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.

中文翻译：

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对第 1 类嗜铬细胞瘤和副神经节瘤的核成像表型的新见解

嗜铬细胞瘤和副神经节瘤 (PPGL) 属于神经嵴细胞源性肿瘤家族。在多达 70% 的病例中，它们与 15 个充分表征的 PPGL 驱动基因或融合基因的种系和体细胞突变有关。PPGLs 可以分为三个主要集群，其中集群 1 包括以假缺氧特征为特征的 PPGLs。尽管第 1 类肿瘤具有几个共同特征，但它们表现出独特的行为。我们在此提出对基于葡萄糖摄取、儿茶酚胺代谢和生长抑素受体表达的 1 组 PPGL 成像表型的独特见解。最近的数据表明，琥珀酸是琥珀酸脱氢酶缺陷型 PPGL 成像表型的主要参与者。