title: 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.
abstract: Von Hippel-Lindau syndrome (VHLS) is an autosomal dominant hereditary familial disorder characterized by development of malignant and benign neoplasms. Differential diagnosis of the adrenal and pancreatic masses are difficult in patients with VHLS. Iodine-123 metaiodobenzylguanidine (I-123 MIBG) and indium-111 somatostatin receptor scintigraphies (In-111 SRS) have important roles in the differential diagnosis of adrenal and pancreatic masses in those patients. In this case report, we present the findings of I-123 MIBG single-photon emission computerized tomography (SPECT/CT) and In-111 SRS SPECT/CT in three members of a family with VHLS. In case 1, a residual neuroendocrine tumor (NET) was detected in the head of pancreas on In-111 SRS SPECT/CT images. In case 2 and 3, I-123 MIBG SPECT/CT confirmed the adrenal masses as pheochromocytoma, and the extra-adrenal mass as NET, before surgery. We thought that In-111 SRS and I-123 MIBG scan might be helpful in the routine work up of VHLS patients for diagnostic and therapeutic purposes. Hybrid SPECT/CT system may improve diagnostic accuracy of planar images since it assesses morphologic and functional information together.
authors.affiliation: Ankara Numune Training and Research Hospital, Clinic of Nuclear Medicine, Ankara, Turkey Phone: +90 542 286 75 75 E-mail: psarican@yahoo.com.
authors.forename: Pelin
authors.initials: P
authors.lastname: Arıcan
authors.forename: Berna
authors.initials: B
authors.lastname: Okudan Tekin
authors.forename: Seniha
authors.initials: S
authors.lastname: Naldöken
authors.forename: Rıza
authors.initials: R
authors.lastname: Şefizade
authors.forename: Dilek
authors.initials: D
authors.lastname: Berker