[Advances in the etiopathogenesis, diagnosis and treatment of paragangliomas].

Paragangliomas are rare, benign or less frequently malignant tumors developing from cells of the paraganglia, a diffuse neuroendocrine system dispersed from skull base to the pelvic floor. Although paragangliomas may arise in any portion of this system, they most commonly occur below the diaphragm. Even 50% of the tumors may be hereditary and therefore genetic testing should be taken into consideration in all patients with paragangliomas. In many patients their presence leads to headaches, palpitations, sweating, or hypertension. However, subjects with nonfunctional tumors are either asymptomatic at presentation or experience only local symptoms caused by the mass effect. Functional paragangliomas can be almost always revealed by measurements of plasma concentrations of free metanephrines or 24-hour urinary outputs of fractionated metanephrines and catecholamines. There are also several morphological and functional imaging methods available that help localize neoplasm and assess its extent. In this paper, we discuss the published literature on the etiopathogenesis, diagnostic work up and the different treatment options for patients with paraganglioma. This review looks also at the recent advances in the physiology and molecular basis of these tumors.