Plexus papilloma is a rare benign brain tumor that originates in the choroid plexus surrounding the ventricles of the brain. Plexus papillomas mainly affect infants and children under 12 years of age. Untreated tumors can lead to severe deficits of certain brain areas and the development of hydrocephalus due to increased accumulation of cerebrospinal fluid (CSF). The prognosis after surgical removal is good.
What is a plexus papilloma?
A rare benign (benign) plexus papilloma develops on the plexus of veins that externally encase the individual ventricles of the brain for supply and disposal. If left untreated, it gradually obstructs the flow of cerebrospinal fluid (CSF), which circulates between the ventricles and is continuously exchanged. In particular, disturbed outflow at a constant rate of CSF production can lead to increased intracranial pressure with corresponding symptoms. In addition, the spatial demand of the plexus papilloma presses certain brain areas and leads to corresponding symptoms up to functional deficits. Infants under 2 years of age and children up to about 12 years of age are mainly affected, with girls and boys being equally affected. Plexus papilloma can be surgically removed with a good subsequent prognosis. Benign plexus papilloma is contrasted with malignant (malignant) plexus tumor, which is prone to recurrence even after surgical removal.
Causes
The relative rarity of plexus papilloma or plexus carcinoma has not yet revealed any statistical abnormalities regarding possible precipitating factors for their development. Tumor research has not made much progress in this area because there are apparently no clear starting points. From time to time, attempts are made to make certain gene mutations responsible. 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 Li-Fraumeni syndrome, which is inherited in an autosomal-dominant, i.e. non-sex-typical, manner, leads to a high probability of developing multiple tumors at a young age in those affected. Another research approach consisted in the attempt to attribute the development of a plexus papilloma or carcinoma to certain viral infections. This approach also did not yield any robust findings. In conclusion, there is (still) no scientifically validated evidence on the causes of the development of a plexus papilloma or carcinoma.
Symptoms, complaints, and signs
The location of a plexus papilloma on one or more cerebral ventricles means that initially nonspecific, but nevertheless typical, symptoms occur at an early stage of the tumor. It is mainly the papilloma itself which, due to its volume, impedes the circulation, respectively the outflow of the cerebrospinal fluid. At the same time, the papilloma tissue also produces additional cerebrospinal fluid, so that inflow and outflow of the CSF are no longer in harmony. Initially, there is an increase in intracranial pressure, which leads to the nonspecific symptoms of headache, nausea to vomiting, seizures and irritability. Particularly in young children, in whom it is difficult to correctly classify the non-specific symptoms, the development of hydrocephalus can occur in an advanced stage if left untreated. The immediate pressure exerted by the plexus papilloma on the surrounding brain area may also result in neuronal dysfunction, causing disturbances or deficits in certain motor and/or sensory abilities.
Diagnosis and disease progression
When the nonspecific symptoms described above occur that cannot be attributed to a specific disease, the presence of neurologic disease in the brain is suspected. While an electroencephalogram (EEG) provides initial indications of whether and where a brain tumor might be present, the imaging techniques computed tomography (CT) and magnetic resonance imaging (MRI) allow more precise statements to be made. The focus of diagnosis shifts from CT to MRI because soft structures are more clearly imaged by MRI. An MRI can provide a good image of a plexus papilloma. The tissue of the tumor stands out as a homogeneous mass with a cauliflower-like structure.An analysis of the cerebrospinal fluid can provide information as to whether there is an inflammation of the nerves that has led to the symptoms. The final certainty as to whether the tumor is benign or malignant can be provided by a biopsy followed by a subtle examination. Ultimately, the only effective treatment option is complete surgical removal of the tumor.
Complications
In plexus papilloma, complications usually occur only when 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, it can lead to high pressure in the brain and thus to failures of the various brain areas. As a result, the affected person usually suffers from paralysis and other motor disorders. Likewise, the high pressure in the brain also causes severe headaches and, not infrequently, vomiting and nausea. Sufferers themselves also suffer from cramps and significantly increased irritability. Hydrocephalus can also develop as a result of the plexus papilloma if the water is not removed from the brain. Without treatment, the brain will be irreversibly damaged by the plexus papilloma. Treatment is usually not associated with any particular complications. The tumor can be removed by surgery. There are usually no further complaints. Affected individuals may require radiation therapy after treatment. If treatment is successful, the patient’s life expectancy is not affected.
When should you see a doctor?
Because plexus papilloma is a tumor, it must always be examined and treated by a physician. There is no self-healing with this disease and usually death of the affected person 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 in case of plexus papilloma when there is a formation of a lump on the head of the affected person. Patients suffer from headaches and nausea, and these symptoms occur for no particular reason and do not disappear on their own. In this case, painkillers cannot relieve the pain either. Likewise, hydrocephalus indicates plexus papilloma and should always be examined by a physician. In the further course, motor deficits also occur, which also indicate the brain tumor. The examination and diagnosis is usually done with the help of an MRI. However, further treatment is performed in a hospital by surgical intervention. Whether the patient’s life expectancy is reduced by the plexus papilloma cannot generally be predicted.
Treatment and therapy
Because there is no way to cause a plexus papilloma to die by medication or other means, the only effective treatment available in the case of a clear diagnosis is complete surgical removal of the papilloma. Only in a few cases can significant relief of symptoms already be achieved by artificially draining the cerebrospinal fluid to reduce intracranial pressure. Specialized centers are able to completely remove the tumor as gently as possible by microsurgical means or endoscopically. The aim is also to restore the circulation of cerebrospinal fluid by reopening possible drains. During the operation, so-called neuronavigation and imaging procedures 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 radiation therapy is necessary and useful.
Prevention
Because no clear trigger factors for the development of plexus papilloma are known to date, and viral diseases or genetic predispositions have not been proven to be causative factors, there are no preventive measures that could prevent disease from the tumor. However, because mostly infants and children under 12 years of age are affected, persistent and recurrent symptoms in children, such as headache, malaise, and possible personality changes that cannot be attributed to other diseases, should also be well clarified neurologically.
Aftercare
In most cases, only a few or limited measures of direct aftercare are available to those affected by plexus papilloma. Therefore, the affected person should seek medical attention at the first symptoms or signs of the disease to prevent the occurrence of further symptoms or complications. There can be no self-healing, so treatment by a physician is always necessary. In most cases, those affected have to undergo surgical intervention. The child should take it easy and rest afterwards. Efforts or stressful and physical activities should be refrained from in order not to put unnecessary strain on the body. Regular checks and examinations by a doctor are also necessary after a successful operation in order to detect and remove further tumors at an early stage. Children need to be supported especially by their parents and relatives during the therapy. This can also prevent possible psychological upsets or depression. If the plexus papilloma is detected early and treated properly, the affected person usually does not experience a reduced life expectancy.
What you can do yourself
Plexus papilloma occurs in children and infants. By their very nature, these individuals are not sufficiently capable of taking self-help measures that would result in a cure. Therefore, legal guardians, relatives or close people from the social environment are increasingly responsible to achieve an improvement of the situation in the interest of the child by taking different approaches. An open approach to the disease is indicated in everyday life. Risk factors and the entire circumstances should be explained to the child sufficiently and understandably. Open questions should be answered honestly and informatively. This behavior prevents irritation or unpleasant surprises. If there is a sufficient flow of information, it is often easier to deal with the illness. Since a hospital stay is necessary during brain tumor treatment, measures should be taken to promote well-being and enjoyment of life. Play and fun should be included daily within the scope of possibilities. A positive basic attitude of the adults has a good effect on the child. It has a motivating effect and reduces misperceptions as well as fears. Contact with peers and also with other patients can be perceived as pleasant by the child. Mutual exchange of accumulated experiences creates opportunities for better coping with the overall situation.
