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Craniopharyngioma: The Most Formidable of Intracranial Tumors

Almost 80 years ago, the father of modern neurosurgery, Dr. Harvey Cushing, declared craniopharyngiomas “the most formidable of intracranial tumors.” Back in 1939, before the use of the surgical microscope, the endoscope, modern anesthetic techniques and hormone replacement therapies, Cushing learned that surgery for these tumors was extremely challenging and risky. Fast forward to the present, and Cushing’s characterization of these tumors remains accurate, however, our comprehensive team approach for patients with craniopharyngiomas has changed dramatically and overall outcomes are much improved.

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Articles of Interest

Living With Craniopharyngioma

Being diagnosed with a brain tumor can be terrifying. Not knowing what to expect or what life will look like in the coming days is one of the scariest experiences for patients and their families. A craniopharyngioma diagnosis can evoke the same emotions but is perhaps more fearsome due to the extreme rarity of the condition.

All About Craniopharyngioma

Surgery is the most common treatment used. The goal is to surgically remove the whole tumor but this is not always possible. If the tumor has grown into a major blood vessel or the optic nerve, or if the surgery itself could damage the brain and the child's functioning, then other treatments may be used.

Approach to the Patient: New Era Emerges for Craniopharyngioma Management

Papillary craniopharyngioma (PCP) and adamantinomatous craniopharyngioma (ACP) are distinct, slow-growing tumors of the suprasellar region. Their location, composition, and biology have historically evaded successful surgical radiation and medical therapy. Meanwhile compromise of critical structures either by tumor or treatments increase morbidity, impacting patient and carer quality of life.

Craniopharyngioma

Craniopharyngiomas are rare malformational tumours of low histological malignancy arising along the craniopharyngeal duct. The two histological subtypes, adamantinomatous craniopharyngioma (ACP) and papillary craniopharyngioma (PCP), differ in genesis and age distribution. ACPs are diagnosed with a bimodal peak of incidence (5-15 years and 45-60 years), whereas PCPs are restricted to adults mainly in the fifth and sixth decades of life.

Craniopharyngioma in Adults: What patients should know about

Craniopharyngiomas are relatively rare, with an incidence rate of approximately 0.5 to 2 cases per million people annually. They account for about 1-3% of all brain tumors in children and 1-2% of all primary intracranial neoplasms in adults. These tumors exhibit a bimodal age distribution, primarily affecting children aged 5-14 years and adults aged 50-74 years. There is no significant gender or racial bias in the occurrence of craniopharyngiomas.

Craniopharyngioma: A comprehensive review of the clinical presentation, radiological findings, management, and future Perspective

Craniopharyngioma (CP) is a rare intracranial tumor arising from the epithelial remnants of Rathke's pouch, most frequently originating in the sellar/parasellar region. Histologically, CP is a benign low-grade tumor (WHO grade 1) with two distinct phenotypes...

Status Quo and Research Trends of Craniopharyngioma Research: A 10-Year Bibliometric Analyses (From 2011 to 2020)

Different from other medical fields, CP is one of the most complex tumors could be analyzed by automatic algorithm. Although CP is a pathologically benign tumor and its histological grade is WHO I, it often affects the prognosis and outcome of patients because CP occurs in the special location of the hypothalamus-pituitary axis. There is no fixed treatment plan that is effective for this kind of pathology. Therefore, each patient with CP should be evaluated and treated separately.

Resources

American Association of Neurological Surgeons

Craniopharyngiomas are histological benign, but locally aggressive, tumors that develop near the pituitary gland at the base of the brain.

Brain Tumour Research

There are three different types of craniopharyngioma: adamantinomatous, papillary, and a mixed or transitional subtype that usually behaves like an adamantinomatous tumour, but actually contains a mixture of both forms of this tumour. Craniopharyngiomas can be diagnosed in both adults and children, usually forming at the base of the brain near the pituitary gland. They can therefore have a large impact on hormone levels...

Endocrine Reviews

It is crucial that craniopharyngioma be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients' clinical and quality of life consequences by experienced multidisciplinary teams.

Endocrine Society

Although patient survival is high, the consequences of the tumor and its treatment can lead to severe comorbidities resulting in poor quality of life, in particular for those patients who bear tumors with hypothalamic involvement. Accordingly, in these patients at risk for the development of a hypothalamic syndrome, hypothalamus-sparing treatment strategies such as limited resection followed by irradiation are recommended. In this review, we provide an update on various aspects of CP, with emphasis on recent advances in the understanding of tumor pathogenesis, clinical consequences, management, and therapies.

NORD

There are two types of craniopharyngioma: adamantinomatous and papillary. The first type most commonly occurs in children, while the second is more common in adults. Adamantinomatous craniopharyngiomas arise from cells from an embryologic structure known as the craniopharyngeal duct.

StatPearls

Craniopharyngioma is a rare, virtually benign tumor of the central nervous system (CNS). It is a partly cystic embryonic malformation that can occur in the sellar/parasellar region and can produce a wide array of symptomatology such as headaches, nausea and vomiting, visual disturbances, and endocrine disturbances. It represents a special challenge for the clinicians that treat it; these clinicians commonly include neurosurgery, neuro-ophthalmology, neurology, endocrinology, and pediatrics. The challenge is due to the tumor's ability to adhere to the surfaces that surround it. For this reason, it is extremely difficult to control, and it is also notorious for its high rates of recurrence.

The Royal Marsden

Craniopharyngiomas result from the growth of cells that, early in foetal development, have failed to migrate to their usual area. Craniopharyngiomas are benign tumours that do not spread; they can be solid, cystic (full of fluid), calcified or full of debris. They are slow-growing tumours that can take two to three years or longer to manifest themselves before a diagnosis is made. They can cause serious problems by interfering with important structures near them. Craniopharyngiomas represent 5–10% of childhood brain tumours, with about 30 cases a year in children in the UK. They also occur in adults. There is no known cause.

Vikas Rao MD

Treatment for a craniopharyngioma usually involves surgery to remove the tumor and has a very high rate of success. Surgery may be performed with or without radiation therapy to ensure complete removal. The most difficult part of treating a craniopharyngioma is weighing the necessity for maximal removal to avoid recurrence against minimal invasion to reduce side effects. Some tumors may be treated through stereotactic radiosurgery alone, which is considerably less invasive and associated with fewer risks.

You and Your Hormones

Craniopharyngiomas are rare, slow-growing benign brain tumours which most commonly occur above the pituitary gland, but occasionally are found within the bony cup containing the pituitary gland.