The medical term for a tumor on an autonomous nerve node in the autonomic nervous system (paraganglion) is a paraganglioma or chemodectrome. Depending on which paraganglion is affected, symptoms and treatment will vary. The tumors tend to run in families.
What is a paraganglioma?
The paraganglioma or chemodectrome is a tumor and develops from an autonomous nerve node of the autonomic nervous system, which medicine also knows as a paraganglion. The neoplasm can be both benign and malignant; in most cases, however, it is a benign tumor. See ezhoushan for What does Internet Addiction Stand for.
Paragangliomas can be divided into different types: Paraganglioma tympanicum forms in the middle ear and mainly affects women around the age of 50, while paraganglioma jugulare is also known as glomus jugulare tumor and occurs in the jugular fossa ossis temporalis at the base of the skull. Also at the base of the skull, but along the jugular foramen, runs the vagus nerve, where the vagal paraganglioma can manifest itself.
Visceral paragangliomas cause cell proliferation on internal organs; they particularly frequently affect the urinary bladder. Paraganglioma aorticum, on the other hand, is an aggressive tumor on the nerve node of the main artery (aorta), which leads to death in half of the cases.
Paragangliomas that form specifically in the abdominal aorta and affect the ganglia of the abdominal cavity are sometimes also known as retroperitoneal paragangliomas. The neoplasm occurs most frequently as paraganglioma caroticum on the carotid artery (arteria carotis).
Causes
The cause of the development of a paraganglioma lies in uncontrolled cell growth. Human cells have a variety of mechanisms that fight damaged or destructive cells. Micro-level control processes, for example, detect damage in the cell’s DNA, after which it can self-destruct; Biology also calls this process apoptosis (“cell suicide”).
The immune system can also intervene. When a tumor develops, this mechanism fails and the cell divides, displacing healthy tissue and causing the corresponding symptoms. In addition to affecting the affected paraganglion, paragangliomas may also affect surrounding tissues.
There is a connection between various triggers and tumor formation, especially for malignant tumors such as those that arise as part of cancer. These include radiation, certain chemicals, viruses, and lifestyle factors such as diet. However, the specific cause can often not be clearly determined individually. The familial phenocytoma paraganglion syndrome has a genetic cause, for which three genes are known to date.
Symptoms, Ailments & Signs
Complaints that typically occur as a result of paraganglioma differ depending on the location of the tumor. Many paragangliomas cause high blood pressure because of the substances they produce and release into the body; However, like all the others, this symptom does not necessarily have to be present. In paraganglioma jugulare, for example, it typically does not manifest itself.
In many cases, the paraganglioma tympanicum leads to hearing problems such as tinnitus, ringing in the ears and limited hearing ability up to deafness. The symptoms manifest themselves primarily on the side on which the tumor is growing. Dizziness and fourth cranial nerve impairment may also occur.
Tinnitus, facial paralysis and dysphagia also belong to the characteristic symptoms of paraganglioma jugulare. If the cranial nerves in this area are affected, their (partial) failure can cause additional symptoms.
Diagnosis & course of disease
Doctors usually use imaging methods for diagnosis in order to locate the paraganglioma precisely, to recognize its extent and, if necessary, to visualize metastases. Magnetic resonance imaging (MRI) with T2 weighting is often suitable because it has a very high spatial resolution. Computed tomography (CT) or positron emission tomography (PET) can also be considered, if necessary as a whole-body scan.
Complications
In most cases, paragangliomas are benign tumors. However, about ten percent of all paragangliomas tend to become malignant. As it is not possible to tell from the symptoms whether a development to malignancy has already set in, the tumors should always be surgically removed as a precaution.
However, complications can also occur in the presence of a benign paraganglioma. That again depends on where the tumor is located. In some cases, this can lead to hearing impairment and even complete deafness. Facial paralysis and dysphagia are also occasionally observed. Further complications are related to the fact that some paragangliomas are neuroendocrine tumors.
When the paraganglioma is in the adrenal medulla, it produces larger amounts of catecholamines such as norepinephrine, adrenaline, or metanephrines. This special form of paraganglioma is also known as pheochromocytoma. Due to the formation of hormones, a pheochromocytoma represents a great danger for the patient, regardless of whether the tumor is benign or malignant. This leads to either permanent high blood pressure or paroxysmal high blood pressure attacks.
The phases of high blood pressure are associated with tachycardia, dizziness, headaches, increased blood sugar or vomiting. As a result, strokes and heart failure can develop. In rare cases, paragangliomas located outside the adrenal medulla also produce catecholamines and lead to similar symptoms.
When should you go to the doctor?
The paraganglioma risk group primarily includes adult women around the age of fifty. If you suffer from problems in the ear area, increased vigilance is necessary. Consult a doctor if you experience reduced hearing, ringing in your ears or swelling in your ears. Since a malignant course of the disease can develop in some cases with the paraganglioma, a doctor should be consulted at the first irregularities. Disturbances in the act of swallowing, changes in vocalization and signs of paralysis in the face indicate a disease that requires treatment.
If you experience unsteady gait, dizziness or nausea, you need to see a doctor. Deafness or one-sided hearing are signs of a serious health problem. A doctor must be consulted so that the cause can be investigated and a diagnosis made possible. High blood pressure, tachycardia and sleep disorders are further indications of a discrepancy.
If the symptoms persist for several days or if they gradually increase in intensity, a visit to the doctor is strongly recommended. In most cases, slow growth of the paraganglioma is documented, leading to a continuous deterioration in health. There is also a need for action in the case of fear, an aggressive appearance and mood swings. If major changes in behavior or personality are noticed, a doctor is needed. Reduced performance and a general feeling of being unwell should be discussed with a doctor.
Treatment & Therapy
The treatment of paraganglioma depends not only on the individual characteristics of the affected patient, but also on the type of tumor. In many cases, surgical removal is an option. In the case of paraganglioma jugulare, it is very promising with a success rate of 96 percent; however, permanent damage is possible.
Paragangliomas that have infiltrated the bone are often difficult to completely surgically remove. Many places do not allow sampling before the actual removal because the affected structures are too fine. If the affected tissue is surgically removed, a histological examination can provide more precise information about the nature of the tumor. The tumors often form oval to round structures that can have a red-brown capsule.
They have a network of capillaries on the surface that supply nutrients to the paraganglioma. 10 to 40 percent of tumors are cancerous or malignant; the exact number varies depending on which paraganglion is affected. Without successful treatment, they can spread or metastasize and thus affect other organs.
Paraganglioma aorticum has a high mortality rate of around 50 percent. Radiation or chemotherapy is less common as part of the treatment of paragangliomas. However, this application is very controversial among medical professionals.
Outlook & Forecast
Paragangliomas offer a relatively poor prognosis. Tumor diseases must be detected early to avoid serious health complications. In the case of a tumor disease with paragangliomas, life expectancy is not necessarily limited. The sooner treatment is given, the better the chances of recovery.
If the paragangliomas are not detected and removed, there is a risk that they will spread to surrounding areas of tissue. Paragangliomas can be benign and malignant. The benign variant offers a better prognosis. Malignant paragangliomas can lead to serious health problems and, in the worst case, can be fatal.
The prognosis for paragangliomas is made by the responsible specialist who is in charge of the therapy. He takes various factors into account for the prognosis. These include the previous course of the disease, the severity of the disease and the constitution of the patient. If the patient is physically fit, the prognosis is usually better. The prognosis is adjusted several times during the course of the disease, because the risks can be regularly reassessed on the basis of current examination results. The patient learns about his prognosis during a consultation.
In the case of tumor diseases such as paragangliomas, these consultations take place at regular intervals. The quality of life is limited due to the aggressive therapies and due to the tumor symptoms themselves. Most patients are unable to work during the illness.
Prevention
Prevention is only possible in a very general way, for example through a healthy lifestyle. For patients who already have a paraganglioma, check-ups that may be recommended by their doctor are important in order to detect a tumor return or spread at an early stage. For people in whose relatives the familial phenocytoma-paraganglion syndrome is known, a predictive diagnosis may be considered.
Aftercare
In most cases, those affected with a paraganglioma have only a few or only limited follow-up measures available. In the case of this disease, a quick diagnosis and subsequent treatment are very important, so that there are no further complications or other symptoms for the person affected. The earlier a doctor is consulted, the better the further course of the disease, so that the person concerned should ideally consult a doctor at the first sign of the paraganglioma.
The disease can also be genetically determined, so that the children of those affected should also undergo regular examinations in order to detect such a tumor at an early stage. The symptoms can also be alleviated relatively well with chemotherapy. In most cases, the person affected is dependent on the support of their own family.
Psychological support is also very important and can also prevent depression or other mental upsets. Regular check-ups by a doctor should also be carried out after successful removal of the tumors in order to prevent the recurrence of this complaint. In many cases, the paraganglioma limits the life expectancy of those affected.
You can do that yourself
Depending on the symptoms that the paraganglioma causes, different measures can be taken. A healthy diet and avoidance of addictive substances help against the typically occurring high blood pressure. In consultation with the doctor, those affected can also do sports.
Natural remedies such as chamomile, mistletoe or a decoction of garlic cloves and lemon juice support the therapy. In the case of hormonal problems, preparations with Maca root and other natural remedies that regulate hormone levels can be taken. In principle, those affected should eat a healthy diet rich in vitamins and avoid foods that increase cortisol levels. If hearing problems occur, the ears should not be subjected to further overload. With the help of some natural remedies such as ginseng, tinnitus and ringing in the ears can at least be reduced. If facial paralysis or swallowing problems occur, bed rest and rest are recommended.
A paraganglioma puts a heavy strain on physical and mental health, which is why physiotherapy, talk therapy and similar measures are indicated. After an operation, the patient has to take it easy. It is important to comply with the doctor’s instructions and to make use of the follow-up examinations. In the event of further complications, the responsible doctor must be informed.