Paraganglioma surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

A paraganglioma is a rare neoplasm that can be found in the abdomen, thorax, and in the head and neck region. They are usually considered benign and complete surgical removal results in cure. However, in about 3% of cases they are malignant and have the ability to metastasize. Paragangliomas are still sometimes called glomus tumors (not to be confused with glomus tumors of the skin) and chemodectomas, but paraganglioma is the currently accepted and preferred term.

Paragangliomas arise from the glomus cells, which are special chemoreceptors located along blood vessels that have a role in regulating blood pressure and blood flow. The main concentration of glomus cells are found are in the carotid body, located in the upper neck at the branching of the common carotid artery, and the aortic bodies, located near the aortic arch. The glomus cells are a part of the paraganglion system composed of the extra-adrenal paraganglia of the autonomic nervous system, derived from the embryonic neural crest. Thus, paragangliomas are a type of neuroendocrine tumor, and are closely related to pheochromocytomas. Although all paragangliomas contain neurosecretory granules, only about 1-3% have clinical evidence of oversecretion.

Surgery

Paraganglioma are rarely cancerous and do not tend to spread to other parts of the body. However, treatment may be needed to relieve symptoms. The main treatment is surgery. Surgery is complex and is usually done by both a neurosurgeon and a head and neck surgeon (neurotologist).

In some cases, a procedure called embolization is performed before surgery to prevent the tumor from bleeding too much during surgery.

After surgery, radiation therapy may be used to treat any part of the tumor that could not be removed completely.

Some glomus tumors can be treated with stereotactic radiosurgery.

Surgery

The main treatment modalities are surgery, embolization and radiotherapy.

References

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