What is Paraganglioma & How is it Treated?

Paraganglioma is a pathological condition in which there is development of a type of tumour in the peripheral nervous system of the body. The peripheral nervous system is that part of nervous system which is outside the brain and the spinal cord. The peripheral nervous system is further subdivided into sympathetic and parasympathetic nervous systems. The function of the peripheral nervous system is controlling various bodily functions to include the heart rate, blood pressure, urination, and intestinal motions.

Thus, a tumor or Paraganglioma developing in this system may cause alterations in function of any of these bodily functions like the blood pressure, urination, heart rate and the like. Paraganglioma can develop anywhere along the peripheral nervous system but they are mainly found in the abdomen and are related to some form of tumors of the adrenal glands called phaeochromocytoma.

Paraganglioma can cause a significant rise in blood pressure and heart rate among other symptoms. The best way to treat Paraganglioma is removal of the tumor by way of surgery. In some cases where the tumor is located at a place which is quite sensitive and is surrounded by major neurovascular structures then the tumor is not able to be removed surgically and radioactive elements are used to slow down the progression of the tumor and then followed with serial scans to check on the status of the tumor.

The primary cause for Paraganglioma is a genetic defect and mutations of certain genes. In some cases, Paraganglioma are attributed to certain genetic syndromes affecting other organs of the body. In case if development of Paraganglioma is not associated with any genetic syndrome then it is termed as nonsyndromic paragangliomas.

The genes responsible for development of Paraganglioma are mutations in genes VHL, RET, SDHA, SDHB, and SDHD These genes perform important functions in the body like controlling cell division and formation of blood vessels. These defective genes can be passed from just one parent to the child or in other forms it is an autosomal dominant form of disorder causing uncontrolled development of cells leading to development of Paraganglioma. Paraganglioma can also develop due to certain inherited medical conditions. These conditions are:

• Hereditary Paraganglioma Syndrome
• Multiple Endocrine Neoplasia 2
• Neurofibromatosis type 1
• Von Hippel-Lindau syndrome.

Some of the symptoms of Paraganglioma are:

• Persistent elevated high blood pressure
• Increased heart rate,
• Persistent headaches
• Facial flushing and sweating
• Severe anxiety and panic attacks.

Some of the less common symptoms of Paraganglioma are:

• Pale skin
• Blurred vision
• Unintentional weight loss
• Constipation
• Abdominal pain
• Hyperglycemia
• Other psychiatric abnormalities.

If Paraganglioma is suspected, then the doctor may prescribe a battery of tests in order to confirm the disease condition. The doctor may begin with collecting urine over a period of 24 hours and sending it for analysis to look for presence of any excessive adrenaline or noradrenaline secretions. Simultaneously blood tests may also be done for the same reason. Once this study is done a CT and MRI scan will be done, especially of the abdominal area to identify presence of any tumors. In some cases a PET scan may also be done to look for presence of Paraganglioma and whether the tumor has spread to any degree. Genetic testing may also be done to check for any gene defects in case if there is a family history of Paraganglioma.

The results of these tests virtually confirm the presence of Paraganglioma.

The front line treatment for Paraganglioma is removal of the tumors by way of surgery. This surgical procedure is done on an inpatient basis. Before the surgery is conducted, the patient is stabilized in terms of controlling and bringing down the blood pressure to normal levels and also regulating the heart rate. In order to do this, the patient may be given some medications in the form of certain antihypertensives to bring down the blood pressure. Once the blood pressure is stabilized then the patient is taken to the operating room for removal of the tumors.

In case if there is a tumor present at a sensitive location where it cannot be removed then the surgeons will use octreotide to curtail the progression of the tumor. Once the surgery is completed, the blood pressure of the patient will be monitored to make sure that it does not rise or fall excessively which may lead to serious complications. Once the blood pressure has been stabilized and is controlled, then the patient is discharged.

Post surgery, the patient will have to followup with the treating doctor with regular CT scans and blood pressure checks to make sure that there is no recurrence of the tumors and to check the status of the tumors which were not able to be removed during surgery.

The prognosis of Paraganglioma is quite good with timely intervention and removal of the tumor and also how much the tumor has spread. Paragangliomas are usually slow growing and it is common for people to live a healthy life for many years after their diagnosis of Paraganglioma.