Badrane Ilham, Urso Luca, Campennì Alfredo, Cittanti Corrado, De Rimini Maria Luisa, Bartolomei Mirco
Department of Translational Medicine, University of Ferrara, Ferrara, Italy.
Nuclear Medicine Unit, Onco-Hematology Department, University Hospital of Ferrara, Ferarra, Italy.
Endocrine. 2025 Jan;87(1):67-72. doi: 10.1007/s12020-024-04062-1. Epub 2024 Oct 7.
The approval in 2017 by the European Medicines Agency (EMA) and in 2018 by the US Food and Drug Administration (FDA) of radioligand therapy (RLT) led to its wide application in therapeutic management of neuroendocrine neoplasms (NENs). However, the indications are currently limited to certain specific histotypes belonging to the broader NEN's family, mainly advanced well-differentiated gastro-entero-pancreatic NENs. As a consequence, several other tumors of the NEN spectrum that can potentially benefit, due to their biological characteristics, from RLT are still ineligible and can be considered "RLT-orphans". Among those, the subgroup of NENs originating from the sympathetic-adrenal-medullary (SAM) axis can be listed. This paper discusses the state of art and perspectives of the theragnostic applications in pheochromocytomas and paragangliomas, considering both the traditional theragnostic model - with radiolabelled metaiodobenzylguanidine (MIBG) - and the innovative one with radiolabeled somatostatin analogs (SSAs), that will hopefully become available for these patients in the near future.
2017年欧洲药品管理局（EMA）以及2018年美国食品药品监督管理局（FDA）对放射性配体疗法（RLT）的批准，使其在神经内分泌肿瘤（NENs）的治疗管理中得到广泛应用。然而，目前其适应症仅限于更广泛的NEN家族中的某些特定组织学类型，主要是晚期高分化胃肠胰神经内分泌肿瘤。因此，由于其生物学特性可能从RLT中获益的其他几种NEN谱系肿瘤仍不符合条件，可被视为“RLT孤儿”。其中，起源于交感-肾上腺髓质（SAM）轴的NEN亚组可被列出。本文讨论了嗜铬细胞瘤和副神经节瘤中诊疗应用的现状和前景，同时考虑了传统的诊疗模式——使用放射性标记的间碘苄胍（MIBG）——以及使用放射性标记的生长抑素类似物（SSAs）的创新模式，有望在不久的将来为这些患者所用。
