Pinealoma is the name given to the rare tumor of the pineal gland. The tumors may originate from the secretory neurons of the pineal gland, stellate cells, or germ cells of the central nervous system. Therapy for the usually slow-growing pinealomas includes the classic three forms of treatment for malignant tumors, surgical removal and radiation and chemotherapy.
What is a pinealoma?
Pinealoma or pineal tumor is the collective term for three different malignant tumors of the pineal gland. The secretory pineal gland or epiphysis is located in the epithalamus, a part of the diencephalon. Its main function is to synthesize melatonin, which interferes with the circadian day-night rhythm and the seasonal rhythm. The three types of pinealomas are distinguished by their origin, which may be from melatonin-producing cells (pinealocytes), stellate cells (astrocytes), or CNS germ cells. The name of the pinealoma is then accordingly pineoblastoma or pinealoblastoma, astrocytoma or germ cell tumor or germinoma. In contrast to the malignant pineoblastoma is the pineocytoma, a benign tumor that can also develop from the parenchyma, the secretory nerve cells of the pinealis, and can be differentiated from the pineoblastoma in terms of differential diagnosis. Astrocytomas arising from stellate cells are the most common tumors of the central nervous system. Astrocytes are classified as part of the supporting tissue of glial cells, making astrocytomas a subgroup of gliomas.
Causes
The causes of the various pinealomas are not (yet) adequately understood. There is established knowledge that the proliferation of individual pinealocytes, astrocytes, or the germ cells no longer “obey” controlling signals, i.e., they develop more or less autonomously. Depending on the cell type and tumor type, self-contained cell collections or neoplasms develop and grow, releasing individual “degenerate” cells into the bloodstream or lymph. If the immune system is unable to identify the degenerate cells “marauding” in the blood or lymph to render them harmless by phagocytosis, malignant daughter tumors develop that can no longer be surgically removed individually. Astrocytomas also result from unchecked proliferation of astrocytes. The causes probably lie in altered genetics of the cell, which is the subject of scientific research.
Symptoms, complaints, and signs
In most cases, pinealomas are not detected in the early stages because no specific symptoms present themselves. The first symptoms that appear can be attributed to displacement compression of the surrounding nerve tissue. The growing pinealoma occupies space and can lead to an unspecific increase in intracranial pressure because the compensatory mechanisms with regard to blood volume and cerebrospinal fluid (CSF) in the intracranial space are overtaxed. Typical first signs are headache, malaise and vomiting. In the course of an untreated pinealoma, specific symptoms such as gait disturbances (ataxia) present themselves in addition to the non-specific symptoms. In many cases, the pinealoma presses on the center of vision in the tectum of the midbrain (four mound plate). As a result, there are deficits and a typical vertical gaze paralysis, the so-called Parinaud syndrome. Depending on the location of the pinealoma in the pineal gland, other neurological deficits and symptoms also occur. In the case of a pronounced increase in intracranial pressure, the formation of hydrocephalus may also occur due to obstruction of the cerebrospinal fluid circulation. Pinealomas, which are characterized by a disturbance in melatonin synthesis, typically cause disturbances in the normal day-night rhythm. In some cases, hormonal disturbances have also been observed, which can initiate premature onset of puberty (pubertas praecox).
Diagnosis and course of the disease
Because of the minimal discomfort caused by early-stage pinealoma, the tumor is usually not discovered until it is advanced. This is equally true for pineoblastomas, astrocytomas, and germinomas. Magnetic resonance imaging (MRI) is considered the best diagnostic tool. Other diagnostic tools such as biopsy, i.e. direct intracranial tissue sampling, fail due to difficult accessibility of the pinealis and the excessive risks that such an intervention would entail.Although imaging usually provides good to very good results, differential diagnosis must exclude other diseases such as malformations of internal cranial nerves or other tumors. If left untreated, malignant pinealomas with varying degrees of progression have an unfavorable prognosis. Of the three possible types of pinealoma, pineoblastomas show the highest degree of malignancy and the most unfavorable progression prognosis. Germinomas have a lower degree of malignancy and develop more slowly. Their danger lies in their locally effective invasiveness.
Complications
Because pinealoma is a tumor, it presents with the usual symptoms and complications of cancer. In the worst case, this can also lead to the death of the affected person if the tumor also spreads to other regions of the body. For this reason, a general prediction cannot be made. The affected person suffers from severe headaches due to the pinealoma, as the intracranial pressure is significantly increased. General malaise and nausea or vomiting may also occur due to this tumor, further reducing the patient’s quality of life. Furthermore, this disease leads to gait disturbances and personality changes. Depression or other psychological upsets can also occur due to the permanent headaches. Furthermore, there are breathing problems and a reduced resilience of the patient. Pinealoma is treated with the help of a surgical procedure. There are no particular complications. However, a general course cannot be predicted. It is also possible that this disease reduces the life expectancy of the patient. Affected individuals are dependent on chemotherapy or radiation.
When should you see a doctor?
When visual symptoms such as visual field paralysis or blurred vision are noticed, medical advice is needed. The signs of illness indicate pinealoma or another serious condition that needs to be diagnosed and treated. Because pinealoma is a tumor disease, prompt treatment is vital. Sufferers are best to consult the doctor directly if disturbances in the field of vision or neurological deficits occur. Persons suffering from a germ cell tumor or an astrocytoma belong to the risk groups and should consult the responsible physician with the mentioned complaints. In addition to the neurologist, the dermatologist, an internist or an oncologist can be consulted. The treatment is performed surgically and by means of radiotherapy or chemotherapy. After the operation, the control examinations suggested by the doctor must be taken up, so that possible complications such as recurrences or metastases can be detected quickly. If unusual symptoms occur, medical advice is needed immediately, because medication may need to be changed or further treatment given.
Treatment and therapy
Only the three classic forms of therapy for malignant tumors are used to treat a pinealoma. These are neurosurgical removal of the growth and radiation therapy and chemotherapy. However, tumor removal presents some challenges due to the location of the pinealis in the diencephalon. In many cases, placement of a cerebrospinal fluid shunt is necessary to relieve intracranial pressure. While neurosurgical removal of the tumor and radiation are locally effective, chemotherapy can be described as a systemic form of treatment that also aims to kill potential metastases.
Outlook and prognosis
In most cases, the further course of pinealoma is negative and in many cases also significantly reduces the life expectancy of the affected person. For this reason, a doctor must be consulted as early as possible in this disease and treatment must also be carried out, so that there is no discomfort or other compilations in the further course. If the pinealoma is not treated, the tumor usually spreads throughout the entire body of the affected person and eventually leads to the death of the patient. Therefore, the patient is dependent on the earliest possible diagnosis and treatment. The tumor is usually treated by surgery or by radiation therapy. The further course of the disease depends very much on the time of diagnosis, so that a general prediction cannot be made.In this case, the tumor may recur even after successful treatment, so that renewed treatment will then be necessary. Since pinealoma may also have a genetic origin, if the patient or children wish to have children, genetic testing and counseling should always be performed to prevent the recurrence of this disease.
Prevention
The causes of pineoblastoma, astrocytoma, or germinoma formation in the pineal gland have not been conclusively studied. There is also no firm understanding of the extent to which a genetic predisposition or gene mutations may play a role. Direct preventive measures to avoid pinealoma are therefore non-existent. Basically, all measures that strengthen the immune defense are recommended. An optimized immune system may be able to recognize degenerate cells of the pinealis that could develop into a pinealoma and render them harmless by phagocytosis.
Follow-up
In most cases, the person affected by a pinealoma has only a few and also limited measures of direct aftercare available. For this reason, the affected person should consult a physician at an early stage to prevent the development of further complications or complaints, which can have a negative impact on the quality of life and also on the life expectancy of the affected person. Self-healing cannot occur with a pinealoma, so that treatment by a doctor is always necessary. Most of those affected are dependent on chemotherapy or radiation therapy. In many cases, the support and help of one’s own family in everyday life is necessary to relieve the affected person during the treatment. Even after successful treatment, regular check-ups by a doctor are very important in order to detect and remove other tumors at an early stage. In many cases, psychological support for the affected person is also necessary. This can also prevent depression and other psychological complaints and upsets. Pinealoma can limit the life expectancy of the affected person if it is detected late. Therefore, no general course can be given in this case.
What you can do yourself
Once the diagnosis of pinealoma has been made, massive interventions await the patient: surgery, radiotherapy, and/or chemotherapy. During this time, it is important to allow yourself and your body to rest so that it is not overwhelmed with the disease and the therapies that have been initiated. In order to deal with the altered sleep-wake rhythm, possible hormonal disturbances and headaches, it is advisable to have a clear medication management, which is agreed with the attending physician and which is absolutely adhered to by the patient. In principle, all measures that strengthen the patient’s immune system are still recommended during this time. A well working immune system can be able to recognize and destroy the degenerated cells of the pinealis. There are several ways to strengthen the immune system. Naturopathic doctors and alternative practitioners recommend detoxification measures with homeopathic remedies and intestinal cleansing (colon hydrotherapy). In this way, the intestine is sanitized, of which it is now known that 80 percent of all immune cells are located in it. During this time, the patient should also make increased use of probiotics. These are preparations containing living microorganisms that multiply in the intestine and are intended to maintain the immune system there. Experience has shown that dietary supplements or medications contain more microorganisms than the probiotic yogurts available in supermarkets. Pharmacists can make detailed statements here and also recommend other dietary supplements that can support the immune system. Even if it is difficult during convalescence, stabilizing the immune system involves a healthy, varied diet and moderate exercise. A daily walk in the fresh air activates the immune cells and thus contributes to recovery.