Chest With Mediastinal Paraganglioma – Male, 50 Years

What Is a Paraganglioma?

Paragangliomas are rare neuroendocrine tumors. They can form in the chest and abdomen (called sympathetic) or the head and neck (called parasympathetic). The tumors are made of cells from the adrenal glands called chromaffin cells, which produce important hormones like dopamine, epinephrine and norepinephrine. Referred to as catecholamines, paragangliomas may release higher amounts of these hormones into the bloodstream. 

Most paranglioma tumors don’t cause symptoms. When they do, sympathetic paragangliomas may cause headaches, heart palpitations and high blood pressure. Parasympathetic paragangliomas may cause changes in vision, swallowing or ringing in the ears.

How Common Is a Paraganglioma?

The condition is extremely rare and occurs in roughly two out of every one million people. It is more common in females than males by a ratio of 3:1. It’s most likely to be diagnosed between the ages of 20 and 50, with 10% of cases occurring in children. Up to 40% of cases are caused by genetic conditions including: succinate dehydrogenase (SDH) mutations, RET mutations, NF1 mutations, von Hippel-Lindau syndrome and Carney-Stratakis syndrome/Carney triad. The cause is unknown in the remainder of cases.

How Is It Treated?

Most paragangliomas are benign when they’re diagnosed and successfully treated. Treatment typically includes surgery, followed by radiation therapy. In 25% of cases, the tumor is malignant and may spread to the lymph nodes, liver, lung or bones. Chemotherapy or other systemic treatment is often required.