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ACE Angiotensin-converting enzyme
ACTH Adrenocorticotropic hormone
AM Adrenomedullin
APMO Adnexal cystadenomas of probable mesonephric origin
ARDS Acute respiratory distress syndrome
ATP Adenosine triphosphate
cAMP Cyclic adenosine monophosphate
CCB Calcium channel blocker
CgA Chromogramin A
CGRP Calcitonin gene–related peptide
COMT Catecholamine-O-methyltransferase
DBH Dopamine-β-hydroxylase
DHPG Dihydroxyphenylglycol
ECD Electrochemical detection
FDG Fluorodeoxyglucose
HIF Hypoxia-inducible factor
HN-PGL Head-neck paraganglioma
HPLC High-pressure liquid chromatography
IP3 Inositol triphosphate
MAO Monoamine oxidase
MEN Multiple endocrine neoplasia
MHBA 3-methoxy-4-hydroxybenzylamine
MIBG Metaiodobenzylguanidine
NF-1 Neurofibromatosis type 1
NSE Neuron-specific enolase
PGL Paraganglioma
PHEO Pheochromocytoma
PNMT Phenylethanolamine-N-methyltransferase
PTHrP Parathyroid hormone–related peptide
RECIST Response evaluation criteria in solid tumors
SDHA Succinate dehydrogenase subunit A
SDHB Succinate dehydrogenase subunit B
SDHC Succinate dehydrogenase subunit C
SDHD Succinate dehydrogenase subunit D
SDHx Succinate dehydrogenase subunits
SPECT Single photon emission computed tomography
SRI Somatostatin receptor imaging
VEGF Vascular endothelial growth factor
VHL von Hippel-Lindau
VIP Vasoactive intestinal polypeptide
VMA Vanillylmandelic acid (3-methoxy-4-hydroxymandelic acid)

The adrenal medulla and paraganglia are part of the autonomic/sympathetic nervous system. The endocrine and nervous systems are alike in that they exert their actions by releasing hormones/neurotransmitters that bind to cell surface receptors in the target tissue, thereby inducing an effect.

Autonomic nerves are not under conscious control. They innervate the heart, adrenal medulla, vascular smooth muscle, and smooth muscle in visceral organs, thereby controlling cardiac rate and output, adrenal medullary secretion of catecholamines, blood pressure, the genitourinary tract, and intestinal motility. Autonomic nerves originate within the central nervous system and have two major divisions according to their anatomic locations:

  1. Parasympathetic preganglionic nerves exit the central nervous system via the cranial nerves and sacral spinal nerves. They terminate in nonchromaffin paraganglia that are most numerous in the neck and associated with the glossopharyngeal and vagus nerves. These ganglia serve as chemoreceptors that are involved in the control of respiration. An important paraganglion at the carotid bifurcation is known as the carotid body. They are also found along the jugular vein and in the jugulotympanic region. Head-neck paragangliomas are tumors that arise from these parasympathetic paraganglia.

  2. Sympathetic preganglionic nerves exit the central nervous system via the thoracic and lumbar spinal nerves. The sympathetic nervous system coordinates the body’s automatic fight-flight response by stimulating the adrenal medulla to secrete catecholamines and by directly stimulating cardiac output and blood flow to muscles while diverting blood flow away from visceral organs.

Sympathetic preganglionic nerves terminate mainly in paravertebral and prevertebral nerve ganglia where they secrete acetylcholine as their neurotransmitter; they are, therefore, known as cholinergic nerves. These nerve ganglia are collectively known as paraganglia and contain neuroendocrine cells that are similar to adrenal medullary cells on light microscopy by chromaffin and immunohistochemical staining. Paraganglia are also found in the mediastinum, particularly adjacent to the cardiac atria, and in the abdomen along the sympathetic nerve chains in paravertebral and prevertebral ...

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