenging. In many cases, removal of the tumor offers a lot of surgical challenges to the healthcare providers, due to the complexity of the tumor site involved. The most important consideration is the anatomical location of the tumor and involvement of nearby vital brain structures, such as the pituitary gland, hypothalamus, optic nerve pathway, and the internal carotid artery. It is often difficult to completely resect the tumor.

The treatment measures for Adamantinomatous Craniopharyngioma may include:

• Symptomatic treatment may involve the use of antiseizure medications, painkillers, steroids (to reduce inflammation), and administration of anti-depressants
• Hormonal disturbances are treated using hormonal supplements
• Placement of ventriculoperitoneal (VP) shunt: It is an invasive procedure that involves the use of a medical device called a VP shunt. The device is placed in the ventricles of the brain to treat hydrocephalus, and thus helps relieve excess CSF pressure on the brain

Surgical treatment: Surgery to remove the entire tumor or just part of the tumor depends on several factors including the risk assessment by a neurosurgeon, with respect to the potential injury to nearby brain tissues. It is also considered based on the location, size, and overall health of the individual. A complete removal of the tumor can result in a cure in many patients.

• Craniotomy: It is a surgical procedure wherein a tiny hole is created in the cranium to access and remove the tumor. The main goal of surgery is to remove as much tumor as possible without damaging the surrounding brain tissue
• In some individuals, the tumor is partially removed, so as not to cause brain damage, resulting in unwanted additional symptoms. A partial removal is usually considered in slow-growing grade I benign tumors
• Microsurgery: The surgery is performed using microsurgical techniques, which can help minimize damage to the accompanying nerve. Since the tumor mostly affects the covering around the nerves, it can be completely removed without affecting the nerve in most cases. The following techniques may be employed:
  • Subtotal removal; when the tumor is incompletely removed
  • Near total removal; when most of the tumor is removed and there is very little tumor remnants
  • Total tumor removal; when the tumor is removed completely
• In some cases, embolization of the tumor may be performed, in order to shrink the tumor size, before major invasive procedures are undertaken. In embolization, the blood supply to the tumor is cut-off, resulting in its shrinkage. This also helps reduce blood loss during a surgical resection
• Radiosurgery for individuals who cannot undergo surgery or have recurrent tumors
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals

Surgical procedures to remove brain tumors carry risks, including infection, possible damage to healthy brain tissue, swelling, or possible fluid build-up in the brain.

Radiation therapy: There are a variety of radiation therapy methods that can be used to treat the tumors. Radiation therapy is performed either after removal of the tumor (to destroy any remaining meningioma cells), or in individuals where surgical removal procedure is not an option. Radiation therapy is helpful to reduce the rate of recurrence. It is also recommended for individuals where the tumor is removed partially.

The types of radiation therapy include:

• Standard external radiation beam therapy using a beam of high-energy X-rays; it is the most common form of radiation therapy used to treat cancers
• 3-dimensional conformal radiation therapy, where 3-d images of the tumor are created using radiological imaging scans to help design appropriate radiation therapy beam size and angles
• Intensity modulated radiotherapy (IMRT): It is a procedure that uses computer-controlled linear accelerators to deliver precise radiation to a malignant tumor
• Stereotactic radiosurgery using Gamma Knife or CyberKnife technique
• Proton radiation therapy: It is a form of external beam therapy using high-energy proton beams to destroy the tumor cells
• Fractionated radiation therapy: It is a good option for individuals whose tumors are too large for radiosurgery, or if the tumor is in a sensitive location. In this therapy, small doses of radiation are administered for a certain set period

Some individuals may experience a few side effects, while others may experience none. These may include fatigue, headaches, hair loss, and scalp irritation. Radiation therapy beams may affect the pituitary gland causing it to dysfunction (bringing about hormonal changes in the body).

Chemotherapy: The response of the tumor to chemotherapy is an important consideration while using this treatment modality. Chemotherapy may not be recommended as a treatment option for treating some tumors. It may be recommended for malignant craniopharyngiomas.

• Chemotherapy may be beneficial in individuals diagnosed with higher grade tumors. Whether to use chemotherapy as a treatment modality is determined by healthcare provider on case-by-case basis
• Chemotherapy may be administered for those tumors that cannot be effectively treated by surgery or radiation therapy. The administration of some drugs may slow the rate of growth of the tumor

The following chemotherapy agents and methods have been used in higher grade tumors with varying beneficial results:

• Immunotherapy to stimulate an affected individual’s immune system
• Somatostatin analogs which prevent the release of growth hormones
• Hydroxyurea, epidermal growth factor receptor inhibitors, platelet-derived growth factor receptor inhibitors, and vascular endothelial growth factor inhibitors

There are side effects for chemotherapy, which depends upon the type and dose of drugs administered. The common chemotherapy side effects include nausea, vomiting, hair loss, loss of appetite, headache, fever, chills, and weakness.

Clinical trials: In advanced stages of tumor progression, there may be some newer treatment options, currently on clinical trials, which can be considered for some patients depending on their respective risk factors.

Regular observation and periodic checkups to monitor the condition is strongly recommended following treatment. In order to avoid a relapse, or be prepared for a recurrence, the entire diagnosis, treatment process, drugs administered, healthcare recommendations post-treatment, etc. should be well-documented and follow-up measures initiated.

How can Adamantinomatous Craniopharyngioma be Prevented?

Presently, there are no specific methods or guidelines to prevent the formation of Adamantinomatous Craniopharyngioma. However, if it is associated with a genetic disorder, the following points may be considered:

• Genetic counseling and testing: If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Regular health check-ups might help those individuals with a history of the condition in the immediate family, in order to help diagnose the tumor early

In general, the factors that may help reduce the incidence of brain tumor may include:

• Reducing exposure to ionizing radiation
• Avoiding cigarette smoke inhalation
• Weight loss, in case one is obese or overweight; maintaining a good BMI
• Availing early treatment for infections
• Using safety gear including wearing helmets when playing sports or while riding two-wheelers
• Wearing seatbelts while driving automobiles

According to the U.S. Preventive Services Task Force (USPSTF), currently there is no standard testing protocols available for brain tumor.

Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory. Often several years of active vigilance are crucial and necessary.

What is the Prognosis of Adamantinomatous Craniopharyngioma? (Outcomes/Resolutions)

The prognosis of Adamantinomatous Craniopharyngioma may vary considerably from one individual to another and is dependent on a set of factors. Adamantinomatous variant prognosis is much worse than that of the papillary variant.

• In many individuals, the prognosis of craniopharyngioma can be generally favorable with adequate and sustained management. Reports indicate that a greater than 20-year survival period is seen in 90% or more cases, with regular follow-up and prolonged treatments. Generally, the prognosis is better in children and young adults when compared to middle-aged and older adults
• Post-surgical complications can often be severe and seen in a large number of cases - it may vary from injury to critical brain structures, worsening of hormone imbalance associated symptoms and neurological issues, including reduced vision, all with a potential to severely disrupt one’s quality of life
• Tumor and treatment complications can result in deaths, usually from the heart, lung, and/or the brain being severely affected, or from hormonal deficiencies. Also, in majority, there is a permanent burden of post-operative deficits
• In many individuals, several surgical procedures may be necessary during their entire lifetime particularly due to local recurrences. The recurrences are higher in individuals with Adamantinomatous Craniopharyngioma than with papillary craniopharyngioma

In rare cases of malignant transformations to high-grade tumors, the prognosis is adversely affected further and results in unfavorable prognoses.

In general, the prognosis may depend upon several factors, which include: 

• Tumor histology or grade of the tumor: The lower the grade, the better is the prognosis
• Age of the individual: Older individuals generally have poorer prognosis than younger individuals
• Overall health of the individual: Individuals with overall excellent health have better prognosis compared with those with poor health
• The size of the tumor: Individuals with small-sized tumors fare better than those with large-sized tumors
• Location of the tumor is an important prognostic factor: Tumors at certain sites involving the central nervous system are more difficult to surgically excise and remove. They can also cause considerable neurological damage at certain sites in the brain and/or spinal cord. Tumors located near vital structures of the brain have poor prognosis
• Tumors that are slow-growing have better prognosis than rapidly-growing tumors
• Presence of certain genetic abnormalities may result in a rapid progression of tumor growth and development
• Sporadic tumors have generally better prognosis than syndromic tumors; tumors in a background of genetic disorders/syndromes may have additional complications
• The ease of accessibility and surgical resectability of the tumor (meaning, if the primary tumor can be removed completely). This is an important prognostic factor
• Extent of residual tumor in the body following a surgical resection: A complete surgical excision with entire tumor removal presents the best outcome. In some cases, large portions of the tumor may be removed; or, only a small portion for tissue biopsy
• Individuals with bulky disease may have a poorer prognosis
• Involvement of other vital organs may complicate the condition
• Whether the tumor is occurring for the first time or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur 
• Response to treatment and absence of post-operative complications: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment
• Functional neurologic status based on neurological evaluation of the patient: A higher score on the Karnofsky Performance Scale may indicate a more favorable outcome

An early diagnosis and prompt treatment of the tumor generally yields better outcomes than a late diagnosis and delayed treatment.

Additional and Relevant Useful Information for Adamantinomatous Craniopharyngioma:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/cancer/