Adamantinomatous Craniopharyngioma - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Adamantinomatous craniopharyngiomas are an abnormally developing mass of cells that arise from the brain tissue. Brain tumors are broadly classed as benign non-cancerous, or malignant. Malignant tumors can spread to other parts of the body. The World Health Organization categorizes central nervous system cancers by grade and histological subtype. A pathologist determines a tumor subtype by studying a tissue biopsy of the tumor under a microscope. This WHO categorization system is used by doctors worldwide to diagnose and treat various malignancies.

What Is Adamantinomatous Craniopharyngioma?

Adamantinomatous craniopharyngioma is a histological subtype of craniopharyngioma, a generally benign and slow-growing tumor that develops near the base of the brain from the sellar and suprasellar area. There are two forms of craniopharyngiomas:

1. Adamantinomatous craniopharyngioma.

2. Papillary craniopharyngioma.

Both craniopharyngioma types are classified as grade I brain tumors (low-grade tumors). Grade I tumors are the least dangerous type of tumor. They develop slowly and are not known to penetrate adjacent tissues. As a result, they have the most excellent prognosis of all brain tumors, with long-term survival reported since surgery is highly likely to be curative.

What Is the Occurrence of Adamantinomatous Craniopharyngioma?

Most adamantinomatous craniopharyngiomas are identified in children. However, they can also afflict middle-aged and older individuals. Even though these slow-growing tumors are uncommon, adamantinomatous craniopharyngioma is the most prevalent histological type, accounting for 86 % to 89 % of all craniopharyngiomas.

What Is the Epidemiology of Adamantinomatous Craniopharyngioma?

• Craniopharyngiomas are uncommon tumors, accounting for fewer than five percent of all primary intracranial tumors (brain tumors).

• Adamantinomatous craniopharyngiomas can afflict people of various ages.

• Children aged five to ten years – most cases are found in this age range.

• Males and females are also afflicted, and no gender preference is shown.

• Individuals of various races and ethnicities may be impacted worldwide. For example, Africans and Far East Asians had more excellent tumor rates.

What Is the Etiology of Adamantinomatous Craniopharyngioma?

The etiology of adamantinomatous craniopharyngioma is unknown. It is an epithelial neoplasm similar to ameloblastoma or an odontogenic cyst (type of jaw tumor). Aberrant development of embryonic tissue during early pregnancy is thought to be the cause. Brain tumors can develop due to environmental, genetic, and lifestyle factors. Specific congenital abnormalities, viral infections, ionizing radiation exposure, and head trauma may all be risk factors.

What Are the Symptoms of Adamantinomatous Craniopharyngioma?

Adamantinomatous craniopharyngioma symptoms are often related to hormonal shortages in the body. Usually, the tumor obstructs the flow of cerebrospinal fluid, resulting in hydrocephalus. The cancer is also linked to eye problems and neurological issues such as memory loss and personality changes. The signs and symptoms of adamantinomatous craniopharyngioma are determined mainly by the tumor's location and size. Most tumors are significant at the diagnosis, measuring more than five centimeters in diameter. Many are in the suprasellar area, whereas others are in the sellar region. They can also be found intranasally (in the nasopharynx), in the sphenoid sinus, at the cerebellopontine angle, and next to the pineal gland. Severe problems may occur during or after therapy. Some of the common symptoms are as follows:

• Headaches.

• Seizures.

• Muscular weakness

• Dizziness.

• Cold intolerance.

• Endocrine disorders.

• Behavior changes; personality changes.

• Blurred vision.

• Muscle weakness.

• Feelings of numbness and tingling.

• Speech issues.

• Hearing loss or hearing impairment.

• Unsteadiness and loss of balance; lack of coordination (vertigo).

• Tremors.

• Confusion.

Which Medical Conditions Are Associated With the Pressure Exerted by Craniopharyngioma on the Pituitary Gland?

The pituitary gland regulates numerous hormonal functions. The pressure exerted by craniopharyngioma may lead to the following conditions:

• Gonadotropin deficiency.

• Growth hormone deficiency.

• Thyroid stimulating hormone deficiency.

• Central diabetes insipidus.

• Adrenocorticotropic hormone deficiency.

How Is Adamantinomatous Craniopharyngioma Diagnosed?

The following tests and exams may be used to diagnose adamantinomatous craniopharyngiomas:

• A complete physical examination and medical history.

• Evaluation of the presenting signs and symptoms.

• Blood tests detect hormonal imbalances and electrolytes in the blood.

• Visual field exams are used to determine visual acuity.

• Neurological, motor skill, and cognitive evaluation.

• Intelligence, memory, linguistic abilities, mental health and function, judgment and reasoning, coordination and balance, reflexes, and sensory perceptions are tested.

• An X-ray of the head and neck.

• Head and neck computed tomography (CT) scan.

• Cerebral angiographic studies or MR (magnetic resonance) angiography.

• Magnetic resonance spectroscopy.

• Cerebrospinal fluid analysis.

• Molecular analysis.

What Is the Treatment of Adamantinomatous Craniopharyngioma?

Treatment for adamantinomatous craniopharyngioma is complex. It may consist of surgery and radiation therapy. Hormone therapy is an essential component of treatment. In rare cases, removing the tumor might aggravate the signs and symptoms. Treatment options for adamantinomatous craniopharyngioma include:

• Symptomatic therapy may include using anti-seizure drugs, pain relievers, or steroids to decrease inflammation.

• Hormonal supplements are used to address hormonal imbalances.

• Ventriculoperitoneal (VP) shunt placement treats hydrocephalus; the device is implanted in the brain's ventricles, relieving excess CSF pressure on the brain.

• Depending on various considerations, the surgical treatment removes the entire tumor or a portion.

• A craniotomy is a surgical operation in which a small hole in the skull is made to access and remove the tumor. The primary objective of surgery is to remove as many tumors as possible while minimizing damage to adjacent brain tissue.

• In some instances, the tumor is only partially removed to avoid causing brain damage and resulting in unwelcome extra symptoms. However, in slow-growing grades, partial removal is frequently contemplated.

• Microsurgery is carried out utilizing microsurgical methods, which can reduce nerve injury. In addition, because the tumor predominantly affects the covering around the nerves, it can usually be removed entirely without damaging the nerve.

What Is the Prognosis of Adamantinomatous Craniopharyngioma?

The prognosis of adamantinomatous craniopharyngioma is determined by several factors, including the size of the tumor and the individual's general health. Long-term prognosis is often unknown; medical screening, constant monitoring, and follow-up therapies are required for extended periods.

Conclusion

Adamantinomatous craniopharyngioma is a histological subtype of craniopharyngioma, a generally benign and slow-growing tumor that develops near the base of the brain from the sellar and suprasellar area. Craniopharyngioma lesions are thought to have started in the suprasellar regions of the brain from the Rathke pouch epithelium above the sella turcica of the skull base. The symptoms of adamantinomatous craniopharyngioma are frequently linked to hormonal imbalances in the body. Thus, diagnosing and treating these tumors as soon as they are detected is necessary.