IMPE Abstracts

Background: Paragangliomas (PGL) and pheochromocytomas (PHEO) are rare neuroendocrine tumors in children, derived from chromaffin cells in the adrenal medulla or associated with paravertebral ganglia, and produce dopamine and catecholamines (norepinephrine, and epinephrin).

Case presentation: A previously healthy 13-year-old girl, developed a severe pulsating headache associated with tachycardia, sweating, weight loss and recurrent vomiting, progressing to icteric sclera in one month. An abdominal ultrasound was performed and demonstrated biliary sludge and solid para-aortic nodular image, measuring 3.0x2.3cm. At this time, she only received symptomatic medication. One month later, during a public health center consultation, she persisted with jaundice, and had worsening vomiting, presented psychomotor agitation, tachycardia and loss of consciousness. The capillary blood glucose was 270mg/dL, blood pressure 180x120mmHg and heart rate 180bpm. A normal cranioencephalic computed tomography (CT) and high amylase level (549U/L, RV: 28-100) were detected, confirming pancreatitis and appropriate treatment prescribed. In the following month, the patient underwent complementary exams, which showed aldosterone of 48.9ng/dL (RV decubitus: 2.52-39.2 /supine: 1.76-23.2), renin of 83ng/mL/h (RV orthostatic position: 0.6-4.18 /supine position: 0.32-1.84). And a left para-aortic mass was detected on abdominal magnetic resonance imaging (MRI) at the time and hypertensive medication (doxazosin and verapamil) were prescribed and the patient was referred to a pediatric endocrinologist unit. Despite antihypertensive treatment, the patient maintained clinical symptoms. Endocrinological investigation evidenced a 24h urinary metanephrines of 66.9ug (VR:<320ug), normetanephrines of 2794.2ug (<390ug) and total metanephrines of 3439.6ug (<1000ug). An abdominal tomography showed an expansive mass, next to the anterior border of the psoas muscle and to the anterolateral border of the spine at the L2-L3 level, both to the left, measuring 5.7x3.5cm. The fundoscopic examination revealed grade IV of hypertensive retinopathy and echocardiography detected a left ventricular cardiac hypertrophy. The diagnosis of paranganglioma was established with target organ damage. At admission, she presented hypertensive peaks up to 210x120mmHg, with blood pressure fluctuations and significant tachycardia (>120bpm). After optimizing the blood pressure with doxazosin, metoprolol, enalapril and hydralazine, the patient underwent abdominal tumor resection. The immunohistochemical showed S-100 (polyclonal) protein-positive sustentacular cells, positive synaptrophysin (DAK-SYNAP clone) and Ki-67 <1% (MIB-1 clone). Almost one year after the procedure, the patient remained asymptomatic, with normal blood pressure and metanephrines, without medication.

Discussion/Conclusion: We report a rare case of paraganglioma in a 13-year-old girl. It is recommended to maintain annual follow-up, because the high-risk of metastases or recurrence.