Multiple Pheochromocytomas in a Patient With Blurred Vision

• Published in: Clinical nuclear medicine 2008-09, Vol.33 (9), p.597-601
• Main Authors: Thoren, Kendra L, Balingit, Antonio G, Billingsley, Jerome
• Format: Article
• Language: English
• Subjects: 3-Iodobenzylguanidine; Adult; Brain - diagnostic imaging; Humans; Magnetic Resonance Imaging; Male; Pheochromocytoma - complications; Pheochromocytoma - diagnosis; Pheochromocytoma - diagnostic imaging; Pheochromocytoma - surgery; Spine - diagnostic imaging; Tomography, X-Ray Computed; Vision Disorders - complications; Vision Disorders - diagnosis; Vision Disorders - diagnostic imaging
• ISSN: 0363-9762; 1536-0229
• DOI: 10.1097/RLU.0b013e3181813096

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 α- and β-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.