家族性肿瘤综合症Von Hippel-Lindau（VHL）病以多种良性和恶性肿瘤为特征，已知这些肿瘤中表达生长抑素受体（SSTR）。我们评估了68Ga-DOTANOC PET/CT扫描在VHL基因阳性的原发或随访中，对复发或同步/间断病变的检测作用。共回顾性分析了14例患者（男性8例，女性6例），平均年龄30±9.86岁。这些患者在基因剂量分析中呈阳性反应，并接受了用于疾病评估的68Ga-DOTANOC PET/CT扫描。确定了病变的数量和部位。通过计算病变的最大标准摄取值（SUVmax）对探针摄取进行了半定量分析。 其中4例患者进行了初始诊断的扫描作为基线，6例患者进行了术后疾病情况的扫描，还有4例患者进行了初始诊断和随访疾病评估的扫描。在14例患者中共检测出67个病变。病变部位包括小脑/椎管/脊柱（17个，平均SUVmax = 7.85）；胰腺神经内分泌肿瘤（NET）（11个，平均SUVmax = 20.64）；视网膜（3个，平均SUVmax = 10.46）；嗜铬细胞瘤（10个，平均SUVmax = 16.32）；副神经节瘤（3个，平均SUVmax = 10.65）；胰腺囊肿（9个，平均SUVmax = 2.54）；肾囊肿（8个，平均SUVmax = 1.56）和其他病变共6个。 我们的结果显示，68Ga-DOTANOC PET/CT可能是对患有VHL的患者进行相关肿瘤筛查和随访的有用手段。它可以作为一种一站式影像学方法，用于VHL患者，并可以替代单独的放射学检查，从而在时间和费用方面更加方便患者。© The Author(s), under exclusive licence to Korean Society of Nuclear Medicine 2023. Springer Nature或其许可方（例如协会或其他合作伙伴）根据与作者或其他权利持有人签订的出版协议，对本文拥有排他性权利；根据此类出版协议和适用法律，作者自行存档接受的手稿版本。

Hereditary tumor syndrome Von Hippel-Lindau (VHL) disease is characterized by various benign and malignant tumors that are known to express somatostatin receptors (SSTR). We evaluated the role of 68Ga-DOTANOC PET/CT scan in patients with positive germline mutation of the VHL gene, presented initially or on follow-up, for the detection of recurrent or synchronous/metachronous lesions.Fourteen patients (8 males; 6 females) with mean age 30 ± 9.86 years were retrospectively analyzed, were tested positive for VHL on gene dosage analysis, and underwent 68 Ga-DOTANOC PET/CT scan for disease evaluation. The number and site of lesions were determined. The tracer uptake was analyzed semi-quantitatively by calculating the maximum standardized uptake values (SUVmax) of lesion.Four of the 14 patients underwent scan for initial diagnosis as baseline, 6 patients for post-therapy disease status, and 4 patients for initial diagnosis as well as follow-up evaluation of the disease. A total of 67 lesions were detected in 14 patients. The sites of lesions were cerebellar/vertebral/spinal (17; mean SUVmax = 7.85); pancreatic neuroendocrine tumor (NET) (11; mean SUVmax = 20.64); retina (3; mean SUVmax = 10.46); pheochromocytoma (10; mean SUVmax = 16.32); paragangliomas (3; mean SUVmax = 10.65); pancreatic cyst (9; mean SUVmax = 2.54); and renal cyst (8; mean SUVmax = 1.56) and miscellaneous lesions constituted 6 lesions.Our results show that 68 Ga-DOTANOC PET/CT may be a useful modality for screening and follow-up of associated tumors in patients with germline gene mutation for VHL. It can be used as a one-stop imaging modality for VHL patients and may substitute for separate radiological investigations, making it more convenient for patients in terms of time and cost.© The Author(s), under exclusive licence to Korean Society of Nuclear Medicine 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.