与 von Hippel-Lindau (VHL) 病相关的血管母细胞瘤经常是多发的，并且在长期随访期间会复发。目前，尚无针对这些肿瘤的全身治疗方法。最近的研究表明生长抑素受体在这些类型的血管母细胞瘤中表达。值得注意的是，通过肽受体放射性核素成像测定，肿瘤中生长抑素受体表达的增加是对生长抑素类似物和肽受体放射性核素治疗反应的预测因素。本研究的目的是描述一名与 VHL 疾病相关的鞍上血管母细胞瘤中生长抑素受体表达增加的患者的病例，并对 VHL 相关血管母细胞瘤患者中生长抑素受体表达进行文献综述。我们在此描述了一名患有 VHL 疾病的 51 岁男性病例，他在磁共振成像中检测到鞍上血管母细胞瘤。使用镓-68-DOTATOC (68Ga-DOTATOC) 进行的肽受体放射性核素成像发现，鞍上血管母细胞瘤以及多发性胰腺神经内分泌肿瘤和双侧嗜铬细胞瘤中生长抑素受体的表达增加。该患者接受兰瑞肽（一种生长抑素类似物）治疗一年。开始兰瑞肽一年后重复 68Ga-DOTATOC 显示血管母细胞瘤对放射性示踪剂的吸收减少，这与代谢反应一致。与 VHL 疾病相关的血管母细胞瘤中存在生长抑素受体是一项新发现。如本例所述，用生长抑素类似物治疗后这些受体的表达降低，使生长抑素受体成为 VHL 疾病患者新的诊断、治疗和随访机会的新靶点。

Hemangioblastomas associated with von Hippel-Lindau (VHL) disease are frequently multiple and recur during prolonged follow-up. Currently, no systemic treatment is available for these tumors. Recent studies have shown the expression of somatostatin receptors in these types of hemangioblastomas. Notably, increased somatostatin receptor expression in a tumor, as determined by peptide-receptor radionuclide imaging, is a predictive factor of response to treatment with somatostatin analogs and peptide-receptor radionuclide therapy. The aim of this study was to describe the case of a patient with increased expression of somatostatin receptors in a suprasellar hemangioblastoma associated with VHL disease and conduct a literature review on somatostatin receptor expression in patients with VHL-associated hemangioblastomas. We describe herein the case of a 51-year-old man with VHL disease who had a suprasellar hemangioblastoma detected on magnetic resonance imaging. Peptide-receptor radionuclide imaging using gallium-68-DOTATOC (68Ga-DOTATOC) identified increased expression of somatostatin receptors in the suprasellar hemangioblastoma, along with multiple pancreatic neuroendocrine tumors and bilateral pheochromocytomas. The patient was treated for 1 year with lanreotide, a somatostatin analog. A repeat 68Ga-DOTATOC 1 year after starting lanreotide revealed decreased radiotracer uptake by the hemangioblastoma, consistent with a metabolic response. The presence of somatostatin receptors in hemangioblastomas associated with VHL disease is a novel finding. The decreased expression of these receptors after treatment with a somatostatin analog, as described in the present case, positions the somatostatin receptor as a new target for novel diagnostic, therapeutic, and follow-up opportunities in patients with VHL disease.