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.