Pheochromocytomas and paragangliomas are rare neuroendocrine tumors (NETs) with an incidence estimated at 2-8 cases per 1 million annually. They are functional catecholamine-producing tumors that arise from sympathetic adrenomedullary chromaffin tissue. In extremely rare cases they are associated with ectopic production of ACTH. This can lead to mixed presentations with diagnostic and treatment challenges.
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Sutaria et al. (2024) studied this question.
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