What Is It?
A craniopharyngioma is a tumor which occurs in the region of the pituitary gland at the base of the brain. It is thought to arise from remnants of embryonic development which are important in the formation of the pituitary gland. These tumors tend to be slow growing but locally destructive. They very frequently contain areas of calcification as well as cystic areas which are filled with fluid. They can vary from predominantly solid to predominantly cystic and everything in between.
Craniopharyngiomas occur both in children and adults, with peaks in both children and in older adults. They generally occur in the region of the pituitary gland, known as the sella turcica, or simply sella. Commonly, they are largely in the supra-sellar region and can expand upward into the base of the brain in an area known as the hypothalamus. The optic nerves, the nerves carrying visual information from the eyes, are also in this region.
What Types of Symptoms Are Typical?
Craniopharyngiomas tend to grow slowly and thus their symptoms usually develop slowly over years. General symptoms such as headache are common but because of their location, craniopharyngiomas also commonly involve the pituitary gland, hypothalamus and the optic nerves. The pituitary gland and hypothalamus are important for several hormonal functions and autonomic function. Therefore, damage to them can lead to several hormonal syndromes.
Examples of these hormonal insufficiencies are growth hormone insufficiency (growth failure in young patients), adrenal insufficiency (hypotension, hypoglycemia, lethargy, confusion, etc.), hypothyroidism (weight gain, fatigue, cold intolerance), and diabetes insipidus (excessive fluid intake and urination). Many patients also have reproductive dysfunction including impotence in men and loss of normal menstrual cycles in women. Young patients can have delayed puberty.
Injury to the optic nerves from the eyes can frequently lead to various degrees of visual loss. Larger tumors can have more of an impact on the hypothalamus and brain which can lead to vague endocrine, autonomic and behavioral manifestations.
Occasionally larger tumors which are located superiorly in the 3rd ventricle, a fluid-filled space in the brain, can lead to hydrocephalus.
How Is The Diagnosis Typically Made?
After a thorough neurological examination, most patients get an imaging study such as a CT scan
or MRI scan
. An MRI typically demonstrates a typical appearance in the sellar and supra-sellar regions. While the appearance of craniopharyngioma is fairly unique, it must be distinguished from other brain tumors which can occur in the same region, such as a pituitary adenoma, Rathke's cleft cyst or meningioma. A definitive diagnosis requires a sample of tumor tissue to be analyzed by a pathologist.
In addition to these imaging studies, most patients with a tumor in this location will require an extensive hormonal work-up, often by an endocrinologist. This includes blood testing of several pituitary hormones and other related hormones. This helps to diagnose any hormonal insufficiencies which may be caused by the tumors compression or destruction of the pituitary gland, stalk or the hypothalamus.
Most patients will also undergo a detailed ophthalmologic evaluation to determine if there is any visual dysfunction.
What Are Some Common Treatments?
The primary medical treatments that many patients with craniopharyngioma will undergo is hormonal replacement therapy for those patients with abnormal hormonal function. This is not aimed at treating the tumor itself but rather is used to replace hormonal function which has been damaged by the tumor.
To treat the tumor itself, most patients will be recommended for surgery to remove as much of the tumor as possible. While total removal is ideal in terms of long term control of the tumor, this is often not possible because of the complex anatomy of the region. Overly aggressive surgery can result in worsening of vision, hormonal function and other neurological disability. Very large tumors that cannot be removed completely and cystic tumors very commonly recur even after multiple procedures. However, this is quite variable.
Surgery is either accomplished through a craniotomy (an opening through the skull) or through the nose in what is called a transsphenoidal approach, or both. Which is appropriate varies depending on the patient and the specific tumor characteristics.
Some tumors that can't be removed completely will undergo radiation treatments to try to control the residual tumor.
Because these are complex tumors, their treatment varies considerably from one patient to another. Each patient should discuss their treatment options with their own physicians.
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This site is not intended to offer medical advice. Every patient is different, and only your personal physician can help to counsel you about what is best for your situation. What we offer is general reference information about various disorders and treatments for your education.