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We report on a 19-year-old male diagnosed with multiple pheochromocytomas after an I-123 metaiodobenzylguanidine (MIBG) scan. Multiple imaging was obtained because of concern for von Hippel-Lindau disease (VHL) after bilateral retinal angiomas were discovered on ophthalmologic consultation as a follow-up from the Emergency Department. Computed tomography (CT) and magnetic resonance imaging (MRI) were able to localize the adrenal and extra-adrenal tumors; however, accurate differentiation between pheochromocytomas and other adrenal tumors was not possible. I-123 MIBG is the test of choice for evaluating functionality and location of pheochromocytomas because of its high sensitivity and specificity. I-123 MIBG was performed before surgery to confirm that the lesions were indeed pheochromocytomas and also to rule out metastatic disease. He was started on alpha- and beta-blocker medication for at least 3 weeks, followed by surgery. The postoperative period was uneventful. DNA testing was performed and revealed mutations in the VHL gene previously reported to be associated with von Hippel-Lindau syndrome type II and genetic counseling was recommended. He was presented in urology tumor board conference with recommendations for follow-up CT and laboratory tests after 3 months. Otherwise, the patient is doing well and asymptomatic at this time.

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