IRCCS, Azienda Ospedaliero-Universitaria di Bologna, Italy; Nuclear Medicine, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Italy.
Nuclear Medicine Department, Medical University of Warsaw, Warsaw, Poland.
Eur J Cancer. 2021 Mar;146:56-73. doi: 10.1016/j.ejca.2021.01.008. Epub 2021 Feb 12.
Nuclear medicine plays an increasingly important role in the management neuroendocrine neoplasms (NEN). Somatostatin analogue (SSA)-based positron emission tomography/computed tomography (PET/CT) and peptide receptor radionuclide therapy (PRRT) have been used in clinical trials and approved by the European Medicines Agency (EMA) and the US Food and Drug Administration (FDA). European Association of Nuclear Medicine (EANM) Focus 3 performed a multidisciplinary Delphi process to deliver a balanced perspective on molecular imaging and radionuclide therapy in well-differentiated neuroendocrine tumours (NETs). NETs form in cells that interact with the nervous system or in glands that produce hormones. These cells, called neuroendocrine cells, can be found throughout the body, but NETs are most often found in the abdomen, especially in the gastrointestinal tract. These tumours may also be found in the lungs, pancreas and adrenal glands. In addition to being rare, NETs are also complex and may be difficult to diagnose. Most NETs are non-functioning; however, a minority present with symptoms related to hypersecretion of bioactive compounds. NETs often do not cause symptoms early in the disease process. When diagnosed, substantial number of patients are already found to have metastatic disease. Several societies' guidelines address Neuroendocrine neoplasms (NENs) management; however, many issues are still debated, due to both the difficulty in acquiring strong clinical evidence in a rare and heterogeneous disease and the different availability of diagnostic and therapeutic options across countries. EANM Focus 3 reached consensus on employing gallium-labelled somatostatin analogue ([Ga]Ga-DOTA-SSA)-based PET/CT with diagnostic CT or magnetic resonance imaging (MRI) for unknown primary NET detection, metastatic NET, NET staging/restaging, suspected extra-adrenal pheochromocytoma/paraganglioma and suspected paraganglioma. Consensus was reached on employing fluorine-fluoro-2-deoxyglucose ([F]FDG) PET/CT in neuroendocrine carcinoma, G3 NET and in G1-2 NET with mismatched lesions (CT-positive/[Ga]Ga-DOTA-SSA-negative). Peptide receptor radionuclide therapy (PRRT) was recommended for second line treatment for gastrointestinal NET with [Ga]Ga-DOTA-SSA uptake in all lesions, in G1/G2 NET at disease progression, and in a subset of G3 NET provided all lesions are positive at [F]FDG and [Ga]Ga-DOTA-SSA. PRRT rechallenge may be used for in patients with stable disease for at least 1 year after therapy completion. An international consensus is not only a prelude to a more standardised management across countries but also serves as a guide for the direction to follow when designing new research studies.
核医学在神经内分泌肿瘤(NEN)的管理中发挥着越来越重要的作用。基于生长抑素类似物(SSA)的正电子发射断层扫描/计算机断层扫描(PET/CT)和肽受体放射性核素治疗(PRRT)已在临床试验中使用,并获得了欧洲药品管理局(EMA)和美国食品和药物管理局(FDA)的批准。欧洲核医学协会(EANM)焦点 3 进行了一项多学科德尔菲流程,以提供对分化良好的神经内分泌肿瘤(NET)的分子成像和放射性核素治疗的平衡观点。NET 形成于与神经系统相互作用的细胞或产生激素的腺体中。这些细胞称为神经内分泌细胞,可以在全身找到,但 NET 最常见于腹部,特别是胃肠道。这些肿瘤也可能发生在肺部、胰腺和肾上腺。除了罕见之外,NET 还很复杂,可能难以诊断。大多数 NET 是无功能的;然而,少数表现出与生物活性化合物过度分泌相关的症状。NET 在疾病早期通常不会引起症状。诊断时,大量患者已经发现患有转移性疾病。一些协会的指南解决了神经内分泌肿瘤(NEN)的管理问题;然而,由于在一种罕见且异质的疾病中获取强有力的临床证据存在困难,以及各国诊断和治疗选择的不同,许多问题仍存在争议。EANM Focus 3 就使用镓标记的生长抑素类似物 ([Ga]Ga-DOTA-SSA) 进行基于 PET/CT 的诊断 CT 或磁共振成像 (MRI) 用于检测未知原发性 NET、转移性 NET、NET 分期/重新分期、疑似肾上腺外嗜铬细胞瘤/副神经节瘤和疑似副神经节瘤达成了共识。在神经内分泌癌、G3 NET 和 CT 阳性/[Ga]Ga-DOTA-SSA 阴性的 G1-2 NET 中,也达成了使用氟-氟-2-脱氧葡萄糖 ([F]FDG) PET/CT 的共识。肽受体放射性核素治疗 (PRRT) 被推荐用于所有病变摄取 [Ga]Ga-DOTA-SSA 的胃肠道 NET 的二线治疗、G1/G2 NET 的疾病进展以及 G3 NET 的亚组,前提是所有病变在 [F]FDG 和 [Ga]Ga-DOTA-SSA 中均为阳性。在治疗完成后至少 1 年疾病稳定的患者中可以重复 PRRT 治疗。国际共识不仅是各国更标准化管理的前奏,也是设计新研究时的指导方向。