Multiple Pheochromocytomas in a Patient With Blurred Vision

Abstract

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.

A Family with Von Hippel-Lindau Syndrome: The Findings of Indium-111 Somatostatin Receptor Scintigraphy, Iodine-123 Metaiodobenzylguanidine Scintigraphy and Single Photon Emission Computerized Tomography

“…Recurrence of bilateral pheochromocytoma was determined with I-123 MIBG scan in that case. Thoren et al (7) reported a case with multiple pheochromocytomas and VHLS. They performed I-123 MIBG before surgery to confirm that the lesions were indeed pheochromocytoma and to rule out any metastatic disease.…”

Fluorodeoxyglucose positron emission tomography-computed tomography scan in von Hippel-Lindau syndrome: A case report and review of literature

“…[18] Iodine-123 MIBG has been reported to diagnose multiple pheochromocytomas in VHL disease presenting initially with retinal angiomatosis. [19]…”

Functional Imaging of Paragangliomas with an Emphasis on Von Hippel–Lindau-Associated Disease: A Mini Review

“…The use of MIBG scintigraphy in a woman with VHL has changed fundamentally her management, as presented in an older case report, when scintigraphy indicated a metastatic PHEO in a lesion initially considered to be a brainstem hemangioblastoma ( 27 ); PHEO metastases were seen with 123 I-MIBG in another case report ( 28 ). However, subjects with VHL have lower VMATs expression in chromaffin tissue, possibly hampering the use of MIBG as an effective functional imaging agent ( 29 , 30 ).…”