Extra adrenal paragangliomas refer to rare neuroendocrine tumors that develop outside the adrenal glands. Paragangliomas are often benign tumors arising from the paraganglia, a collection of specialized cells of the autonomic nervous system. The paraganglia are found in various parts of the body, including the abdomen, thorax, and head and neck region, and most commonly associated with the adrenal glands.
These tumors can arise in multiple locations, such as the chest, abdomen, pelvis, or along the spine, depending on the specific paraganglia involved. Extra adrenal paragangliomas can be found in the organs such as the bladder, rectum, uterus, or even in tissues near major blood vessels.
The exact cause of extra adrenal paragangliomas is not well understood. However, the majority of these tumors are associated with genetic mutations, particularly in certain genes such as SDHD, SDHB, SDHC, or RET.
Symptoms of extra adrenal paragangliomas vary depending on the location and size of the tumor. Some individuals may remain asymptomatic, while others may experience symptoms such as high blood pressure, rapid heartbeat, headache, palpitations, sweating, or weight loss.
Treatment typically involves surgical removal of the tumor, along with close monitoring of blood pressure and hormone levels. In some cases, radioactive iodine or external beam radiation therapy may be used to destroy any remaining tumor cells. Genetic testing and counseling are often recommended for patients with extra adrenal paragangliomas due to the potential hereditary component.
