Arıcan Pelin, Okudan Tekin Berna, Naldöken Seniha, Şefizade Rıza, Berker Dilek
Ankara Numune Training and Research Hospital, Clinic of Nuclear Medicine, Ankara, Turkey Phone: +90 542 286 75 75 E-mail:
Mol Imaging Radionucl Ther. 2017 Feb 5;26(1):38-42. 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 (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.
冯·希佩尔-林道综合征（VHLS）是一种常染色体显性遗传性家族性疾病，其特征为恶性和良性肿瘤的发生。对于VHLS患者，肾上腺和胰腺肿块的鉴别诊断较为困难。碘-123间碘苄胍（I-123 MIBG）和铟-111生长抑素受体闪烁扫描（In-111 SRS）在这些患者肾上腺和胰腺肿块的鉴别诊断中发挥着重要作用。在本病例报告中，我们展示了I-123 MIBG单光子发射计算机断层扫描（SPECT/CT）和In-111 SRS SPECT/CT在一个VHLS家族三名成员中的检查结果。病例1中，In-111 SRS SPECT/CT图像在胰头检测到残留神经内分泌肿瘤（NET）。病例2和3中，I-123 MIBG SPECT/CT在手术前确诊肾上腺肿块为嗜铬细胞瘤，肾上腺外肿块为NET。我们认为In-111 SRS和I-123 MIBG扫描可能有助于VHLS患者进行常规检查以用于诊断和治疗目的。混合SPECT/CT系统可同时评估形态学和功能信息，从而提高平面图像的诊断准确性。
