Paraganglioma

The medicine describes the tumor on an autonomic nerve node in the vegetative nervous system (paraganglion) as Paraganglioma or Chemodectrom. Depending on which paraganglion is affected, symptoms and treatment will vary. The tumors occur in families.

What is a paraganglioma?

The Paraganglioma or Chemodectrom is a tumor and develops from an autonomic nerve node of the vegetative nervous system, which medicine also knows as a paraganglion. The neoplasm can be both benign and malignant; however, the majority is a benign tumor.

Paragangliomas can be divided into different types: That Paraganglioma tympanicum forms in the middle ear and mainly affects women around the age of 50, while the Paraganglioma jugulare Also known as the jugular glomus tumor, it occurs in the jugular fossa at the base of the skull. The vagus nerve, where the paraganglioma vagale can manifest itself, also runs along the base of the skull, but along the jugular foramen.

Visceral paragangliomas cause cell growths on internal organs; they particularly often 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, which form specifically on the abdominal aorta and affect the abdominal cavity ganglia, are also known in part as retroperitoneal paragangliomas. Most often, the new formation occurs as a paraganglioma carotidum on the carotid artery.

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. Control processes at the micro level recognize, for example, damage in the cell's DNA, which can then destroy itself; biology also calls this process apoptosis ("cell suicide").

The immune system can also intervene. When tumors develop, this mechanism fails and the cell divides, displacing healthy tissue and causing symptoms. Paragangliomas not only have to affect the affected paraganglion, but can also affect surrounding tissue.

Especially for malignant tumors such as those that arise in the context of cancer there is a connection between various triggers and tumor formation. These include radiation, certain chemicals, viruses, and lifestyle factors such as diet. However, the specific cause can often not be clearly identified individually. Familial phenocytoma paraganglion syndrome has a genetic cause for which three genes are known.

Symptoms, ailments & signs

Symptoms 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 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 impaired hearing or even deafness. Symptoms mainly manifest on the side on which the tumor grows. In addition, dizziness and impairment of the fourth cranial nerve can occur.

The characteristic symptoms of paraganglioma jugulare also include tinnitus, facial paralysis and swallowing disorders. If the cranial nerves in this area are affected, their (partial) failure can cause additional symptoms.

Diagnosis & course of disease

Doctors usually use imaging techniques for diagnosis in order to precisely localize the paraganglioma, recognize its extent and, if necessary, make metastases visible. 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 used, possibly as a whole-body scan.

Complications

In most cases, paragangliomas are benign tumors. However, about ten percent of all paragangliomas show a tendency towards malignant degeneration. As the symptoms do not reveal whether a malignancy has already developed, the tumors should always be surgically removed as a precaution.

However, complications can occur even if a benign paraganglioma is present. That again depends on where the tumor is located. In some cases this can lead to hearing impairment or even total deafness. Facial paralysis and swallowing disorders are also sometimes observed. Further complications are related to the property of some paragangliomas that they are neuroendocrine tumors.

When the paraganglioma is in the adrenal medulla, it makes larger amounts of catecholamines such as noradrenaline, adrenaline, or metanephrine. This special form of paraganglioma is also known as a pheochromocytoma. Due to the formation of hormones, a pheochromocytoma represents a great risk for the patient regardless of whether the tumor is benign or malignant. Either permanent high blood pressure or attacks of high blood pressure occur here.

The phases of high blood pressure are associated with palpitations, dizziness, headache, high blood sugar or vomiting. As a result, strokes and heart failure can develop. In rare cases, paragangliomas that are located outside the adrenal medulla also form catecholamines and lead to similar symptoms.

When should you go to the doctor?

The risk group of paraganglioma mainly includes adult women around fifty years of age. If you experience discomfort in the area of ​​the ear, increased vigilance is necessary. If you have impaired hearing, ringing in the ears or swelling of the ear, you should consult a doctor. Since a malignant course of the disease can develop in some cases with paraganglioma, a doctor should be consulted as soon as the first irregularities occur. Disturbances in the act of swallowing, changes in sound and paralysis in the face indicate a disease that requires treatment.

If you experience unsteadiness, dizziness or nausea, a doctor is needed. Deafness or one-sided hearing are signs of a serious health disorder. A doctor must be consulted so that research into the cause can be initiated and a diagnosis can be made. High blood pressure, palpitations 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 a doctor is strongly recommended. In most cases, a slow growth of the paraganglioma is documented, which leads to a continuous deterioration in the state of health. There is also a need for action in the case of fear, aggressive behavior and mood swings. If severe changes in behavior or personality are noticed, a doctor is required. Reduced performance and general malaise should be discussed with a doctor.

Treatment & Therapy

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 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 a sample to be taken before the actual removal, as the affected structures are too fine. If the affected tissue is surgically removed, a histological examination can provide more detailed 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 the paraganglioma with nutrients. 10 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.

The Paraganglioma aorticum has a high mortality rate of around 50 percent. Radiation or chemotherapy are less common in the treatment of paragangliomas. However, this application is very controversial among medical professionals.

Outlook & forecast

Paragangliomas offer a relatively poor prognosis. Cancer diseases need to be detected early to avoid serious health complications. If you have a tumor with paragangliomas, life expectancy is not necessarily restricted. The earlier therapy is given, the better the chances of recovery.

If the paragangliomas are not discovered and removed, there is a risk that they will spread to surrounding areas of the tissue. Paragangliomas can be benign and malignant. The benign variant offers a better prognosis. Malignant paragangliomas can lead to serious health impairments and in the worst case be fatal.

The prognosis for paragangliomas is made by the specialist responsible for 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 test 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 because of the aggressive therapies and 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, it may be important to have check-ups recommended by their doctor in order to detect a return or spread of the tumor at an early stage. For people who are related to the familial phenocytoma-paraganglion syndrome, predictive diagnostics may be considered.

Aftercare

In the case of a paraganglioma, in most cases only a few or only limited follow-up measures are 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 complaints for the person concerned. The earlier a doctor is consulted, the better the further course of the disease is usually, so that the person affected should ideally see a doctor at the first signs of paraganglioma.

The disease can also have genetic causes, so that the children of the person affected should also undergo regular examinations in order to detect such a tumor at an early stage. Chemotherapy can also relieve the symptoms relatively well. In most cases, the affected person is dependent on the support of their 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 should be carried out by a doctor in order to prevent this complaint from recurring. In many cases, the paraganglioma limits the life expectancy of the person affected.

You can do that yourself

Depending on the discomfort that the paraganglioma is causing, 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 brew made from garlic cloves and lemon juice support the therapy. In the case of hormonal complaints, preparations containing Maca root and other natural remedies that regulate the hormone level can be taken. Basically, sufferers should eat healthy and 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 comparable measures are indicated. After an operation, the sick person has to take it easy. It is important to adhere to the doctor's instructions and to take advantage of the follow-up examinations. In the event of further complications, the responsible doctor must be informed.