The plexus papilloma is a rare, benign brain tumor that originates in the vein network (plexus choroideus) surrounding the cerebral ventricles. Plexus papillomas mainly affect infants and children under 12 years of age. Untreated tumours, due to increased accumulation of cerebrospinal fluid (liquor), can lead to serious failure of certain areas of the brain and to the formation of a hydrocephalus (hydrocephalus). The prognosis after surgical removal is good.
What is plexus papilloma?
The rare benign (non-cancerous) plexus papilloma develops on the vein network that encloses the individual cerebral ventricles for supply and disposal. If left untreated, the cerebrospinal fluid (liquor) circulating between the ventricles and continuously being exchanged will gradually become obstructed. Above all, it can lead to increased intracranial pressure with the corresponding symptoms due to a disturbed outflow with the liquor production rate remaining the same. See polyhobbies for Meanings of Hollow Nack (or Hyperlordosis).
In addition, the space requirement of the plexus papilloma presses certain areas of the brain and leads to corresponding symptoms up to and including functional failures. Small children under the age of 2 and children up to the age of about 12 are particularly affected, with girls and boys being equally affected. The plexus papilloma can be surgically removed with a good subsequent prognosis. The benign plexus papilloma is opposed to the malignant plexus tumor, which tends to recur even after surgical removal.
The relative rarity of plexus papilloma or plexus carcinoma has not shown any statistical abnormalities with regard to possible triggering factors for their development. Tumor research has not made any real progress in this area because there are obviously no clear starting points. From time to time attempts are made to blame certain gene mutations.
For example, attempts have been made, with little success, to link plexus papillomas to Aicardi syndrome or Li-Fraumeni syndrome. Aicardi syndrome is an X-linked hereditary disease that leads to malformations in the brain, and the autosomal dominant, i.e. not gender-typical, inherited Li-Fraumeni syndrome leads to a high probability of those affected developing multiple tumors at a young age fall ill.
Another approach to research has been to try to trace the development of plexus papilloma or carcinoma to certain viral infections. This approach did not yield any reliable findings either. In conclusion, it should be noted that there is (still) no scientifically proven knowledge about the causes of a plexus papilloma or carcinoma.
Symptoms, Ailments & Signs
The location of a plexus papilloma on one or more cerebral ventricles means that symptoms that are initially non-specific but nevertheless typical appear in the early stages of the tumor. It is above all the papilloma itself that impedes the circulation or the outflow of the liquor due to its volume. At the same time, the papilloma tissue also produces additional brain water, so that the inflow and outflow of the liquor are no longer consistent with each other.
First there is an increase in intracranial pressure, which leads to the unspecific symptoms of headache, nausea to the point of vomiting, seizures and irritability. Especially in small children, where it is difficult to correctly classify the unspecific symptoms, if left untreated, hydrocephalus (hydrocephalus) can develop at an advanced stage.
The direct pressure that the plexus papilloma exerts on the surrounding brain area can also lead to neuronal dysfunctions that cause disorders or failure of certain motor and/or sensory abilities.
Diagnosis & course of disease
If the non-specific symptoms described above occur, which cannot be assigned to any specific disease, there is a suspicion of a neurological disease in the brain. While an electroencophalogram (EEG) provides initial indications of whether and where a brain tumor might be present, the imaging methods computed tomography (CT) and magnetic resonance imaging (MRI) allow more precise statements.
The focus of diagnostics is shifting from CT to MRI because the soft structures are shown more clearly by MRI. An MRI can give a good picture of a plexus papilloma. The tissue of the tumor appears as a homogeneous mass with a cauliflower-like structure. An analysis of the liquor can provide information about whether there is a nerve inflammation that led to the symptoms.
A biopsy followed by a subtle examination can provide the ultimate certainty as to whether the tumor is benign or malignant. Ultimately, the only effective treatment option is the complete surgical removal of the tumor.
Complications from plexus papilloma usually only occur if the disease is left untreated. Self-healing does not occur in this case, so surgical removal of the tumor is necessary. If the tumor is not removed, this can lead to high pressure in the brain and thus to failure of the various brain areas. Those affected usually suffer from paralysis and other motor disorders.
The high pressure in the brain also causes severe headaches and, not infrequently, vomiting and nausea. Those affected also suffer from cramps and significantly increased irritability. A hydrocephalus can also form due to the plexus papilloma if the water is not removed from the brain. Without treatment, the brain is irreversibly damaged by the plexus papilloma.
Treatment, as a rule, is not associated with any particular complications. The tumor can be removed by surgery. Other complaints usually do not occur. People may need radiation therapy after treatment. If the treatment is successful, the life expectancy of the patient is not affected.
When should you go to the doctor?
Since a plexus papilloma is a tumor, it must always be examined and treated by a doctor. This disease does not heal itself and usually leads to the death of the person affected if there is further accumulation of water in the brain. Early diagnosis and treatment of plexus papilloma has a very positive effect on the further course of this disease and can prevent complications.
The doctor should be consulted for plexus papilloma if a tumor develops on the affected person’s head. The patients suffer from headaches and nausea, these symptoms occurring for no particular reason and not going away on their own. Painkillers can’t relieve the pain either. A hydrocephalus also indicates the plexus papilloma and should always be examined by a doctor. In the further course, there are also motor failures, which also point to the brain tumor.
The examination and diagnosis is usually carried out with the help of an MRI. However, further treatment is carried out in a hospital by means of a surgical procedure. Whether the patient’s life expectancy will be reduced by the plexus papilloma cannot generally be predicted.
Treatment & Therapy
Since there is no way to kill a plexus papilloma with drugs or other means, once the diagnosis is clear, the only effective treatment available is complete surgical removal of the papilloma. Only in a few cases can a significant alleviation of the symptoms be achieved by artificially draining the liquor, which reduces intracranial pressure.
Specialized centers are able to completely remove the tumor microsurgically or endoscopically as gently as possible. The aim is also to enable the circulation of the cerebrospinal fluid again by reopening possible drains. During the operation, so-called neuronavigation and imaging methods are used to increase safety and tissue protection, which enable constant monitoring of the surgical procedure.
There is no consensus among experts as to whether postoperative continuation of treatment with radiotherapy is necessary and sensible.
Because no clear trigger factors for the development of a plexus papilloma are known to date and viral diseases or genetic predispositions could not be proven as the cause, there are no preventive measures that could prevent the tumor from developing.
However, because small children and children under the age of 12 are usually affected, persistent and recurring symptoms such as headaches, malaise and possible personality changes in children that cannot be assigned to other diseases should also be properly clarified neurologically.
In most cases, those affected with a plexus papilloma have only a few or limited direct follow-up measures available. Therefore, the affected person should consult a doctor as soon as the first symptoms or signs of the disease appear in order to prevent the occurrence of further symptoms or complications. It cannot heal on its own, so treatment by a doctor is always necessary.
In most cases, those affected require surgical intervention. The child should then take it easy and rest. Efforts or stressful and physical activities should be avoided in order not to unnecessarily burden the body. Regular checks and examinations by a doctor are also necessary after a successful intervention in order to identify and remove further tumors at an early stage.
The children have to be supported in particular by their parents and relatives during the therapy. This can also prevent possible mental upsets or depression. If the plexus papilloma is recognized early and treated correctly, the affected person usually does not have a reduced life expectancy.
You can do that yourself
Plexus papilloma occurs in children and infants. Naturally, they are not sufficiently able to take self-help measures that would lead to healing. Therefore, legal guardians, relatives or close people from the social environment have a greater responsibility to achieve an improvement in the situation in the interests of the child by taking various approaches.
Dealing openly with the disease is indicated in everyday life. Risk factors and the overall circumstances should be sufficiently and understandably explained to the child. Open questions are to be answered honestly and informatively. This behavior prevents irritation or unpleasant surprises. With a sufficient flow of information, dealing with the disease is often easier to manage.
Since a hospital stay is necessary for the treatment of the brain tumor, measures should be taken to promote well-being and joie de vivre. Games and fun are to be integrated daily as far as possible. A positive basic attitude of adults has a good effect on the child. It has a motivating effect and reduces discomfort and fears. Contact with peers and other sick people can be perceived as pleasant by the child. The mutual exchange of accumulated experiences creates opportunities for better coping with the overall situation.