Treatment of Paraganglioma

About the disease

Paraganglioma is a rare tumor which develops in peripheral nervous system cells. These cells are located in the back of the brain and spinal cord in the nerves. This neuroendocrine neoplasm is considered to be rare. It can affect different body parts. IIt can develop in head and neck, in more rare cases thorax and abdomen.

Paraganglioma can be either benign or malignant depending on the nature of cells where it develops. Overall, it is impossible to tell whether the cells in paraganglioma are benign or malignant, because  it is impossible to take biopsy from peripheral nervous system. Only by observation can the doctors determine if paraganglioma is cancerous. Observing the symptoms and also determining if paraganglioma develops quickly is the only effective way to differentiate cancerous paraganglioma from a benign one.

If paraganglioma became recurrent, the probability of its malignancy becomes high. According to statistics, 50% of recurrent cases of paraganglioma metastasize to other parts of the body. If paraganglioma is malignant, recurrent and it metastasized, 5-year survival prognosis is 40% to 45%. Nonetheless, it is very rare for paraganglioma to become malignant and recurrent.

Paraganglioma can develop at any age and also in any ethnicity group. The exact cause of paraganglioma had not been determined yet. Overall, 75% of all paraganglioma cases develop for some unknown reason, while other 25% are caused by genetic predisposition. Gene mutation constitutes the genetic predisposition in this case.

Symptoms

Most commonly paraganglioma is asymptomatic.

It can manifest itself in painless mass

In case paraganglioma became malignant there can be the following symptoms:

• Headache
• Nausea
• Vomiting
• Weight loss
• Weakness
• Dizziness
• Fatigue

Diagnosis

• During general examination the doctor will examine the mass on the neck or back of the head, if it is present. In some cases the mass develops in the thorax or abdominal area.
• Imaging tests, such as MRI and CT scan, can identify the tumor.
• Hormonal tests can check the levels of hormones which can be changed.
• Nerve response tests can be used to check the nerve endings and determine if they function normally.

Treatment

• Surgical resection can be used to remove the tumor and prevent it from spreading in case it is malignant.
• Radiation therapy can be used as well to shrink the tumor and also prevent it from spreading.
• Embolization is used to block the tumor from growing as well.

Authors: Dr. Nadezhda Ivanisova, Dr. Farrukh Ahmed