Catecholamine-producing tumors may arise in the adrenal gland (pheochromocytomas) or in extraadrenal chromaffin cells (secreting paragangliomas).
Catecholamines are hormones made by the adrenal glands. These glands are on top of the kidneys. Catecholamines are released into the blood when a person is under physical or emotional stress. The main catecholamines are dopamine, norepinephrine, and epinephrine (which used to be called adrenalin).
Their frequency is about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered adrenal mass.
An increase in the production of catecholamines causes symptoms (mainly headaches, palpitations and excess sweating) and signs (mainly hypertension, weight loss and diabetes) reflecting the effects of epinephrine and norepinephrine on alpha and beta-adrenergic receptors.
Catecholamine-producing tumors mimic paroxysmal conditions with hypertension and/or cardiac rhythm disorders, including panic attacks, in which sympathetic activation linked to anxiety reproduces the same signs and symptoms.
These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis type 1 and von Hippel-Lindau disease.
Familial cases are diagnosed earlier and are more frequently bilateral and recurring than sporadic cases.
The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrines.
The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine (MIBG) scintigraphy.
Treatment requires resection of the tumor, generally by laparoscopic surgery.
About 10% of tumors are malignant either at first operation or during follow-up, malignancy being diagnosed by the presence of lymph node, visceral or bone metastases.
Recurrences and malignancy are more frequent in cases with large or extraadrenal tumors.
Patients, especially those with familial or extraadrenal tumors, should be followed-up indefinitely.