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We report a case of a 10-year-old girl who presented with an acute neurological deficit preceded by a 2 month history of headaches. The patient was treated for acute viral encephalitis because of the presence of inflammation. A computed tomography (CT) scan of her brain scan showed a cerebral ischaemic stroke, and paroxysmal arterial hypertension led us to suspect pheochromocytoma. The diagnosis was confirmed by elevated urinary catecholamine and by imaging (abdominal x-ray and abdominal/pelvic CT scan). Bilateral adrenal gland tumours were successfully removed. This report illustrates an uncommon presentation of pheochromocytoma in children and shows the importance of a careful investigation for pheochromocytoma as a cause of hypertension in children with cerebral ischaemic stroke.
Pheochromocytoma is rare in children. It is of vital importance to know this atypical presentation of pheochromocytoma. The diagnosis of pheochromocytoma should be suspected in patients with focal cerebral symptoms, particularly in the presence of intermittent hypertension or other paroxysmal symptoms suggestive of pheochromocytoma.
A 10-year-old girl was admitted to our hospital for an acute neurological deficit preceded by a 2 month history of headaches. There was no family history of hypertension or pheochromocytoma. At the initial evaluation, the patient was pyrexic (40°C) with a blood pressure of 120/50 mm Hg; Glasgow Coma Scale (GCS) was estimated at 14 with a right hemiparesis and dysarthria.
Computed tomography (CT) scan of the brain showed left temporo-parietal hypodensity.
Complementary examinations indicated an inflammatory status (C reactive protein (CRP) 136 μg/ml, total white blood cell count (WBC) 13 000/mm3). Analysis of cerebrospinal fluid, obtained through a lumbar puncture, showed a clear fluid and a slight increase of white blood cells (25 WBCs/mm3) and normal concentrations of protein and glucose. Gram staining, microbiological culture and cerebrospinal fluid latex antigen were negative.
Treatment with acyclovir was started and 48 h later the patient’s neurological state had deteriorated, requiring tracheal intubation and artificial ventilation. A second brain CT scan was performed (fig 1A) which showed temporo-parietal ischaemia.
Paroxysmal arterial hypertension (160/100 mm Hg) led us to suspect pheochromocytoma. Our patient had transitional electric disorders on her electrocardiogram (ECG) with a normal troponin test. Echocardiography demonstrated total distension of the coronary artery.
Abdominal echocardiography and abdominal/pelvic CT were performed. The abdominal/pelvic CT revealed a 3.2 ×2.4 cm right suprarenal mass and a 2×1 cm left suprarenal mass (fig 1B). Evaluation of a 24 h urine specimen revealed a dopamine value of 2.97 μmol/day (normal <0.5 μmol/day).
The patient’s blood pressure was controlled with a β-blocker and an angiotensin I converting enzyme inhibitor. She then underwent a laparotomy and a large mass was identified above the right kidney. The tumour was removed with preservation of the right kidney, with total adrenalectomy on the right and partial adrenalectomy on the left. There was some intraoperative haemodynamic instability, with considerable hypertension during induction and arrhythmias that required administration of nicardipine and lidocaine.
Histologically, the tumour was composed of large pleomorphic chromaffin cells with no microscopic evidence for local invasion of tissue or blood vessels—features suggesting the tumour was benign.
Postoperatively, the patient’s hypertension has been easily controlled without treatment. Postoperative imaging (abdominal/pelvic CT scan, and thoracic magnetic resonance imaging (MRI)) has been negative for tumour recurrence and metastatic disease.
Pheochromocytomas are tumours arising from chromaffin cells of the adrenal medulla. These tumours are rare in children and account for 0.5–2.0% of all causes of hypertension in children.1 The presentation of pheochromocytoma in children has some distinct differences from its presentation in adults. Children tend to have a higher incidence of bilateral lesions (20–30%) and extra-adrenal lesions (20%).
Diagnosis of pheochromocytoma is important because the hypertension is usually curable by eradication of the tumour.2 Childhood adrenal tumours presenting with hypertension show an atypical course and a variable presentation. Reversal of hypertension by surgery is crucial. Hypertension appears to be uniformly present and is sustained in 80–90% of affected children at the time of diagnosis. Occasionally, children with sustained hypertension also have paroxysmal episodes. Wide fluctuations in blood pressure are characteristic, and pronounced increases may be followed by hypotension and syncope. Confirmation of hypertension is needed within 48 h, because of the paroxysmal episodes. However, 20% of children will remain asymptomatic.1
Cerebral ischaemia is a rare manifestation of pheochromocytoma. Van et al described two Taiwanese children with pheochromocytoma presenting as stroke with cerebral infarction and intracranial haemorrhage.2
Rakototiana et al reported the case of a 6-year-old boy whose pheochromocytoma was revealed by cerebral ischaemia as a consequence of acute cardiac failure.5 In our case, the ischaemia was in the territory supplied by the left middle cerebral artery. It resulted from vasospasm secondary to the hypertension or directly due to the high concentrations of catecholamines. Indeed, our patient had transitional electric disorders on her ECG with total distension of the coronary artery; this may also explain the vasospasm which remains a serious and potentially lethal cardiovascular complication.
Biochemical presentation of excessive catecholamine production is an essential step in the diagnosis of pheochromocytoma. Traditional biochemical tests include measurements of urinary and plasma catecholamine, urinary metanephrines (normetanephrine and metanephrine), and urinary vanillylmandelic acid (VMA). Measurements of plasma-free metanephrines (normetanephrine and metanephrine) represent a more recently available test.6
Chronic catecholamine excess in pheochromocytoma is accompanied by an increase in inflammation markers. Subjects with pheochromocytoma show significantly higher concentrations of leucocytes and neutrophils with positive proteins of the acute phase response (CRP, fibrinogen), as occurred in our case.
Among the atypical forms of phaeochromocytoma, the isolated inflammatory form is rare and difficult to diagnose clinically.
Imaging techniques such as CT or MRI and functional ligands such as 123I-MIBG are used to localise biochemically proven tumours. After the use of appropriate preoperative treatment to block the effects of secreted catecholamine, laparoscopic tumour removal is the preferred procedure. If removal of pheochromocytoma is timely, the prognosis is excellent. However, the prognosis is poor in patients with metastases, which occur particularly in patients with large, extra-adrenal tumours.
In our case, the clinical diagnosis of pheochromocytoma was proven by highly elevated urinary catecholamine, and confirmed histologically after the operation. The successful removal of the tumour led to the almost complete recovery of the neurological deficiencies.
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.