Revista Finlay (Sep 2021)
Pheochromocytomas and Paragangliomas Diagnosis and Treatment
Abstract
Pheochromocytomas and paragangliomas are catecholamine-producing neuroendocrine tumors from the sympathetic or parasympathetic nervous system. They originate in the chromaffin cells of the adrenal medulla and the extra-adrenal sympathetic ganglia. Its annual incidence is 2-8 cases per one million people. The 70 % are sporadic and the 30 % are associated with familial genetic syndromes with mutations in the germ line of genes such as: NF1, VHL, RET and SDHx. Patients generally suffer from headaches, palpitations, and sweating. Hypertension can present in a sustained or paroxysmal form. Diagnosis requires confirmation by biochemical methods of elevation of fractionated catecholamines and metanephrines in plasma or urine for 24 hours, in addition to imaging evidence of tumor location. The treatment of choice is surgery, although prior to its performance, patients must have hypertension under control. In cases of malignant tumors, treatment with radiation and chemotherapy is recommended. The benefit of many therapies directed against molecular targets in these patients is currently being studied. This article aims to carry out a review of this tumor biological aspect, its clinical presentation, diagnostic methods and the most recent therapeutic strategies.
