Papillary tumors of the pineal region are very rare brain tumors that typically form on the back wall of the third cerebral ventricle. One of the main problems that a papillary tumor of the pineal region causes is its location. It means that even after little growth, the circulation and drainage of the cerebrospinal fluid is usually impeded, so that increased intracranial pressure sets in with the well-known non-specific symptoms such as headaches, malaise, and vomiting. Treatment is aimed at complete removal of the tumor.
What is a papillary tumor of the pineal region?
A papillary tumor of the pineal region (PTPR) is a very rare brain tumor and forms primarily on the back wall of the 3rd cerebral ventricle. The PTPR can also be regarded as a special form of a pinealoma, a tumor of the epiphysis or pineal gland (glandula pinealis). It primarily causes problems because of its bulkiness because its physical presence impedes the circulation and drainage of the cerebrospinal fluid (Liquor cerebrospinalis). See ezhoushan for What does Bacterial Endocarditis Stand for.
This leads to a congestion of the cerebrospinal fluid and an increase in intracranial pressure with unspecific but symptomatic complaints. Due to its papillary structure, the outer shell of a PTPR shows an epithelial-like character. The papillary tumor of the pineal region mainly affects children and adolescents. A definitive description of the tumor was given for the first time in 2003. The degree of malignancy of the PTPR is given as II to III according to the WHO classification. The WHO classification of tumors for malignancy ranges from I to IV, with IV being classified as the most aggressively growing and malignant tumors.
The tissue of the papillary tumor of the pineal region originates from the ectodermis, the third germ layer, namely the neuroectodermal part, from which all nerve tissue of the peripheral and central nervous system develops. The origin of the tumor probably goes back to degenerated ependymal cells of the organum subcommissurale. Ependymal cells form a thin layer of epithelium that lines the ventricles of the brain and the central canal of the spinal cord.
The organum subcommissurale is located at the transition from the third to the fourth brain ventricle and is involved in the secretion and reabsorption of the liquor and forms a small region that manages the transport of substances between the brain and blood, i.e. it crosses the blood-brain barrier in a controlled manner. The reasons why ependymal tissue develops into a PTPR are not (yet) well understood. In this respect, one can only speculate about the cause of the disease.
Attempts to link the development of a PTPR to genetic abnormalities have so far failed. The extremely rare occurrence of a PTPR obviously prevents research funds from being made available in sufficient measure to understand the connections.
Symptoms, Ailments & Signs
A papillary tumor of the pineal region does not cause any complaints or symptoms in the early stages. Only when a certain size is reached does its unfavorable position at the transition from the third to the fourth ventricle become noticeable and the fact that the subcommissural organ is disturbed in its function of reabsorption of the liquor increases the imbalance between secretion and outflow of the liquor, which leads to an increased intracranial pressure.
Therefore, the non-specific but nevertheless symptomatic complaints arise, such as increasing headache and malaise, which can also lead to vomiting. If the PTPR continues to grow, the tumor presses on the tectum, the roof of the midbrain, and can thereby cause the so-called Parinaud syndrome.
These are neurological deficits associated with voluntary and reflexive eye movements such as vertical gaze paralysis, pathological nystagmus (eye tremors) and similar symptoms. Because the tumor also triggers mechanical pressure on the pituitary gland, the release of melatonin, which regulates the sleep-wake cycle, is disrupted.
Diagnosis & course of disease
The diagnosis of PTPR is not very easy because there are no symptoms in the early stages and the first symptoms that appear, which are based on increased intracranial pressure, can also have other causes. In addition, there were occasional misdiagnoses because PTPR resembles tumors of the choroid plexus and papillary ependymomas.
When PTPR is suspected, contrast-enhanced magnetic resonance imaging (MRI) has become established as the diagnostic imaging method. As a final confirmation of a diagnosis, a direct examination of the tumor tissue can be carried out, which can be obtained via a biopsy. The location of the PTPR does not always allow for a biopsy due to individual anatomical conditions.
In many cases, this tumor is unfortunately only recognized relatively late, so that early diagnosis and treatment is usually not possible. Those affected primarily suffer from severe headaches that occur for no particular reason and usually cannot be alleviated with the help of painkillers. Vomiting or permanent discomfort can also occur and have a very negative effect on the quality of life of those affected.
If there is no treatment, the tumor usually spreads to other regions of the brain, which can lead to limitations in everyday life or to paralysis and personality changes. Gaze paralysis can also occur, so that the patients are dependent on the help of other people in their everyday lives.
This tumor is treated with surgical removal. There are no complications. However, further chemotherapy does not work, so that those affected are dependent on radiotherapy. Even in this case, complications are not to be expected. However, those affected are dependent on regular check-ups even after the treatment. It cannot be predicted whether the disease will lead to a reduction in life expectancy.
When should you go to the doctor?
If there are symptoms such as feeling unwell, a decrease in resilience and a diffuse feeling of illness, a doctor should be consulted. In the case of headaches or a feeling of pressure inside the head, it is necessary to clarify the cause. If the symptoms persist or gradually increase in intensity, you should see a doctor. Paralysis, eye movement disorders or general functional disruption must be examined and treated. The disease is characterized by vertical gaze paralysis in those affected.
If it occurs, it is advisable to see a doctor immediately. Without medical care, the tumor in the brain enlarges and the risk of a life-threatening situation increases. A reduced quality of life, withdrawal from social and community activities or a slow change in personality are to be understood as warning signals from the organism. There is a health impairment that requires action.
If there are abnormalities in behavior, sleep disorders or irregularities in the sleep-wake cycle, the person concerned needs medical help. If mental performance decreases continuously, everyday professional and school obligations can no longer be fulfilled as usual, or if frustration, aggression or feelings of shame show up, a doctor is needed. In the case of apathy, changes in weight and psychosomatic disorders of the digestive tract, a visit to the doctor is recommended.
Treatment & Therapy
Erstes Ziel bei der Behandlung eines papillären Tumors der Pinealisregion ist seine chirurgische Entfernung. Die vollständige Resektion des PTPR unter Anwendung mikrochirurgischer oder endoskopischer Verfahren stößt allerdings auf große technische Herausforderungen aufgrund der anatomischen Lage des Tumors am Übergang vom dritten zum vierten Gehirnventrikel und ist nicht immer möglich. Es hat sich gezeigt, dass Tumoren diesen Typs nicht auf Cytostatika zur Hemmung des Wachstums reagieren, so dass eine begleitende Chemotherapie meist ausscheidet.
Radiation therapy remains the only alternative or additional therapy to surgical resection. A well-dosed and targeted irradiation of the tumor tissue or possible remnants of the tumor tissue that cannot be surgically removed are usually effective in terms of successful treatment and healing. The only disadvantage is that even successful irradiation cannot prevent possible recurrences of the PTPR, so that postoperative observation over a longer period of time is indicated.
Outlook & Forecast
The prognosis for a papillary tumor in the pineal region must be evaluated according to the individual circumstances. The location of the resulting tumor often causes difficulties and complications. If the course of the disease is favorable, the tumor can be detected early and completely removed by surgery. Cancer therapy is then applied. Even under optimal conditions, this is associated with various risks and side effects. The quality of life of the patient is immensely limited. Still, full recovery can occur over the long term.
In most patients, however, the tumor is only diagnosed at an advanced stage. In addition, its location is often suboptimal. This causes problems in removing the unwanted tissue change in the brain. Radiation therapy can stop the tumor from growing or start regression.
If, despite all efforts, it is not possible to completely remove the papillary tumor, the patient may die prematurely. The same development can be expected if no medical treatment is sought. The tumor gradually increases in size. In addition, cancer cells can be transported to other parts of the body via the bloodstream. There, metastases form and ultimately further tumors develop. For this reason, the earliest possible diagnosis is crucial for a good prognosis.
There is no reliable scientific knowledge about the causes that lead to the development of a PTPR, and approaches to associate the tumors with genetic abnormalities and mutations have not led any further. Therefore, no preventive measures are known that could prevent the formation of a papillary tumor in the pineal region.
Follow-up care is part of any tumor treatment. Cancerous growths can form again after a successful therapy and shorten the patient’s life span. Doctors hope that starting treatment in the early stages will give them the best chance of recovery. This is why follow-up care always takes place after the treatment of a papillary tumor in the pineal region.
This tends to form metastases, which often turns out to be life-threatening. Even before the end of the initial therapy, the doctor and patient agree on the location and scope of the follow-up checks. A clinic and the practice of a resident doctor come into question. Follow-up visits take place quarterly for the first year after diagnosis. Then the rhythm widens. From the fifth year of being symptom-free, annual follow-up care is sufficient.
The papillary tumor of the pineal region requires rehabilitation immediately after the initial therapy. This takes place on an outpatient or inpatient basis. Experts help to reintegrate into the social environment. Medication will also be discontinued if necessary.
Every follow-up examination includes a detailed discussion in which the doctor asks about the general condition. In addition, he uses magnetic resonance imaging as an imaging diagnostic method, which provides information about the current status. Some doctors will also order a biopsy.
You can do that yourself
Patients who have been diagnosed with a papillary tumor of the pineal region initially require close specialist monitoring. At the same time, it is important to reconcile everyday life and illness.
First of all, the diet has to be changed, as certain foods are no longer allowed to be eaten. Bladder cancer, for example, does not allow for foods and drinks that are too salty. In addition, stimulants such as alcohol and nicotine should be avoided. It is important to avoid further diseases of the urinary tract. In winter, adequate clothing and a warm home are important. In summer, allergic reactions and contact with UV radiation should be avoided as far as possible. Moderate sport may be practiced in consultation with the medical specialist. In the case of a papillary tumor in the pineal region, swimming and upper body training are recommended. Gymnastic exercises as well as yoga or Pilates may also be carried out.
In addition, care should be taken to ensure that no unusual symptoms occur. In any case, the doctor must be informed about complaints that indicate a complication of the tumor. Doctors, psycho-oncologists and counseling centers support tumor patients on their way by giving tips and acting as advisors.