Craniopharyngioma – also known as Erdheim tumor – is a benign brain tumor. This slow-growing tumor mainly occurs in children and adolescents and develops as a malformation in the area of the pituitary gland (pituitary gland). The diagnosis of craniopharyngioma is often difficult because the symptoms can indicate a variety of other diseases.
What is a craniopharyngioma?
Schematic representation of the location of a brain tumor in the brain. See AbbreviationFinder for abbreviations related to Craniopharyngioma.
Craniopharyngioma is a slow-growing brain tumor. The tumor is benign and usually only causes symptoms at a very late stage. The craniopharyngioma develops in the area of the pituitary gland, the so-called pituitary gland.
A distinction is made between two types, the adamantine and the papillary craniopharyngioma. Adamantine craniopharyngioma most often occurs in children between the ages of 5 and 10 years. The papillary type, on the other hand, occurs exclusively in adults, with the age generally being around 60 to 75 years.
The pituitary gland is responsible for hormone production. Hormones have an influence on growth, metabolism, the development of puberty, weight regulation and the regulation of the body’s fluid balance. Since the craniopharyngioma grows in the area of the pituitary gland, disturbances in the above-mentioned areas can occur in the further course. The craniopharyngioma is often accompanied by visual disturbances, since the optic nerves cross in the area of the pituitary gland and are often affected.
The craniopharyngioma is a chambered, fluid-filled mass encased in a solid capsule. The liquid contains high levels of cholesterol. There are lime deposits in the solid components. The craniopharyngioma grows very slowly and does not metastasize.
Causes
The cause of a craniopharyngioma is a malformation in the brain, more precisely in the pituitary gland (pituitary gland).
During the development of the fetus in the womb, a duct develops in the pituitary gland, which disappears again in the course of the maturation process. This duct is also known as the craniopharyngeal duct. If residual cells remain when the duct recedes, pathological growths can then develop. A craniopharyngioma can develop from these growths.
However, why the residual cells of the embryonic duct change and begin to proliferate has not yet been clarified.
Symptoms, Ailments & Signs
A craniopharyngioma does not initially produce any clear symptoms or symptoms. The benign brain tumor only causes signs of disease as it grows. Symptoms such as headaches, blurred vision and growth disorders often only develop years later. The increased intracranial pressure can also lead to pain attacks and neurological deficits.
Typical signs of a brain tumor are also increased thirst, nausea and vomiting and increased urine output. The gastrointestinal complaints occur primarily in the morning and on an empty stomach. Due to its localization in the area of the optic nerve, visual disturbances and visual field defects can occur.
Proximity to the pituitary gland and hypothalamus can promote hormonal imbalances. This leads to significantly delayed growth, especially in children and adolescents. The formation of secondary sexual characteristics can also be disturbed as a result of a craniopharyngioma. In addition, the hormonal imbalances can lead to hypofunction of the adrenal glands and thyroid glands, which in turn results in various physical complaints.
In severe cases, diabetes insipidus can develop, which manifests itself in fatigue, severe thirst and reduced performance. The often very serious effects of brain tumors can severely impair the quality of life and cause psychological problems in many patients.
Diagnosis & History
It can take a long time to diagnose craniopharyngioma due to the difficulty of diagnosis. A variety of diseases are possible based on the symptoms. As a rule, delayed growth is noted in children, so the first point of contact is the pediatrician.
After the anamnesis has been recorded, a blood analysis is carried out, in which, among other things, the hormone level is measured. Since visual disturbances are often also present, the ophthalmologist takes over the further diagnostics. In cooperation with a neurologist, imaging methods such as B. X-ray, CT and magnetic resonance imaging used. Small craniopharyngiomas can also be easily detected, especially with the help of MRI.
The craniopharyngioma 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 a craniopharyngioma can be seen in two parts, since the tumor forms again in 30 percent of all cases after an operation. Only those who remain free of recurrence for more than five years can be considered completely cured. The prerequisite for this, however, is that the craniopharyngioma has been completely removed. In many cases – if the tumor grows into the hypothalamus – there can be late intellectual consequences.
Complications
Due to the craniopharyngioma, patients experience various disorders and complaints. In most cases, children in particular are affected by these symptoms, so that they develop disorders and consequential damage can also occur in adulthood. Those affected primarily suffer from headaches and blurred vision. The headache can also spread to other regions of the body and cause pain there as well.
Children suffer from growth disorders and increased thirst due to craniopharyngioma. This also leads to frequent urination and thus not infrequently to depression or other complaints. Craniopharyngioma can also spread to other areas of the body without treatment.
This complaint is treated by removing the tumour. Usually there are no special complications. However, those affected also need radiation therapy to completely remove the tumor. In many cases, the course of the disease is positive and the life expectancy of the patient is not affected by the craniopharyngioma.
When should you go to the doctor?
If unusual visual disturbances and other signs of a brain tumor are noticed, medical advice is required. A doctor should also be consulted if there is a severe headache or loss of visual field on one or both sides. Blindness indicates that the tumor is already well advanced – the affected person must consult their family doctor immediately. Other warning signs that require immediate clarification are personality changes, dizziness or problems with concentration and memory.
Parents who notice growth disorders in their child or a lack of puberty should contact the pediatrician. The actual treatment takes place in the hospital. After the procedure, the person concerned must go to the doctor for the prescribed check-ups and inform the doctor of any symptoms and abnormalities. If a craniopharyngioma is suspected, medical advice must be sought in any case. The right place to go is your family doctor or a neurologist. Depending on the type and severity of the symptoms, different specialists must also be consulted.
Treatment & Therapy
The first choice in treating a craniopharyngioma is surgery. The aim is to completely remove the brain tumor without affecting the neighboring brain regions.
In about 80 percent of all cases, however, it is not possible to completely remove the benign brain tumor. As a rule, the craniopharyngioma 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 craniopharyngioma turns out to be extremely difficult. Tumor tissue and healthy brain structures are difficult to distinguish from one another.
The craniopharyngioma has a high recurrence rate, which means that within the first five years there is a high risk that the craniopharyngioma will recur, which makes repeat surgery unavoidable. If the craniopharyngioma is a large, non-chambered cyst in the event of a recurrence, a puncture may also be sufficient.
If the craniopharyngioma could not be completely removed or only a puncture was carried out, radiotherapy can be carried out afterwards. The radiation treatment is carried out over a period of several weeks and is intended to prevent recurrence growth.
Furthermore, drug therapy is initiated for a craniopharyngioma. Since hormones are produced in the pituitary gland, it is usually necessary to initiate hormone replacement therapy. If the thyroid gland has been affected, levothyroxine can be used to treat an underactive thyroid. Furthermore, testosterone – as well as estrogen – and progestin preparations are used to treat hypofunction of the genital organs.
Since a craniopharyngioma can also cause other symptoms, drug therapy is tailored to the individual affected.
Outlook & Forecast
The prognosis for patients with a craniopharyngioma is only relatively good if this brain tumor is detected early. If it can be completely removed during surgery, that improves the long-term chances of survival. But the relapse rate is high. It suggests that a realistic prognosis for craniopharyngiomas is difficult.
The prospects are only good for small, very compact and therefore easily removable craniopharyngiomas. For the majority of those affected with craniopharyngiomas, over 80 percent of those affected require lifelong hormone replacement products. Radiation therapy is often required after successful craniopharyngioma surgery. The prognosis for 70 to 80 percent of the patients treated in this way is that they will survive the next ten years.
Radiation therapy can cause hormone failures that require treatment. The operation and complete removal of a craniopharyngioma can no longer improve vision or memory losses that have already occurred. They represent permanent damage.
The prognosis is not so good for obese patients with a craniopharyngioma. About 30 percent of people with craniopharyngiomas are overweight or obese. For these people, the risks of weight-related complications such as diabetes mellitus or cardiovascular diseases are increased. With lifelong medical follow-up care, people with a successfully operated and irradiated craniopharyngioma can continue to live without recurrence.
Prevention
To date, no effective measures are known to prevent craniopharyngioma. 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, you should exercise regularly.
Furthermore, nicotine, alcohol and drugs should be avoided. In children in particular, care should be taken to ensure that no unnecessary X-ray examinations are carried out. Because radiation has the consequence that one can later develop cancer, which also does not rule out a benign craniopharyngioma.
Aftercare
In most cases, those affected with a craniopharyngioma have few or no special options for aftercare. In this case, early diagnosis is very important to prevent further complications or worsening of the symptoms. As a rule, the craniopharyngioma cannot heal on its own, so the affected person should consult a doctor as soon as the first symptoms and signs of the disease appear.
A craniopharyngioma usually requires surgery to remove the tumor. After such a procedure, the person concerned should definitely rest and take care of his body. Exertion or other stressful activities are not recommended in order not to unnecessarily burden the body.
In many cases, it is necessary to take various medications, whereby the person concerned should always ensure that they are taken regularly and that the dosage is correct. The support of the patient from friends or family is also very important with this disease and can prevent the development of depression or other psychological upsets. The life expectancy of those affected may be reduced as a result of this disease.
You can do that yourself
Patients with craniopharyngioma suffer from various diffuse symptoms that limit their quality of life and lead to uncertainty about their state of health. Typical symptoms of a craniopharyngioma are, for example, headaches and an increased feeling of thirst. The resulting frequent urination sometimes restricts the patients in their everyday life and in their activities outside the home. The most important self-help measure for craniopharyngiomas is to consult a doctor quickly despite the non-specific symptoms.
As soon as the diagnosis is established, the treating medical team initiates the therapy. The patient usually goes to a hospital and undergoes an operation with the aim of removing the brain tumor as completely as possible. During this time, the performance of the child patients is severely limited. It is also impossible to attend school during this time, so that those affected usually fall behind in their school career. Distance learning and the support of teachers and classmates partially prevents this.
After a successful removal, patients promote their own health by going to the necessary medical follow-up checks. Because a craniopharyngioma is characterized by a high recurrence rate, which requires quick action. After the operation, the patients abstain from strenuous sports in their own interest.
