Craniopharyngioma
Ricarda Schwarz studied medicine in Würzburg, where she also completed her doctorate. After a wide range of tasks in practical medical training (PJ) in Flensburg, Hamburg and New Zealand, she is now working in neuroradiology and radiology at the Tübingen University Hospital.
More about the experts All content is checked by medical journalists.The craniopharyngioma is a benign tumor in the head. It mostly occurs in children and causes hormonal imbalances, visual disturbances and / or headaches. Surgery and drug therapy can cure this brain tumor in many cases. Here you can read everything you need to know about craniopharyngioma.
ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. D43C71D33
Craniopharyngioma: description
The craniopharyngioma is a benign tumor in the head. It develops from cell debris from embryonic development. Since the craniopharyngioma grows very slowly, the first symptoms usually appear in children between five and ten years of age. But adults can also develop this tumor. It occurs most frequently between the ages of 50 and 75. Overall, however, the craniopharyngioma is rather rare. In children it makes up five to ten percent, in adults only three to five percent of all tumors in the skull.
Doctors differentiate between two forms of craniopharyngioma:
- The adamantinomatous craniopharyngioma mainly affects children and adolescents. It is interspersed with many small, often fluid-filled inclusions. In addition, there are horn cells and calcium, which can often be seen well in imaging procedures.
- Papillary craniopharyngioma occurs mainly in adults. It rarely keratinizes or calcified.
Despite their differences, both types of tumors are treated equally.
Craniopharyngioma: symptoms
Although it is basically a benign tumor, depending on its location and size, the craniopharyngioma can sometimes trigger dangerous symptoms. If it presses on the optic nerve, for example, visual disturbances can occur. Most of the time, the outer visual fields fail first. Larger tumors can even cause those affected to go blind.
The early signs of a craniopharyngioma are often changes in personality, concentration, and memory, often months before the diagnosis. In children, the first noticeable presence of the tumor is a growth disorder or a failure to go through puberty. The reasons for this are disorders in the hormonal balance:
Hormonal disorders caused by a craniopharyngioma
The craniopharyngioma can disrupt the function of the pituitary gland (pituitary gland) and that of its superordinate center (hypothalamus). Both produce hormones, which in turn regulate the production of hormones in other glands in the body (such as the thyroid). The craniopharyngioma can ultimately influence the formation of various hormones in the body and subsequently trigger a wide variety of complaints. The most common are short stature, obesity (adiposity), diabetes insipidus, and eating disorders.
Growth disorders such as short or tall stature can occur when the craniopharyngioma affects the production of growth hormone in the pituitary gland. In adults, this can lead to disorders of lipid metabolism and acromegaly (enlarged toes, fingers, chin and nose, high blood pressure, increased sweating, etc.).
Overweight and underweight, osteoporosis and increased or decreased hairiness are possible consequences if the craniopharyngioma disrupts the production of the stress hormone cortisol in the adrenal glands by impairing the pituitary gland.
The craniopharyngioma can also interfere with the body's fluid balance: This is regulated by the antidiuretic hormone (ADH), which is formed in the hypothalamus, stored in the pituitary gland and released into the blood from there when required. It ensures that not too much water is excreted with the urine. In this way, ADH also affects blood salt levels and blood pressure. If the tumor triggers an ADH deficiency, diabetes insipidus develops: In this hormone deficiency disease, those affected excrete many liters of clear urine (polyuria) and have to drink a lot in order not to dry out (polydipsia).
The release of female (estrogens) and male (testosterone) sex hormones, which is controlled by the pituitary gland, can also influence a craniopharyngioma. Possible consequences are, for example, that puberty is delayed or absent, or menstrual bleeding is disturbed or stopped.
By influencing the pituitary gland, a craniopharyngioma can also impair the function and hormone secretion of the thyroid gland. For example, an excess of thyroid hormones can lead to palpitations, diarrhea and excessive sweating. If, on the other hand, too few thyroid hormones are produced, many metabolic processes freeze. Affected people are cold, constipated, tired and lacking motivation.
General symptoms of a brain tumor
As with other brain tumors, symptoms such as headache, nausea, vomiting, muscle paralysis and water head can also occur with a craniopharyngioma. Such symptoms usually only appear in large tumors.
Craniopharyngioma: causes and risk factors
The craniopharyngioma arises from cells that formed a connecting duct between the brain and the throat (ductus craniopharyngeus) during embryonic development. A craniopharyngioma can develop from remnants of these cells in children and adults as a result of sudden, uncontrolled multiplication. It grows in the so-called Turkish saddle (Sella turcica). This is a bony hollow in the anterior base of the skull in which the pituitary gland is located. The two optic nerves cross in the immediate vicinity. Most of the symptoms of this tumor are due to the displacement of neighboring structures.
It is not yet known why the cell remains degenerate and form a craniopharyngioma.
Craniopharyngioma: examinations and diagnosis
If a craniopharyngioma is suspected, a visit to an endocrinologist, a specialist in hormonal disorders, is particularly important. He will have the patient's complaints described in detail in order to gain any indications of a disruption of certain hormones. The measurement of different hormone concentrations in the blood, saliva or urine is also informative.
The most important imaging technique for a craniopharyngioma is magnetic resonance imaging (MRI). The exact location and size of the tumor can be determined. If an MRI is not possible for certain reasons (e.g. because the patient suffers from claustrophobia or has a pacemaker), computed tomography (CT) can be performed as an alternative. Here, too, a craniopharyngioma including its typical calcifications can be shown precisely.
An examination by the ophthalmologist serves to clarify any visual disturbances. A visit to the neurologist (neurologist) is required if the patient has increased intracranial pressure or cranial nerve failure.
Craniopharyngioma: treatment
Treatment options for craniopharyngioma include drug treatment, surgery, and radiation therapy. The exact therapy plan is individually adapted to the patient.
Drug treatment for craniopharyngioma is not aimed at getting rid of the tumor (drugs cannot do this). Rather, a deficiency in hormones caused by the tumor is compensated for (hormone replacement therapy). For example, ADH, thyroid, growth, sex and stress hormones can be replaced by drugs. The exact dosage is not very easy, because the individual hormones are formed in different amounts during the course of the day and depending on the respective phase of life. This should be taken into account as optimally as possible when dosing. This requires regular monitoring of treatment.
During an operation, an attempt is made to remove the craniopharyngioma as completely as possible. Access is preferably via the nose (transnasal operation).
Sometimes, however, a complete removal of the tumor is not possible because the risk of complications would be too great (development of personality disorders, permanent diabetes insipidus, etc.). So you only remove part of the tumor and treat the remaining tumor with radiation therapy. Irradiation can also be useful in the case of a recurring craniopharyngioma (relapse) and for emptying the fluid-filled cavities (cysts) in the tumor.
Examination and treatment
For more information on examination and treatment, read the article on brain cancer.
Craniopharyngioma: disease course and prognosis
The prognosis of a craniopharyngioma depends largely on whether it is discovered early and can be completely removed surgically. In the long term, it is very good for small, compact and easily removable tumors. However, 80 to 90 percent of patients have to compensate for existing hormone failures with the help of hormone replacement preparations for the rest of their lives.
If a craniopharyngioma is treated with surgery and subsequent radiation, the cure rate after ten years is 70 to 83 percent. A long-term consequence of the irradiation can also be hormone failures, which have to be treated with medication for the entire life.
Disorders of vision, memory and memory that existed before the operation can usually not be remedied by the operation.
About 30 percent of patients with craniopharyngioma are severely obese. The risk of secondary diseases such as diabetes mellitus and heart disease is therefore high.
In general, the following applies: Patients with a craniopharyngioma are dependent on lifelong follow-up care - not only because of the hormone replacement therapy required in many patients and the risk of complications from obesity. The high relapse rate in craniopharyngioma should not be underestimated.
Tags: interview hospital alcohol
