The rare tumor of the pineal gland is called pinealoma. The tumors can arise from the secretory nerve cells of the pineal gland, from the stellate cells or from the germ cells of the central nervous system. The three classic forms of treatment for malignant tumors, surgical removal, radiotherapy and chemotherapy, are available as therapy for the mostly slow-growing pinealoma.
What is a pinealoma?
Pinealoma or pineal tumor is the collective term for three different malignant tumors of the pineal gland (pineal gland). The secretory pineal gland, or epiphysis, is located in the epithalamus, a part of the diencephalon. Their main task is to synthesize melatonin, which intervenes in the circadian day-night rhythm and in the seasonal rhythm. See dictionaryforall for 22q11 Microdeletion Syndrome in Dictionary.
The three types of pinealoma differ in their origin, which can be from the melatonin-producing cells (pinealocytes), from the stellate cells (astrocytes) or from the germ cells of the CNS. The designation of the pinealoma is then correspondingly 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 the stellate cells are the most common tumors of the central nervous system. Astrocytes are assigned to glial cells as part of the supporting tissue, so that astrocytomas form a subgroup of gliomas.
Causes
The causes of the various pinealomas have not (yet) been adequately researched. There is secure knowledge that the proliferation of individual pinealocytes, astrocytes or germ cells no longer “obeys” the controlling signals, i.e. they develop more or less autonomously. Depending on the cell type and tumor type, self-contained accumulations of cells or neoplasms develop and grow, which release individual “degenerate” cells into the bloodstream or into the lymph.
If the immune system is not able to identify the degenerated cells “marauding” in the blood or in the lymph in order to render them harmless by means of phagocytosis, malignant daughter tumors develop which can no longer be surgically removed individually. Astrocytomas are also caused by unchecked proliferation of astrocytes. The causes probably lie in altered genetics of the cell, which is the subject of scientific research.
Symptoms, Ailments & Signs
Pinealomas are usually not discovered in the early stages because there are no specific symptoms. The first symptoms that appear can be traced back to displacement compressions of the surrounding nerve tissue. The growing pinealoma takes up space and can lead to an unspecific increase in intracranial pressure because the balancing mechanisms in terms of blood volume and cerebrospinal fluid (liquor) in the intracranial space are overstrained.
Typical first signs are headache, malaise and vomiting. In the course of an untreated pinealoma, specific symptoms such as gait disorders (ataxia) appear in addition to the non-specific symptoms. In many cases, the pinealoma presses on the visual center in the tectum of the midbrain (four hillocks plate).
This leads to 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. A pronounced increase in intracranial pressure can also lead to the formation of a hydrocephalus (hydrocephalus) due to obstruction of the liquor circulation.
Pinealoma, which is characterized by a disruption in melatonin synthesis, typically causes disruptions in the normal day-night rhythm. In some cases, hormonal imbalances have also been observed, which can lead to a premature initiation of puberty (Pubertas praecox).
Diagnosis & course of disease
Due to the only minor symptoms that emanate from a pinealoma in the early stages, the tumor is usually only discovered at an advanced stage. This applies equally to pineoblastomas, astrocytomas and germinomas. Magnetic resonance imaging (MRI) is the best diagnostic tool.
Other diagnostic tools such as biopsy, i.e. direct intracranial tissue removal, fail due to the difficult access to the pinealis and the excessive risks that such an intervention would entail. Although the imaging procedure usually delivers good to very good results, other diseases such as malformations of the inner cranial nerves or other tumors must be excluded in the differential diagnosis.
If left untreated, malignant pinealoma with variable progression has an unfavorable prognosis. Of the three possible types of pinealoma, pineoblastomas show the highest degree of malignancy and the worst prognosis. Germinomas have a lower grade of malignancy and develop more slowly. Their danger lies in their locally effective invasiveness.
Complications
Because pinealoma is a tumor, the disease has the usual symptoms and complications of cancer. In the worst case, this can even lead to the death of the affected person if the tumor spreads to other regions of the body. For this reason, a general prediction cannot be given.
Those affected suffer from severe headaches due to the pinealoma, since the intracranial pressure is significantly increased. A general malaise and nausea or vomiting can also be caused by this tumor and further reduce the patient’s quality of life. Furthermore, this disease leads to gait disturbances and personality changes. Depression or other psychological upsets can also occur as a result of the permanent headache.
Furthermore, it comes to breathing difficulties and reduced exercise capacity of the patient. The pinealoma is treated with the help of a surgical procedure. There are no particular complications. However, a general course cannot be predicted. This disease may also reduce the patient’s life expectancy. Those affected are dependent on chemotherapy or radiation.
When should you go to the doctor?
If visual problems such as paralyzed vision or blurred vision are noticed, medical advice is required. The signs of the disease suggest pinealoma or another serious condition that needs to be diagnosed and treated. Since pinealoma is a tumor disease, prompt treatment is vital. It is best for those affected to consult their doctor directly if they experience disturbances in their field of vision or neurological deficits.
People suffering from a germ cell tumor or an astrocytoma are among the risk groups and should contact the responsible doctor with the symptoms mentioned. In addition to the neurologist, the dermatologist, an internist or an oncologist can be consulted. Treatment is surgical and uses radiation or chemotherapy. After the procedure, the check-ups recommended by the doctor must be carried out so that any complications such as recurrences or metastases can be recognized quickly. In the case of unusual symptoms, medical advice is required immediately, because the medication may have to be changed or further treatment carried out.
Treatment & Therapy
Only the three classic forms of therapy for malignant tumors can be used to treat a pinealoma. These are the neurosurgical removal of the growth as well as radiotherapy and chemotherapy. However, due to the location of the pinealis in the diencephalon, tumor removal poses some challenges.
In many cases, a CSF shunt is necessary to reduce 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 possible metastases.
Outlook & Forecast
In most cases, the further course of a pinealoma is negative and in many cases also significantly reduces the life expectancy of the affected person. For this reason, with this disease, a doctor must be consulted as early as possible and treatment must also be carried out so that there are no further complaints or other compilations. If the pinealoma is not treated, the tumor usually spreads throughout the body of the affected person and ultimately leads to the death of the patient. Therefore, the patient needs to be diagnosed and treated as early as possible.
The tumor is usually treated by surgery or radiation. The further course depends very much on the time of diagnosis, so that a general prediction cannot be made here. The tumor can reappear even after successful treatment, so that a new treatment will then be necessary. Since pinealoma may also have a genetic origin, genetic testing and counseling should always be carried out if the patient or children wish to have children in order to prevent the disease from recurring.
Prevention
The causes of the formation of a pineoblastoma, astrocytoma or germinoma in the pineal gland have not yet been conclusively researched. There is also no reliable knowledge of the extent to which a genetic predisposition or gene mutations can play a role. Direct preventive measures to avoid a pinealoma are therefore non-existent.
In principle, all measures that strengthen the immune system are recommended. An optimized immune system may be able to recognize degenerated cells of the pinealis, from which a pinealoma could develop, and render them harmless through phagocytosis.
Aftercare
In most cases, those affected with a pinealoma have only a few and only limited measures of direct aftercare available. For this reason, the affected person should consult a doctor at an early stage so that no further complications or symptoms develop that could have a negative impact on the quality of life and life expectancy of the person affected.
A pinealoma cannot heal itself, so 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 is also necessary in everyday life in order to relieve the affected person during the treatment.
Even after successful treatment, regular check-ups by a doctor are very important in order to identify and remove other tumors at an early stage. In many cases, psychological support for the person concerned is also necessary. This can also prevent depression and other psychological complaints and upsets. Pinealoma can reduce the life expectancy of those affected if it is detected late. Therefore, no general course can be given.
You can do that yourself
Once the diagnosis of pinealoma has been made, the patient has to undergo massive interventions: 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 by the illness and the therapies that have been initiated.
In order to deal with the altered sleep-wake rhythm, the possible hormonal imbalances and headaches, clear medication management is recommended, which has been agreed with the doctor treating the patient and which the patient strictly adheres to. In principle, all measures that strengthen the patient ‘s immune system are still recommended during this time. A well-functioning immune system may be able to recognize and destroy the degenerated cells of the pinealis. There are several ways to strengthen the immune system. Naturopathically trained physicians and naturopathsrecommend detoxification measures with homeopathic remedies and colon cleansing (colon hydrotherapy). In this way, the intestine is sanitized, which is now known to contain 80 percent of all immune cells.
During this time, the patient should also take more probiotics. These are preparations that contain living microorganisms that multiply in the intestine and are supposed to care for the immune system there. Experience has shown that dietary supplements or medicines contain more microorganisms than the probiotic yoghurts that you can buy 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 the convalescence period: A healthy, varied diet and moderate exercise are necessary to stabilize the immune system. The daily walk in the fresh air activates the immune cells and thus contributes to recovery.