RESUMEN
Sporadic pheochromocytoma is a rare tumor that should be taken into account in patients with hypertensive crisis, arrhythmias, and panic disorder. Familial pheochromocytoma is frequently found in subjects with von Hippel-Lindau disease, multiple endocrine neoplasia type II, neurofibromatosis, and SDHD gene mutations. The prevalence of sporadic pheochromocytoma is very low, approximately 0.05% among subjects with essential hypertension and even less in the general population. However, aggressive diagnostic intervention is recommended whenever a pheochromocytoma is suspected because the uncontrolled catecholamine release from the tumor can lead to serious and potentially lethal complications. Plasma free metanephrines have been shown to have high sensitivity and specificity in the biochemical diagnosis of sporadic and familial pheochromocytoma. Measurement of 24-hour urinary fractionated metanephrines may be an acceptable alternative in many patients. The current approach to the diagnostic localization of pheochromocytoma relies on computed tomography (CT), magnetic resonance imaging (MRI) and [123-I] and [131-I] MIBG scintigraphy. CT and MRI have very high sensitivity but low specificity, whereas MIBG scintigraphy has good specificity but its sensitivity is less than optimal, especially for the detection of metastases. In difficult cases, PET imaging appears to be promising.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/complicaciones , Hipertensión/etiología , Feocromocitoma/complicaciones , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/fisiopatología , Algoritmos , Humanos , Sistemas Neurosecretores/fisiopatología , Feocromocitoma/diagnóstico , Feocromocitoma/fisiopatología , Sistema Nervioso Simpático/fisiopatologíaRESUMEN
We report one sixty-seven years-old female who presented with hypertension refractory to antihypertensive drugs. She had an elevated BP for approximately 15 years. In the last 8-10 months her hypertension had become difficult to control. Her BP ranged between 180/100 mmHg and 220/1220 mmHg on atenolol 100 mg once daily, methyldopa 500 mg three times daily, furosemide 25 mg twice daily, doxazosine 4 mg twice daily. When she was referred to our unit serum creatinine was 2.3 mg/dL and she had a mild proteinuria (70 mg/dL) without microematuria. Ultrasonography showed a left kidney size in the low-normal range (LD 11 cm) and a small right kidney (LD 9 cm). Renal angiography showed a severe, ostial stenosis of the left renal artery and a total thrombosis of the right renal artery with a blood supply to the right kidney provided by collateral channels. An ACE-I was added to the therapy but a sharp increase in serum creatinina (up to 6.4 mg/dL) prompted us to withdraw the drug. She underwent a renal angioplasty on the left side and a Palmaz stent was placed. The control angiography showed a good anatomical result. Three months after the manoeuvre the patient was again referred to our unit with headache, nausea vomiting and hyper-tension refractory to amlodipine 10 mg/day, doxazosine 4 mg twice a a day, atenolol 50 mg/day, furosemide 50 mg/day. A doppler ultrasonography and a magnetic resonance angiogram showed no restenosis on the treated artery. An ACE-I was again administered and BP on this drug was 145/90 mmHg after one month and 130/85 after three months. Headache, nausea and vomiting disappeared. Serum creatinina kept unchanged (2.2 mg/dL). Comment. In this case the benefit of angioplasty on blood pressure control was indirect. Apparently the manoeuvre showed no effect on blood pressure, but the angioplasty allowed us to use of an ACE-Inhibitor, without any negative effect on renal function, and thus to adequately control blood pressure.