The tumor on an autonomic nerve node in the autonomic nervous system (paraganglion) is called a paraganglioma or chemodectroma in medicine. Depending on which paraganglion is affected, symptoms and treatment vary. The tumors run in families.
What is a paraganglioma?
Paraganglioma, or chemodectroma, is a tumor and develops from an autonomic nerve node of the autonomic nervous system, also known to medicine as a paraganglion. The neoplasm can be benign or malignant; however, the majority is a benign tumor. Paragangliomas can be divided into several types: Paraganglioma tympanicum forms in the middle ear and primarily affects women around the age of 50, while paraganglioma jugulare is also known as glomus jugulare tumor and occurs in the fossa jugularis ossis temporalis at the base of the skull. Also at the base of the skull, but at the jugular foramen, runs the vagus nerve, where paraganglioma vagale may manifest. Visceral paragangliomas cause cell proliferation on internal organs; they particularly commonly affect the urinary bladder. Paraganglioma aorticum, on the other hand, is an aggressive tumor on the nerve node of the aorta, which leads to death in half of the cases. Paragangliomas that form specifically on the abdominal aorta and involve abdominal sinus ganglia are sometimes known as retroperitoneal paragangliomas. Most commonly, the neoplasm occurs as paraganglioma caroticum on the carotid artery.
Causes
The cause of paraganglioma is due to uncontrolled cell growth. Human cells have a variety of mechanisms that combat damaged or destructive cells. Control processes at the micro level, for example, detect damage to the cell’s DNA, whereupon it can destroy itself; biology also calls this process apoptosis (“cell suicide”). The immune system can also intervene. In tumorigenesis, this mechanism fails and the cell divides, displacing healthy tissue and causing corresponding symptoms. Paragangliomas need not only affect the affected paraganglion, but can also affect surrounding tissue. Particularly for malignant tumors such as those that develop in the course of cancer, a connection exists between various triggers and tumor formation. These include radiation, certain chemical substances, viruses and lifestyle factors such as diet. Individually, however, the specific cause often cannot be clearly determined. In familial phenocytoma-paraganglion syndrome, there is a genetic cause for which three genes are known to date.
Symptoms, complaints, and signs
Complaints that typically occur as a result of paraganglioma vary 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 others, this symptom need not be present. In paraganglioma jugulare, for example, it typically does not manifest. In many cases, paraganglioma tympanicum leads to hearing complaints such as tinnitus, ringing in the ears, and impaired hearing, even deafness. The symptoms manifest themselves mainly on the side on which the tumor grows. In addition, dizziness and impairment of the fourth cranial nerve may occur. Also among the characteristic symptoms of paraganglioma jugulare is tinnitus, as well as facial paralysis and dysphagia. If the cranial nerves in this area are affected, their (partial) failure may cause additional symptoms.
Diagnosis and course of the disease
For diagnosis, physicians usually consult imaging techniques to precisely locate the paraganglioma, identify its extent, and, if necessary, 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) may also be considered, if necessary as a whole-body scan.
Complications
In most cases, paragangliomas are benign tumors. However, approximately ten percent of all paragangliomas show a tendency to malignant degeneration.Since it is not possible to tell from the symptoms whether the tumor has already developed into a malignancy, it should always be surgically removed as a precaution. However, complications can occur even in the presence of a benign paraganglioma. Now, this again depends on where the tumor is located. In some cases, for example, hearing impairment or even complete deafness may occur. Facial paralysis and dysphagia are also sometimes observed. Other complications are related to the characteristic of some paragangliomas that they are neuroendocrine tumors. When located in the adrenal medulla, paraganglioma produces larger amounts of catecholamines such as norepinephrine, epinephrine, or metanephrines. This special form of paraganglioma is also called a pheochromocytoma. Due to the hormone formation, a pheochromocytoma poses a great danger to the patient even regardless of whether the tumor is benign or malignant. In this case, either permanent high blood pressure or seizure-like high blood pressure attacks occur. The phases of high blood pressure are associated with palpitations, dizziness, headaches, elevation of blood sugar or vomiting. Strokes and heart failure may develop as a result. In rare cases, paragangliomas, which are located outside the adrenal medulla, also produce catecholamines and cause similar symptoms.
When should you see a doctor?
The risk group for paraganglioma primarily includes adult women around the age of fifty. If they suffer discomfort in the area of the ear, increased vigilance is necessary. In case of decreased hearing, ringing in the ears or swelling in the ear, a doctor should be consulted. Since paraganglioma can develop a malignant course in some cases, a doctor should be consulted at the first irregularities. Disturbances of the swallowing act, changes in phonation as well as paralysis symptoms in the face indicate a disease requiring treatment. If gait unsteadiness, dizziness or nausea occurs, a physician is needed. Deafness or one-sided hearing are signs of a serious health disorder. A physician must be consulted so that an investigation of the cause can be initiated and a diagnosis can be made. High blood pressure, palpitations as well as sleep disturbances are further indications of a disorder. If the symptoms persist for several days or gradually increase in intensity, a visit to the doctor is urgently recommended. In most cases, a slow growth of the paraganglioma is documented, which leads to a continuous deterioration of the health condition. In case of anxiety, an aggressive demeanor, as well as mood swings, there is also a need for action. If severe changes in behavior or personality are noted, a physician is needed. Decreased performance as well as a general feeling of malaise should be discussed with a physician.
Treatment and therapy
Treatment of paraganglioma depends not only on individual characteristics of the affected patient, but also on the type of tumor. In many cases, surgical removal is an option. In 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 remove completely by surgery. Many sites do not allow sampling prior to actual removal because the affected structures are too fine. If the affected tissue is surgically removed, histologic examination can provide more detailed information about the nature of the tumor. The tumors often form oval to round structures that may have a reddish-brown capsule. They have a network of capillaries on the surface that supply nutrients to the paraganglioma. Ten to 40 percent of tumors are malignant 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 about 50 percent. Rarely, radiation or chemotherapy is also used in the treatment of paragangliomas. However, this use is highly controversial among physicians.
Outlook and prognosis
Paragangliomas offer a relatively poor prognosis. Tumor disease must be detected early to avoid serious health complications.In the case of tumor disease with paragangliomas, life expectancy is not necessarily limited. The earlier the therapy, the better the prospects for recovery. If 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 variety offers a better prognosis. Malignant paragangliomas can lead to severe health limitations and, in the worst cases, can be fatal. The prognosis for paragangliomas is determined by the specialist in charge of the therapy. He takes various factors into account for the prognosis. These include the previous course of the disease, its severity and the patient’s constitution. 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. For tumor diseases such as paragangliomas, these counseling sessions 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 disease.
Prevention
Prevention is only possible in very general terms, for example, through a healthy lifestyle. For patients who already have paraganglioma, check-ups recommended by their physician, if any, are important to detect tumor recurrence or spread early. For individuals with a known family history of familial phenocytoma-paraganglion syndrome, predictive diagnostics may be considered.
Follow-up
In most cases of paraganglioma, few or even limited measures of follow-up care are available to the affected individual. With this disease, first and foremost a quick diagnosis and also a subsequent treatment is very important, so that it does not come to further complications or to other complaints in the affected person. The earlier a doctor is consulted, the better the further course of the disease usually is, so that the affected person should ideally consult a doctor already at the first signs of paraganglioma. The disease can also be genetically determined, so that the children of the affected person should also undergo regular examinations in order to detect such a tumor at an early stage. The symptoms can also be relatively well alleviated by chemotherapy. In most cases, the affected person is dependent on the support of his or her own family. Psychological support is also very important and can also prevent depression or other psychological upsets. Likewise, even after successful removal of the tumors, regular check-ups by a doctor should be performed to prevent the recurrence of this complaint. In many cases, paraganglioma limits the life expectancy of the affected person.
Here’s what you can do yourself
Depending on the discomfort caused by paraganglioma, different measures can be taken. Against the typically occurring high blood pressure helps a healthy diet and the avoidance of addictive substances. In consultation with the doctor, those affected can also exercise. Natural remedies such as chamomile, mistletoe or a decoction of garlic cloves and lemon juice support the therapy. In the case of hormonal complaints, preparations containing maca root and other natural remedies can be taken to regulate hormone levels. Basically, sufferers should eat a healthy diet rich in vitamins and avoid foods that increase cortisol levels. When 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 difficulty swallowing occurs, bed rest and sparing are recommended. A paraganglioma puts a great strain on physical and mental health, which is why physiotherapy, talking therapies and similar measures are indicated. After surgery, the patient must take care of himself. It is important to follow the doctor’s instructions and to make use of the aftercare examinations. In case of further complications, the responsible physician must be informed.
