Hereditary Paraganglioma-Pheochromocytoma Syndrome

Also known as: PGL/PCC.

What is hereditary paraganglioma-pheochromocytoma syndrome?

Hereditary paraganglioma-pheochromocytoma syndrome is a genetic condition that’s passed down through families. Individuals with the condition are more likely to develop two rare forms of cancer. One is known as paragangliomas, which are tumors on the spine or lower skull. The other kinds are pheochromocytomas, which are tumors on the adrenal glands.

What causes hereditary paraganglioma-pheochromocytoma syndrome?

Hereditary paraganglioma-pheochromocytoma syndrome is a disorder that’s caused by genetic mutations. It’s inherited and passed down from parents to their children.

What are the symptoms of hereditary paraganglioma-pheochromocytoma syndrome?

The presence of the tumors, often in large numbers, is the primary sign of hereditary paraganglioma-pheochromocytoma syndrome. Many of these tumors become cancerous and lead to other symptoms such as headache, high blood pressure, sweating, heart problems, anxiety, coughing, trouble swallowing and hearing loss, among other symptoms.

What are hereditary paraganglioma-pheochromocytoma syndrome care options?

Children with hereditary paraganglioma-pheochromocytoma syndrome are often under frequent monitoring due to their high risk of developing cancer. When cancer occurs, it is often treated with surgery to remove the tumor, as well as chemotherapy and radiation therapy to rid the body of cancer.

It is important to have annual follow by a comprehensive Neuro-Oncology program in order to have blood and urine tests as well as ultrasound or MRI studies that can detect these tumors at an early stage.


Reviewed by: Ziad A Khatib, MD

This page was last updated on: December 16, 2020 02:25 PM

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