Papillary tumors of the pineal region are very rare brain tumors that typically form on the posterior wall of the third ventricle of the brain. One of the main problems caused by a papillary tumor of the pineal region is its location. It leads to the fact that even after small growth usually circulation and drainage of cerebrospinal fluid is obstructed, so that increased intracranial pressure occurs with the well-known nonspecific symptoms such as headache, malaise, vomiting. Complete removal of the tumor is targeted as therapy.
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 predominantly on the posterior wall of the 3rd ventricle of the brain. PTPR can also be considered a specific form of pinealoma, a tumor of the pineal gland or pineal gland. It primarily causes problems due to its need for space, because its physical presence obstructs the circulation and drainage of cerebrospinal fluid (CSF). This results in cerebrospinal fluid congestion and an increase in intracranial pressure with nonspecific but symptomatic symptoms. The outer envelope of a PTPR shows an epithelial-like character due to its papillary structure. The papillary tumor of the pineal region predominantly affects children and adolescents. A defining description of the tumor was first given in 2003, and the malignancy grade of PTPR is reported as II to III according to WHO classification. The WHO classification for tumors in terms of malignancy ranges from I to IV, with tumors with the most aggressive growth and high malignancy classified under IV.
Causes
The tissue of the papillary tumor of the pineal region originates from the ectodermis, the third cotyledon, and specifically the neuroectodermal part from which all nervous 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 epithelial layer that acts as a covering layer lining the cerebral ventricles and the central canal of the spinal cord. The organum subcommissurale is located at the junction of the third and fourth cerebral ventricles and is involved in the secretion and reabsorption of cerebrospinal fluid, forming a small region that accomplishes the transport of substances between the brain and the blood, thus crossing the blood-brain barrier in a controlled manner. The reasons why ependymal tissue develops into a PTPR are not (yet) sufficiently understood. In this respect, the causation of the disease can only be speculated. Approaches to link the development of a PTPR to genetic abnormalities have failed so far. The exceedingly rare occurrence of PTPR apparently prevents research funds from being made available on a sufficient scale to understand the relationships.
Symptoms, complaints, and signs
A papillary tumor of the pineal region does not cause any complaints or symptoms in the early stages. Only when it reaches a certain size does its unfavorable location at the junction of the third and fourth ventricles become noticeable, and the fact that the subcommissural organ is disturbed in its function of reabsorbing CSF increases the imbalance between secretion and outflow of CSF, resulting in increased intracranial pressure. Therefore, the nonspecific but nevertheless symptomatic complaints such as increasing headache and malaise, which may also lead to vomiting, present themselves. As the PTPR continues to grow, the tumor presses on the quadruple plate (tectum), the midbrain roof, and can thereby cause the so-called Parinaud syndrome. This is neurological deficits related to voluntary and reflex eye movements, such as vertical gaze paralysis, pathological nystagmus (eye tremor), and similar symptoms. Because the tumor also triggers mechanical pressure on the pituitary gland, melatonin secretion, a regulator of the sleep-wake rhythm, is disrupted.
Diagnosis and disease progression
The diagnosis of PTPR is not very easy because no symptoms are apparent in the early stages, and the first symptoms that appear, which are due to increased intracranial pressure, may have other causes. In addition, there have been occasional misdiagnoses because a PTPR has similarities to tumors of the choroid plexus and to papillary ependymomas.When PTPR is suspected, contrast-enhanced magnetic resonance imaging (MRI) has become the accepted diagnostic imaging technique. Direct examination of tumor tissue, which can be obtained via biopsy, can be performed as final confirmation of a diagnosis. The location of the PTPR does not always allow for biopsy due to individual anatomic conditions.
Complications
In many cases, this tumor is unfortunately detected relatively late, so early diagnosis and treatment are usually not possible. Those affected suffer primarily from severe headaches that occur for no particular reason and usually cannot be relieved with the help of painkillers. Vomiting or a permanent feeling of malaise can also occur and have a very negative effect on the quality of life of the affected person. If there is no treatment, the tumor usually spreads to other regions of the brain, so that restrictions in everyday life or paralysis and personality changes can occur. Gaze paralysis can also occur, so that patients are dependent on the help of other people in their daily lives. The treatment of this tumor is surgical removal. Complications do not occur. However, further chemotherapy is not effective, so that the patients are dependent on radiation therapy. In this case, too, complications are not to be expected. However, even after treatment, patients are dependent on regular check-ups. Whether there is a reduction in life expectancy as a result of the disease cannot be predicted.
When should you see a doctor?
If symptoms such as malaise, a decrease in the ability to cope with stress, and a diffuse feeling of illness occur, a physician should be consulted. In case of headache or a feeling of pressure inside the head, clarification of the cause is necessary. If the complaints persist or gradually increase in intensity, a visit to the doctor is necessary. Paralysis, disturbances of the eye movements or general interruptions of the functional ability have to be examined and treated. Characteristic of the disease is vertical gaze paralysis in the affected person. If it occurs, a visit to a physician is advised immediately. Without medical care, the tumor in the brain enlarges and the risk of life threat increases. Reduced quality of life, withdrawal from social as well as societal activities or a slow change of personality are to be understood as warning signals of the organism. There is a health impairment where there is a need for action. If there are abnormalities in behavior, sleep disturbances or irregularities in the sleep-wake rhythm, the affected person needs medical help. If the mental performance decreases continuously, the daily professional as well as school obligations can no longer be fulfilled as usual or frustration, aggression or feelings of shame appear, a doctor is needed. In case of apathy, changes in weight as well as psychosomatically caused disorders of the digestive tract, a visit to the doctor is recommended.
Treatment and therapy
The first goal in the treatment of a papillary tumor of the pineal region is its surgical removal. However, complete resection of the PTPR using microsurgical or endoscopic procedures encounters major technical challenges because of the anatomic location of the tumor at the junction of the third and fourth cerebral ventricles and is not always possible. Tumors of this type have been shown not to respond to cytostatic drugs to inhibit growth, so concomitant chemotherapy is usually ruled out. Radiation therapy remains as the only alternative or additional therapy to surgical resection. Well-dosed and targeted radiation of the tumor tissue or possible remnants of the tumor tissue that could not be surgically removed are usually effective in terms of successful treatment and cure. The only drawback is that even successful irradiation cannot prevent possible recurrences of PTPR, so postoperative observation for a longer period is indicated.
Outlook and prognosis
The prognosis for a papillary tumor of the pineal region must be evaluated according to individual circumstances. The location of the resulting tumor often provides difficulties and complications.If the course of the disease is favorable, the tumor can be detected early and completely removed by surgery. Subsequently, a cancer therapy is applied. Even under optimal conditions, this is associated with various risks and side effects. The patient’s quality of life is immensely limited. Nevertheless, a complete recovery can occur in the long term. In most patients, however, the tumor is diagnosed only at an advanced stage. In addition, its location is often suboptimal. This leads to problems in removing the unwanted tissue change in the brain. Radiotherapy can prevent the tumor from growing or induce regression. If, despite all efforts, complete removal of the papillary tumor is not possible, the premature death of the affected person is imminent. The same development is to be expected if no medical treatment is taken. The size of the tumor gradually increases. Moreover, cancer cells can be transported to other parts of the organism through the bloodstream. There, the formation of metastases and ultimately the development of further tumors occurs. For this reason, the earliest possible diagnosis is crucial for a good prognosis.
Prevention
No robust scientific evidence is available on the causes that lead to the development of PTPR, and approaches linking the tumors to genetic abnormalities and mutations have not led anywhere. Therefore, no preventive measures are known to prevent the development of a papillary tumor of the pineal region.
Follow-up
Follow-up is a part of any tumor treatment. Cancerous tumors can reform after successful therapy and shorten the patient’s lifespan. Doctors hope that starting treatment at an early stage will give patients the best chance of recovery. Therefore, even after treatment of a papillary tumor of the pineal region, there is always follow-up. This tumor tends to metastasize, which not infrequently proves to be life-threatening. Even before the end of the initial therapy, the physician and the patient agree on the place and extent of follow-up examinations. Both a clinic and the office of a physician in private practice come into question. Follow-up examinations take place every quarter during the first year after diagnosis. After that, the frequency increases. From the fifth year of freedom from symptoms, annual follow-up 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. Specialists help to reintegrate the patient into the social environment. Medication is also adjusted if necessary. Each follow-up examination includes a detailed discussion in which the physician asks about the patient’s general condition. In addition, he uses magnetic resonance imaging as a diagnostic imaging procedure, which provides information about the current actual condition. Some physicians also arrange for a biopsy.
Here’s what you can do yourself
Patients who have been diagnosed with a papillary tumor of the pineal region initially require close specialist monitoring. Accompanying this, it is important to balance everyday life and the disease. First of all, the diet must be changed, as certain foods may no longer be consumed. Bladder cancer, for example, does not allow 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 sports may be practiced in consultation with the specialist physician. In the case of a papillary tumor of the pineal region, swimming and upper body training are recommended. In addition, gymnastic exercises as well as yoga or Pilates may be performed. In addition, care should be taken that no unusual symptoms occur. In any case, the doctor must be informed about complaints that indicate a complication of the tumor condition. Doctors, psychooncologists and counseling centers support tumor patients on their way by giving tips and acting as advisors.
