Feocromocitoma: nuevas perspectivas en diagnóstico y seguimiento

DRA ANA VIEITES; DRA MARTA BARONTINI; DRA GABRIELA SANSO; DR IGNACIO BERGADA

Revista Hospital de Niños Ricardo Gutierrez

HNRG

Lugar: CABA; Año: 2018 vol. 60

0521-517X

Arterial hypertension is a rare condition in paediatrics, but due to the high proportion of secondary causes, the search for the aetiology is crucial for an adequate diagnosis and treatment.Pheochromocytoma is an endocrine tumor originated in the chromaffin tissue. It is an important cause of secondary hypertension. It can be sporadic or part of several hereditary syndromes, such as von Hippel Lindau disease, Multiple Endocrine Neoplasia type 2, Pheochromocytoma / Familiar Paraganglioma syndromes type 1, 2, 3 and 4, and type 1 Neurofibromatosis. In addition, more than 15 susceptible genes have been described in familial pheochromocytoma. Being highly heritable tumors, genetic testing of tumor susceptibility is recommended in all patients, even in absence of a family history. The diagnosis of the hereditary disease improves the patient´s attention and allows early diagnosis in relatives.