What is familial paraganglioma?
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.
How is paraganglioma inherited?
Inheritance. Hereditary paraganglioma-pheochromocytoma is inherited in an autosomal dominant pattern , which means one copy of the altered gene in each cell is sufficient to increase the risk of developing tumors. An additional mutation that deletes the normal copy of the gene is needed to cause the condition.
How do you get rid of paraganglioma?
Treatment of pheochromocytoma or paraganglioma that has spread to nearby organs or lymph nodes is surgery to completely remove the tumor. Nearby organs that the cancer has spread to, such as the kidney, liver, part of a major blood vessel, and lymph nodes, may also be removed.
Is paraganglioma curable?
There are currently no cures for cancerous paraganglioma. However, existing treatment options may reduce the tumors and prolong survival.
What percentage of paragangliomas are hereditary?
The incidence of paragangliomas is lower, but these tumors are more often associated with hereditary predisposition. Altogether, about 35%-40% of all PGL/PCC are associated with a hereditary predisposition.
Are paragangliomas cancerous?
A paraganglioma is a rare but treatable neuroendocrine tumor that usually forms along major blood vessels and nerve pathways in your neck and head. In most cases, the tumor is benign, but it can be malignant (cancer).
How common is paraganglioma?
Do paragangliomas come back?
This is called follow-up care. People treated for a pheochromocytoma or paraganglioma need lifelong follow-up care. This is important because pheochromocytomas and paragangliomas can recur years after initial treatment. This care may include regular physical examinations, medical tests, or both.
Is paraganglioma a genetic disorder?
A rare, genetic disorder marked by tumors called paragangliomas, which are found in paraganglia. Paraganglia are groups of nerve-like cells found near the adrenal glands and near blood vessels or nerves in the head, neck, chest, abdomen, and pelvis.
What type of brain tumors are hereditary?
About 5% of brain tumors may be linked to hereditary genetic factors or conditions, including Li-Fraumeni syndrome, neurofibromatosis, nevoid basal cell carcinoma syndrome, tuberous sclerosis, Turcot syndrome, and von Hippel-Lindau disease.
Is paraganglioma hereditary?
Description. Hereditary paraganglioma-pheochromocytoma is an inherited condition characterized by the growth of tumors in structures called paraganglia. Paraganglia are groups of cells that are found near nerve cell bunches called ganglia. A tumor involving the paraganglia is known as a paraganglioma.