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?

People 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 up in the Cancer Genetics Clinic in order to have blood and urine tests as well as ultrasound or MRI studies that can detect these tumors at an early stage.


Upcoming Events

Camp UOTS

Camp U.O.T.S. is an annual weeklong, overnight camp for children with cancer and blood disorders who are treated at Nicklaus Children’s Hospital.

Learn more and register

Reviewed by: Ziad A Khatib, MD

This page was last updated on: 5/2/2018 10:56:30 AM



video
Remote controlled transport cars are used to provide a positive experience for young children who are being taken into surgery at Nicklaus Children's Hospital.

From the Newsdesk

Medical Mission to Algeria Helps Children in Need of Spinal Surgeries
03/06/2018 — A group of children in Algeria who underwent complex surgeries as part of a 2016 U.S.-sponsored medical mission have many reasons to celebrate, and can do so with better movement of their limbs.
Outpatient Surgery Success Story
02/28/2018 — Caludell noticed that 6 of her 11 children had belly buttons that stuck out, and they seemed to become more pronounced around the time they entered kindergarten. When her daughters started to become self-concious about how their belly buttons looked, Claudell was able to schedule all 6 umbilical repair surgeries on the same day at the Miami Children's  Ambulatory Surgery Center.