SEVERE HYPERTENSION IN A YOUNG BOY WITH PARAGANGLIOMA OF THE ORGAN OF ZUCKERKANDL TREATED WITH THERMOABLATION

Abstract

Objective: Paragangliomas are tumors of the paraganglia, chromaffin tissue complexes of the neuroendocrine system distributed along the paravertebral and para-aortic axes. A small number of these tumors arise from the organ of Zuckerkandl that is the smallest endocrine gland, composed by paraganglia. These organs are accessory tissues of the autonomic nervous system that undergo involution during childhood. By adulthood distinct microscopic groups of extra-adrenal chromaffin cells endure and have the potential to develop into tumors that are rare cases of secondary hypertension. Design and method: A 19-year-old professional footballer, since two years, developed severe symptomatic hypertension forcing him to stop all activities. During each episode he reported headache, sweating, tachycardia, blurred vision and paradoxycal hypotension. No drugs were effective to lower blood pressure. Laboratory hormonal assessment were discordant, with high daily urinary cathecolamines but daily adrenaline, noradrenaline and dopamine in range. We performed a CT scan and a MRI of the abdomen that showed a nodular lesion of 7 mm near the left crus of diaphragm, where the left superior mesenteric artery originates (Figure 1). An iodine-123-meta-iodobenzylguanidine scan revealed an abnormal radiotracer uptake in the region of the mass identified by CT.Results: A multidisciplinary team composed by cardiologist, anesthesiologists and interventional radiologist, previous a particular preoperative management, performed an innovative surgical technique consisting in a two-steps CT-guided percutaneous radiofrequency ablation of the mass (Figure 2), at 65 and 100 W of energy for 5 minutes, after appropriate hydrodissection of perilesional tissues. A small tissue sample was obtained and pathology found the tissue was suggestive of an organ of Zuckerkandl. Conclusions: This is absolutely the first case in literature, since exist only few cases of robot-assisted laparoscopic excision, laparoscopic resection or laparotomy. The patient recovered well from the operation and was eventually discharged in a stable state. He's no longer suffering from hypertension and he started playing football again. 24-hour ambulatory blood pressure monitoring, after 15 days from discharge, shows satisfying blood pressure values (Figure 3).