Craniopharyngiomas Overview

Craniopharyngiomas are a type of noncancerous (benign) brain tumor that develops near the pituitary gland. This pea-sized organ is located behind the nose, near the base of the brain, and is responsible for producing, storing and releasing hormones that help monitor and regulate various bodily functions. In addition to the pituitary gland, craniopharyngiomas can affect the hypothalamus (which manages hormones and controls the autonomic nervous system) and the cranial nerves (which transmit signals between the brain, face, neck and torso).

Craniopharyngiomas are very rare, with the National Organization for Rare Disorders (NORD) reporting that they develop in approximately 0.5 to 2 per million individuals each year. When compared to other types of tumors, craniopharyngiomas are relatively slow-growing. However, they have the potential to grow very large, sometimes extending to both sides of the brain.

Together with Tampa General Hospital’s Ear, Nose and Throat (ENT) Institute, our Pituitary Tumor Center—part of our renowned Neuroscience Institute—offers treatment for craniopharyngiomas.

Craniopharyngioma vs. Pituitary Adenoma

Craniopharyngiomas and pituitary adenomas are similar in that they’re both noncancerous tumors that can affect hormone production. But while craniopharyngiomas are located near the pituitary gland, pituitary adenomas develop within the gland. Notably, although craniopharyngiomas are considered to be slow-growing, they still tend to be more aggressive than pituitary adenomas.

Causes and Risk Factors of Craniopharyngiomas

Researchers are still working to determine exactly what causes craniopharyngiomas to develop, and as of this time, there are no confirmed risk factors. However, studies suggest that craniopharyngiomas may develop from the cells present in an embryo that help form the pituitary gland. Craniopharyngiomas can occur at any age but most commonly affect children and older adults.

Signs and Symptoms of Craniopharyngiomas

Craniopharyngiomas can produce symptoms when they exert pressure on nearby tissue, and the symptoms present will depend on which structure(s) are being affected:

• Pituitary gland – If a craniopharyngioma compresses the pituitary gland, it can prevent it from producing sufficient amounts of certain hormones, which can in turn impact bodily functions. Depending on which hormone(s) are deficient, a craniopharyngioma can cause fatigue, weakness, increased thirst (polydipsia), increased urination (polyuria), reduced appetite, nausea, vomiting, unexplained weight loss, memory problems, heart issues, low blood pressure (hypotension), increased LDL cholesterol and osteoporosis, as well as absent or irregular menstruation in women. Craniopharyngiomas can also slow children’s growth and delay puberty.
• Hypothalamus – Pressure on the hypothalamus can increase the risk of developing conditions such as Froehlich’s syndrome, hypothalamic obesity and non-24-hour sleep-wake syndrome.
• Optic nerve – When a craniopharyngioma exerts pressure on an optic nerve (a type of cranial nerve), it can cause blurred vision and peripheral vision problems.

Because these symptoms can result from various other conditions, it can sometimes make it difficult to diagnose a craniopharyngioma.

Diagnosing Craniopharyngiomas

If your medical history and symptoms suggest that you might have a craniopharyngioma, your physician may order one or more of the following tests to confirm your diagnosis:

• Biopsy
• Blood testing
• Computed tomography (CT) scan
• Magnetic resonance imaging (MRI) scan
• Neurological testing (including vision, hearing, balance, coordination, reflex, growth and development tests)
• Urinalysis
• X-ray

Craniopharyngioma Treatment

Craniopharyngiomas are primarily treated with surgery. The goal of surgery is to remove as much of the craniopharyngioma as possible while also minimizing any resulting damage to the adjacent organs, glands and nerves. It may be performed using an open or minimally invasive approach:

• Open craniotomy – The surgeon opens the patient’s skull to remove the tumor.
• Minimally invasive transsphenoidal procedure – The surgeon inserts miniature surgical tools through the patient’s nose to remove the tumor.
• Orbitozygomatic craniotomy – The surgeon makes an incision in the scalp behind the patient’s hairline, removes the bone forming the contour of the orbit and cheek, removes the tumor and then replaces the bone.

In some cases, patients may need to undergo radiation therapy and/or hormone therapy following surgery. Notably, chemotherapy and targeted therapy are sometimes used to treat a rare craniopharyngioma subtype known as papillary craniopharyngioma.

It’s important to note that craniopharyngiomas often return after treatment. Plus, the complications that may result from a craniopharyngioma don’t always resolve once the tumor is removed. As such, the medical community generally considers craniopharyngiomas to be a chronic condition requiring ongoing treatment.

TGH’s Approach to Treating Craniopharyngiomas

If you suspect that you might have a craniopharyngioma, you can feel confident entrusting your care to the multidisciplinary team at Tampa General Hospital. Our neurosurgeons and ENT surgeons are internationally known and respected for their expertise in both traditional and endoscopic approaches to skull base surgery. In fact, in affiliation with the University of South Florida, our team has been recognized as a Multidisciplinary Team of Distinction by the North American Skull Base Society. You can schedule a consultation with one of the experienced providers at our Tampa, FL, treatment center by calling (800) 822-3627.