What causes a paraganglioma?

Paragangliomas are rare tumors that arise from neuroendocrine cells of the paraganglia, which are part of the autonomic nervous system. They can be caused by genetic mutations, particularly in genes associated with hereditary syndromes like von Hippel-Lindau disease, multiple endocrine neoplasia type 2, and familial paraganglioma syndrome. Environmental factors and certain hormonal stimuli may also play a role, although the exact cause is often unclear. These tumors can be found in various locations, most commonly in the adrenal glands and along the sympathetic and parasympathetic nervous systems.