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/Von Hippel-Lindau Sendromlu Aile: Indiyum-111 Somatostatin Reseptor Sintigrafisi, Iyot-123 Metaiyodobenziiguanidin Sintigrafisi ve Biigisayarh Tek Foton Emisyoniu Tomografisi Buigulari

• Published in: Molecular imaging and radionuclide therapy 2017-02, Vol.26 (1), p.38
• Main Authors: Ancan, Pelin, Tekin, Bema Okudan, Naldoken, Seniha, Sefizade, Riza, Berker, Dilek
• Format: Article
• Language: English
• Subjects: CAT scans; Development and progression; Diagnosis; Genetic aspects; Health aspects; Indium; Pancreatic cancer
• ISSN: 2146-1414
• DOI: 10.4274/mirt.70894

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 (1-123 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 1-123 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, 1-123 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 1-123 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. Keywords: Von Hippel-Lindau disease, neuroendocrine tumors, scintigraphy, single-photon emission computerized tomography Von Hippel-Lindau sendromu (VHLS) malign ve benign tumorler ile karakterize otozomal dominant herediter ailesel bir hastaliktir. VHLS'li hastalarda adrenal ve pankreatik kitlelerin ayirici tanisi zordur. Bu hastalarda iyot-123 metaiyodobenziiguanidin (I-123 MIBG) ve indiyum-111 somatostatin reseptor sintigrafileri (In-111 SRS) adrenal ve pankreatik kitlelerin ayinci tanisinda onemli rol oynar. Bu olgu sunumunda VHLS'li ailenin uc uyesinin 1-123 MIBG biigisayarh tek foton emisyoniu tomografisi (SPECT/BT) ve In-111 SRS SPECT/BT bulgularini sunuyoruz. Olgu 1'de, In-111 SRS SPECT/BT goruntulerinde pankreas basinda rezidu noroendokrin tumor (NET) saptandi. Olgu 2 ve 3'de, 1-123 MIBG SPECT/BT cerrahi oncesi adrenal bezdeki kitleleri feokromositoma ve adrenal disi kitleyi NET olarak onayladi. In-111 SRS ve 1-123 MIBG sintigrafilerinin VHLS'li hastalann tani ve tedavisi icin rutin kullanimda yararli olabilecegini dusunduk. Hibrid SPECT/BT morfolojik ve fonksiyonel bilgileri birlikte degerlendirdigi icin, planar goruntulerin tanisal dogrulugunu artirir. Anahtar kelimeler: Von Hippel-Lindau hastaligi, noroendokrin tumorler, sintigrafi, bilgisayarli tek foton emisyoniu tomografi