Oh Jong-Ryool, Kulkarni Harshad, Carreras Cecilia, Schalch Georg, Min Jung-Joon, Baum Richard P
Department of Nuclear Medicine, Chonnam National University Hwasun Hospital, Hwasun, South Korea.
Department of Nuclear Medicine and Center for PET/CT, Zentralklinik Bad Berka, 99437 Bad Berka, Germany.
Nucl Med Mol Imaging. 2012 Jun;46(2):129-33. doi: 10.1007/s13139-012-0133-0. Epub 2012 Apr 27.
Von Hippel-Lindau (VHL) disease is a dominantly inherited familial cancer syndrome with a variety of benign and malignant tumors such as retinal and central nervous system hemangioblastomas, endolymphatic sac tumors, renal cysts and tumors, pancreatic cysts and tumors, pheochromocytomas, and epididymal cystadenomas. Cross-sectional modalities (computed tomography and magnetic resonance imaging) as well as ultrasound play a major role in the initial evaluation and follow-up of the various manifestations of VHL disease. Ga-68-labeled somatostatin receptor analogs already have a significant role in the diagnosis, staging, and therapy management of neuroendocrine neoplasms and neural crest tumors. Herein, we report a case presenting a variety of malignancies in VHL and showing the usefulness of Ga-68 somatostatin receptor PET/CT as a one-stop-shop imaging modality in the management of VHL disease.
冯·希佩尔-林道（VHL）病是一种常染色体显性遗传的家族性癌症综合征，可出现多种良性和恶性肿瘤，如视网膜和中枢神经系统血管母细胞瘤、内淋巴囊肿瘤、肾囊肿和肿瘤、胰腺囊肿和肿瘤、嗜铬细胞瘤以及附睾囊腺瘤。横断面检查方法（计算机断层扫描和磁共振成像）以及超声在VHL病各种表现的初始评估和随访中起主要作用。镓-68标记的生长抑素受体类似物在神经内分泌肿瘤和神经嵴肿瘤的诊断、分期及治疗管理中已发挥重要作用。在此，我们报告1例VHL病伴多种恶性肿瘤的病例，并展示镓-68生长抑素受体PET/CT作为一站式成像方式在VHL病管理中的实用性。
