期刊
TRENDS IN ENDOCRINOLOGY AND METABOLISM
卷 28, 期 11, 页码 807-817出版社
ELSEVIER SCIENCE LONDON
DOI: 10.1016/j.tem.2017.08.001
关键词
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资金
- Eunice Kennedy Shriver National Institute of Child Health and Human Development of the National Institutes of Health in Bethesda, Maryland
- A*MIDEX - 'Investissements d'Avenir' French Government [ANR-11-IDEX-0001-02]
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.
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