Hereditary Paraganglioma-Pheochromocytoma (PGL/PCC) Syndrome
What is hereditary paraganglioma-pheochromocytoma syndrome?
Hereditary paraganglioma-pheochromocytoma syndrome (PGL/PCC) is a group of familial cancer syndromes characterized by the presence of:
- Paragangliomas (PGL) — tumors that arise from neuroendocrine tissues symmetrically distributed along the spine from the base of the skull to the pelvis
- Pheochromocytomas (PCC) — a type of paraganglioma that is confined to the adrenal gland, a small hormone producing organ located on top of each kidney
How is paraganglioma-pheochromocytoma syndrome diagnosed?
Hereditary paraganglioma-pheochromocytoma (PGL-PCC) syndrome is diagnosed by the presence of certain clinical signs, a family history that shows the presence of PGL-PCC, and genetic testing that confirms the gene mutation.
Continue reading to learn more about each of these diagnostic activities and reproductive options.
What cancer risks do people with paraganglioma-pheochromocytoma syndrome face and what cancer screenings are recommended for them?
The primary risk in hereditary PGL/PCC is the development of paragangliomas and pheochromocytomas. While the majority of these tumors are benign, in certain cases they may be malignant and thus more likely to spread to other parts of the body.
What resources are available for people with paraganglioma-pheochromocytoma syndrome?
Organizations exist to support children and adults with paraganglioma-pheochromoctyoma syndrome, and the National Library of Medicine provides reliable information in Gene Reviews.
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