Craniopharyngeoma – also known as Erdheim tumor – is a benign brain tumor. This slow-growing tumor occurs predominantly in children and adolescents and arises as a malformation in the area of the pituitary gland (pituitary gland). Diagnosis of craniopharyngeoma is often difficult, as symptoms may indicate a variety of other conditions.
What is craniopharyngeoma?
Schematic diagram showing the location of a brain tumor in the brain. Click to enlarge. Craniopharyngeoma is a slow-growing brain tumor. The tumor is benign and usually causes symptoms at a very late stage. The craniopharyngeoma develops in the area of the pituitary gland, the so-called pituitary gland. There are two types of craniopharyngeoma, adamantine and papillary. The adamantinous craniopharyngeoma usually occurs in children between the ages of 5 and 10. The papillary type, on the other hand, occurs exclusively in adults, and here the age is usually around 60 to 75 years. The pituitary gland is responsible for hormone production. Hormones influence growth, metabolism, pubertal development, weight regulation as well as the regulation of the body’s fluid balance. Since the craniopharyngeoma grows in the area of the pituitary gland, it can lead to disturbances in the above-mentioned areas as it progresses. Craniopharyngeoma is often accompanied by visual disturbances because the optic nerves cross in the area of the pituitary gland and are often affected. Craniopharyngeoma is a chambered, fluid-filled tumor encased in a solid capsule. The fluid contains high levels of cholesterol. There are calcium deposits in the solid components. Craniopharyngeoma grows very slowly and does not metastasize.
Causes
The cause of craniopharyngeoma is a malformation in the brain, more specifically in the pituitary gland (hypophysis). A duct is formed in the pituitary gland during the development of the fetus in the womb, which disappears as the fetus matures. This duct is also known as the craniopharyngeal duct. If residual cells remain during the regression of the duct, pathological growths can develop as a result. A craniopharyngeoma can develop from these growths. However, why the residual cells of the embryonic duct change and begin to proliferate is still not clear.
Symptoms, complaints, and signs
Initially, a craniopharyngeoma does not cause any definite symptoms or complaints. The benign brain tumor does not cause signs of disease until it grows larger. Often, symptoms such as headaches, vision problems, and growth retardation do not develop for years. The increased intracranial pressure can also cause pain attacks and neurological deficits. Typical signs of a brain tumor also include increased thirst, nausea and vomiting, and increased urine output. Gastrointestinal symptoms occur primarily in the morning and on an empty stomach. Due to its localization in the optic nerve area, visual disturbances and visual field loss may occur. Its proximity to the pituitary gland and hypothalamus may promote hormonal deficits. This results in significantly delayed growth, especially in children and adolescents. The formation of secondary sexual characteristics may also be disturbed as a result of a craniopharyngeoma. In addition, the hormonal disorders can cause hypofunction of the adrenal glands and thyroid glands, which in turn results in various physical complaints. In severe cases, diabetes insipidus may develop, which is manifested by fatigue, severe thirst and impaired performance. The often very serious effects of the brain tumor can greatly impair the quality of life and cause psychological discomfort in many patients.
Diagnosis and course
Because of the difficulty of diagnosis, craniopharyngeoma can take a long time to diagnose. Based on the symptoms, a variety of conditions are possible. Delayed growth is usually noted in children, making the pediatrician the first point of contact. After taking the medical history, a blood analysis is performed in which, among other things, the hormone level is measured. Since visual disturbances are often also present, the ophthalmologist takes over further diagnostics. In cooperation with a neurologist, imaging procedures such as X-ray, CT and magnetic resonance imaging are used.Especially with the help of MRI, even small craniopharyngeomas can be easily detected. Craniopharyngeoma is a benign brain tumor. However, since it can also grow into adjacent areas in the brain, serious symptoms can occur over time, which not only reduce the quality of life of those affected, but also pose a risk to their health. The course of craniopharyngeoma can be seen in two parts, since in 30 percent of all cases the tumor forms again after surgery. Only those who remain recurrence-free for more than five years can be considered completely cured. However, this requires that the craniopharyngeoma has been completely removed. In many cases – if the tumor grows into the hypothalamus – there may be late intellectual consequences.
Complications
Due to craniopharyngeoma, patients experience various disorders and complaints. In most cases, children in particular are affected by these complaints, so that developmental disorders occur in them and, therefore, consequential damage can also occur in adulthood. Those affected suffer primarily from headaches and visual disturbances. The headaches can also spread to other regions of the body and cause pain there as well. Children suffer from growth disorders and also increased thirst due to craniopharyngeoma. Because of this, frequent urination also occurs, and thus not infrequently depression or other complaints. Craniopharyngeoma can spread to other regions of the body without treatment. The treatment of this complaint is done by removing the tumor. As a rule, no particular complications occur. However, those affected are also dependent on radiation therapy to ensure that the tumor is completely removed. In many cases, this results in a positive course of the disease and the patient’s life expectancy is not affected by the craniopharyngeoma.
When should one go to the doctor?
When unusual visual disturbances and other signs of a brain tumor are noticed, medical advice is needed. A doctor should also be consulted if severe headaches or loss of visual field on one or both sides occur. Blindness indicates that the tumor is already far advanced – the affected person must see the family doctor immediately. Other warning signs that require immediate clarification are personality changes, dizziness or problems with concentration and memory. Parents who notice growth disturbances or a lack of puberty in their child should call in the pediatrician. The actual treatment takes place in the hospital. After the procedure, the affected person must take advantage of the ordered check-ups with the doctor and inform the medical professional about any complaints and abnormalities. If a craniopharyngeoma is suspected, medical advice must therefore be sought in any case. The right contact point is the family doctor or a neurologist. Depending on the type and severity of the symptoms, various specialists must also be consulted.
Treatment and therapy
The first choice in treating craniopharyngeoma is surgery. The goal is to completely remove the brain tumor without affecting neighboring brain regions. However, in approximately 80 percent of all cases, it is not possible to completely remove the benign brain tumor. Usually, the craniopharyngeoma has already spread so far that regions such as the optic nerves and the hypothalamus are also affected. Especially in the area of the hypothalamus, the removal of the craniopharyngeoma turns out to be extremely difficult. Tumor tissue and healthy brain structures are difficult to distinguish from each other. Craniopharyngeoma has a high recurrence rate, which means that within the first five years there is a high risk that the craniopharyngeoma will recur, making another operation unavoidable. If the craniopharyngeoma is a large, unchambered cyst in the case of recurrence, puncture may be sufficient. If the craniopharyngeoma could not be completely removed or only a puncture was performed, radiation therapy can be performed afterwards. Radiation treatment is given over a period of several weeks and is intended to prevent recurrent growth. Furthermore, drug therapy is initiated for craniopharyngeoma.Since hormones are produced in the pituitary gland, it is usually necessary to initiate hormone replacement therapy. If the thyroid gland has been affected, hypofunction can be treated with the help of levothyroxine. Furthermore, testosterone as well as estrogen and progestin preparations are used to treat hypofunction of the reproductive organs. Because a craniopharyngeoma can also cause other symptoms, drug therapy is tailored to the affected person in each individual case.
Outlook and prognosis
The prognosis for patients with craniopharyngeoma is relatively good only if this brain tumor is detected early. If it can be completely removed during surgery, this improves the long-term chances of survival. But the recurrence rate is high. It suggests that a realistic prognosis for craniopharyngeoma is difficult. The outlook is good only for small, very compact and thus easily removable craniopharyngeomas. For the majority of patients with craniopharyngeomas, more than 80 percent require hormone replacement therapy for the rest of their lives. Successful surgery for craniopharyngeoma is often followed by radiation therapy. The prognosis for 70 to 80 percent of patients treated this way is that they will survive the next ten years. Radiation therapy can cause hormone deficiencies that require treatment. Surgery and complete removal of a craniopharyngeoma cannot improve any loss of vision or memory that has already occurred. They represent permanent damage. In the overweight patients with craniopharyngeoma, the prognosis is not so good. About 30 percent of those with craniopharyngeomas are overweight or obese. For these individuals, the risks of weight-related sequelae such as diabetes mellitus or cardiovascular disease are increased. Under lifelong medical follow-up, people with a successfully operated and irradiated craniopharyngeoma can continue to live relapse-free.
Prevention
To date, there are no known effective measures to prevent craniopharyngeoma. Since the exact cause is unknown, only general behavioral measures can be taken to reduce the general risk of disease and cancer. In addition to a healthy diet, regular exercise should be taken. Furthermore, nicotine, alcohol and drugs should be avoided. Particularly in the case of children, care should be taken to avoid unnecessary X-ray examinations. This is because radiation results in the possibility of developing cancer later, which does not rule out benign craniopharyngeoma.
Follow-up
In most cases, there are few to no special aftercare options available to the person with a craniopharyngeoma. In this case, early diagnosis is very important to prevent further complications or worsening of symptoms. As a rule, the craniopharyngeoma cannot heal on its own, so the affected person should consult a doctor at the first symptoms and signs of the disease. As a rule, craniopharyngeoma requires surgical intervention to remove the tumor. After such an operation, the affected person should in any case rest and take care of his body. Exertion or other stressful activities should be discouraged in order not to put unnecessary strain on the body. In many cases, it is necessary to take various medications, and the patient should always ensure that they are taken regularly and in the correct dosage. The support of the patient by friends or family is also very important in this disease and can prevent the development of depression or other psychological upsets. Possibly, the life expectancy of the affected person is reduced due to this disease.
This is what you can do yourself
Patients with craniopharyngeoma suffer from various diffuse symptoms that limit their quality of life and lead to uncertainty about their health status. For example, headaches and an increased feeling of thirst are typical of craniopharyngeoma. The resulting frequent urination sometimes restricts patients in their daily life and in performing activities outside the home. The most important self-help measure in the case of craniopharyngeoma is to contact a doctor quickly despite the non-specific symptoms. Once the diagnosis is established, the treating medical team initiates therapy.Usually, the patient goes to a hospital and undergoes surgery with the aim of removing the brain tumor as completely as possible. During this time, the performance of the child patients is severely limited. Attending school is also impossible during this time, so that those affected usually fall behind in their school career. Distance learning and the support of teachers and classmates partially prevent this. After successful removal, patients promote their own health by going to necessary medical follow-up visits. This is because craniopharyngeoma is characterized by a high recurrence rate, and quick action is required. Patients refrain from high-stress sports after surgery in their own interest.
