Pheochromacytomoe au cours dune maladie Recklinghausen

ABSTRACT

Recklinghausen Neurofibromatosis is a dominant autosomal disorder affecting one newborn over 2500 to 3500 births. Pheochromocytoma is observed at one to two% of patients affected by this disease. It can be revealed by a permanent or paroxystic hypertension, apostural hypotension or other particular manifestations anxiety, agitation,headaches, sweating, palpitations. The diagnosis is confirmed by biochemical measurements of plasma catecholamines and mainly their urinary metabolites [metanephrine, normetanephrine, vanillymandelic acid.]. The location of pheochromocytoma, necessary to guide the surgical treatment, relies on intravenous pyelography, ultrasonography, computed tomography and mainly scintigraphy. We report a case of pheochromocytoma revealed by hypertension occured in Recklinghausen Neurofibromatosis