Taïeb David, Pacak Karel
Department of Nuclear Medicine, La Timone University Hospital, European Center for Research in Medical Imaging (CERIMED), Aix-Marseille University, Marseille, France.
Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA.
Trends Endocrinol Metab. 2017 Nov;28(11):807-817. doi: 10.1016/j.tem.2017.08.001. Epub 2017 Aug 31.
Pheochromocytomas and paragangliomas (PPGLs) belong to the family of neural crest cell-derived neoplasms. In up to 70% of cases they are associated with germline and somatic mutations in 15 well-characterized PPGL driver or fusion genes. PPGLs can be grouped into three main clusters, where cluster 1 includes PPGLs characterized by a pseudohypoxic signature. Although cluster 1 tumors share several common features, they exhibit unique behaviors. We present here unique insights into the imaging phenotypes of cluster 1 PPGLs based on glucose uptake, catecholamine metabolism, and somatostatin receptor expression. Recent data suggest that succinate is a major player in the imaging phenotype of succinate dehydrogenase-deficient PPGLs. This review emphasizes the emerging stromal cell-succinate interaction and highlights new perspectives in PPGL theranostics.
嗜铬细胞瘤和副神经节瘤（PPGLs）属于神经嵴细胞衍生的肿瘤家族。在高达70%的病例中，它们与15个特征明确的PPGL驱动基因或融合基因中的种系和体细胞突变相关。PPGLs可分为三个主要簇，其中簇1包括以假低氧特征为特点的PPGLs。尽管簇1肿瘤有几个共同特征，但它们表现出独特的行为。我们在此基于葡萄糖摄取、儿茶酚胺代谢和生长抑素受体表达，对簇1 PPGLs的成像表型提出独特见解。近期数据表明，琥珀酸是琥珀酸脱氢酶缺陷型PPGLs成像表型的主要参与者。本综述强调了新兴的基质细胞 - 琥珀酸相互作用，并突出了PPGL诊断与治疗学的新观点。
