PDF(Pubmed)
Abstract：
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.
摘要：
与vonHippel-Lindau（VHL）疾病相关的血管母细胞瘤通常是多发性的，并且在长时间的随访中复发。目前,对于这些肿瘤没有可获得的全身治疗.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的表达。值得注意的是,肿瘤中生长抑素受体表达增加，通过肽受体放射性核素显像确定，是对生长抑素类似物和肽受体放射性核素治疗的反应的预测因素。这项研究的目的是描述与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情况，并对VHL相关血管母细胞瘤患者的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岁男子的病例，该患者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）的肽受体放射性核素显像发现，鞍上血管母细胞瘤中生长抑素受体的表达增加，伴有多发性胰腺神经内分泌肿瘤和双侧嗜铬细胞瘤。患者接受了lanreotide治疗1年，生长抑素类似物.开始lanreotide1年后重复68Ga-DOTATOC显示血管母细胞瘤对放射性示踪剂的摄取减少，与代谢反应一致。生长抑素受体在与VHL疾病相关的血管母细胞瘤中的存在是一个新发现。用生长抑素类似物治疗后这些受体的表达降低，如本案所述，将生长抑素受体定位为新诊断的新靶点，治疗性的,VHL病患者的随访机会。
