Pheochromocytomas and paragangliomas: from DNA to the daily clinical practice

The neuroendocrine system is a diffuse system in which the nervous system and the hormones of the endocrine glands interact. The neuroendocrine organs of the sympathetic and parasympathetic autonomic nervous system are called paraganglia. These organs usually manifest as anatomically discrete bodies, which derive from the neural crest and produce catecholamines and various peptides. Various localizations of paraganglia in the human body are known, including the adrenal gland, organs of Zuckerkandl, and carotid and aortic bodies. Paraganglia are divided into two functional groups, i.e. the sympathoadrenal and the parasympathetic autonomic nervous system. Sympathetic paraganglia are predominantly located in the prevertebral and paravertebral sympathetic trunks, and along the fibers of the hypogastric plexus, innervating pelvic and retroperitoneal organs. Parasympathetic paraganglia are almost exclusively located in the region of cranial as well as thoracic branches of the of the glossopharyngeal nerves and vagal nerves. The principal glossopharyngeal paraganglia are the tympanic (located in the wall of the middle ear), and the carotid bodies (Figure 1). Neoplasms of the paraganglia are called pheochromocytomas (PCC), sympathetic and parasympathetic paragangliomas. The name PCC is derived from the Greek synonym “dark colored tumor”, because it was first described by Pick as a chromium salt-reactive tumor which lead to dark coloration. PCC are tumors which originate in the adrenal medulla. Sympathetic paragangliomas (sPGL), in the literature often described as extra-adrenal PCC, usually produce catecholamines and occur in the abdominal cavity and the aorticopulmonary bodies, but not in the adrenal medulla. Parasympathetic paragangliomas, also called head and neck paraganglioma, usually do not produce catecholamines and are situated in the wall of the middle ear, along the vagal nerve, and the carotid and jugular bodies. In the literature they are still often referred to as chemodectomas, glomus tumors, or carotid body tumors.

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