Summary
Pheochromocytoma

Pheochromocytoma (Pheo) and paraganglioma (PGL) are rarely seen endocrine tumors derived from the adrenal medulla or the neuronal crest known as paraganglia. The Pheo or PGL may emerge sporadically or be part of a hereditary tumor syndrome. These tumors originate by mutation in the VHL, RET genes, and may also compromise genes that are involved as part of syndromes. The clinic will depend on the functionality or sympathetic predominance of Pheo/PGL. Signs and symptoms of sympathetic chromaffin tumor include sustained or paroxysmal hypertension episodes. Present the classic triad of headache, palpitations and diaphoresis of paroxysmal onset, pallor, orthostatic hypotension and syncope, tremor, anxiety. So also as chest pain, arrhythmias, cardiac hypertrophy, cardiomyopathy, to evolve at heart failure.
The diagnosis of Pheo/PGL has been simplified by advances in studies to detect and quantify the levels of catecholamines and their metabolites in blood and urine. The location of the tumor can be through computed tomography and magnetic resonance imaging useful methods. The functional test using nuclear scintigraphy with metaiodobenzylguanidine (MIBG) is highly specific to confirm the natural secretion of catecholamines by the tumor. Pre-treatment is performed for surgical resection of the tumor, starting 1 to 2 weeks prior to surgery. The prognosis of the resected tumor as a whole is very good. To date there is no staging system for Pheo/PGL malignant and life expectancy depend on the location of metastases (MTS), with a lower 5-year survival in patients with pulmonary or hepatic MTS level. May also be observed a simile syndrome stress cardiomyopathy (Tako-tsubo), i.e. the Pheo is able to produce a reversible cardiomyopathy. Early diagnosis of Pheo/PGL may reduce your morbidity and mortality if not diagnosed and may lead to death.

Keywords:Pheochromocytoma ; Paraganglioma ; Hypertension ; Adrenal medulla ; Chromaffin ; Catecholamines