[Pheochromocytoma associated with von Recklinghausen neurofibromatosis].
A pheochromocytoma is an adrenal gland tumor that secretes epinephrine and norepinephrine hormones, and is responsible for regulating heart rate and blood pressure, among other functions. The condition can occur alone or in combination with other disorders, and genetic and environmental factors play a key role. Neurofibromatosis-1 (NF-1) an inherited "autosomal dominant" disorder is one of the most common genetic disorders, characterized by formation of neurofibromas (tumors involving nerve tissue) in the skin, subcutaneous tissue, cranial and spinal root nerves. NF1 generally is diagnosed by physical examination. There is no cure for NF1, but there are ways to treat some of its effects. Neurofibromatosis arterial hypertension caused by pheochromocytoma is extremely rare, less frecuent than 1% in childrens less than 10 years old, and young adults. We present a case of an extremely infrequent association between neurofibromatosis and a pheochromocytoma in a young woman with a newly diagnosed hypertension. We discuss the underlying pathophysiological mechanisms and clinical implications.
Herrera
Ramón N
RN
Departamento de Clínica Médica, Hospital Centro de Salud Zenón J. Santillán, Tucumán, Argentina. nicasioherrera@arnet.com.ar
Miotti
Julio A
JA
Fuentes
Claudio M
CM
Robles
Silvia P
SP
Amenabar
Jesús M
JM
Luciardi
Héctor L
HL
D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000175:diagnosis; D000328:Adult; D005260:Female; D006801:Humans; D006973:Hypertension / Q000209:etiology; D009682:Magnetic Resonance Spectroscopy; D009456:Neurofibromatosis 1 / Q000150:complications*; D010673:Pheochromocytoma / Q000150:complications* / Q000175:diagnosis*