Rare association of aortoarteritis and pheochromocytoma
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Pheochromocytoma is an uncommon but treatable cause of secondary hypertension, it may present with a wide variety of manifestations. The coexistence of pheochromocytoma and vascular abnormalities is described but rarely reported entity. A 36-year-old man admitted to the hospital for severe hypertension. His past medical history was remarkable. He was hospitalized 3 years ago, for the management of a left lenticular cerebral hematoma in the context of hypertensive emergency. He was lost to follow up. Thorough questioning elicited also a history of intermittent headache with sweating and paroxysmal attacks of palpitations with recurrent abdominal pain and severe intermittent claudication of lower limbs (IIB of Leriche and Fontain’s classification).

On physical examination, the patient had an acute hypertensive crisis with an elevated blood pressure of 240/120 mmHg symmetric in the upper limbs, he had sinus tachycardia with a pulse of 110 beats/minute. The patient was anxious, with facial flushing. The cardiovascular examination showed bilateral abolition of the femoral pulse without signs of acute ischemia. The fundus examination revealed a bilateral papillary edema stage IV. Hypertension was initially managed with nicardipine infusion. The electrocardiogram showed sinus tachycardia and ST and T wave changes consistent with left ventricular hypertrophy. The chest radiograph was within normal limits. Transthoracic echocardiography revealed left ventricular hypertrophy with normal systolic function and without any significant valve disease.

Computed tomography (CT) from the thorax to pelvis revealed a right adrenal mass, hypodense, heterogeneously enhancing, enclosing a liquefied area, measuring 42?×?35 mm. CT angiogram showed thickening of the thoracoabdominal aortic wall and the proximal portions of some of its branches suggestive of aortoarteritis with stenosis of more than 50% of the origin of the celiac trunk, trunk stenosis of the right renal artery, bilateral occlusion of the external iliac arteries. The endocrinological evaluation revealed that urinary metanephrines were increased. Plasma cortisol, 24 h cortisol urine levels were within normal ranges. On the other hand dosage of ANCA, rheumatoid factor, tumor markers were negative, antinuclear antibody level was in the upper limit. Thus, the diagnosis of right pheochromocytoma was made, and surgical treatment was recommended. After appropriate preoperative management (blood pressure was 130/80 mmHg on doxazosin 4 mg/day and nicardipine LP 50 mg twice a day), he underwent laparoscopic right adrenalectomy without any complications.

Light microscopy of the specimen revealed characteristic organoid or zellballen nest of cells confirming the diagnosis of pheochromocytoma without signs of vasculitis. During the postoperative period, the blood pressure was normal without any surgical complications. One month after surgery, the blood pressure remained normal without any antihypertensive therapy, the patient had less invalid intermittent claudication with a walking perimeter of more than200 m but still have intermittent abdominal pain. Dosage of 24 hours urinary metanephrines returned normal.

Source:https://www.sciencedirect.com/science/article/pii/S2210261220309263
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