Bilateral Pheochromocytoma and Paraganglioma Tumors Due to Von Hippel-Lindau Syndrome in a 15-Year-Old Boy: A Case Report.

ABSTRACT

Pheochromocytomas and paragangliomas are catecholamine-secreting tumors that originate from chromaffin cells of the adrenal medulla and autonomic neural ganglia, respectively. Patients with pheochromocytomas and paragangliomas typically present with paroxysmal headache, sweating, tachycardia, and hypertension. Although most pheochromocytoma cases are sporadic, many of the cases occur as part of a genetic disorder. Here, we report the case of a 14-year-old male patient who presented with hypertension. Laboratory tests showed elevated levels of serum and urinary catecholamines, metanephrines, and chromogranin. Abdominal ultrasound and computed tomography studies revealed bilateral solid adrenal masses and an isolated splenic mass. Further assessment identified an underlying Von Hippel-Lindau syndrome. The patient was initially treated medically and later surgically. This case highlights the importance of always considering pheochromocytomas and paragangliomas as rare differentials of secondary hypertension, especially in the presence of episodic headaches, sweating, and tachycardia. Furthermore, screening for underlying genetic disorders, such as in our case, should be considered in cases of bilateral tumors, onset at a young age, and presence of extra-adrenal tumors.