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Germline succinate dehydrogenase complex, subunit B (<jats:italic><jats:styled-content style=\"fixed-case\">SDHB<\/jats:styled-content><\/jats:italic>) mutation constitutes the main reliable molecular predictor of malignancy. Plasma and urinary methoxytyramine are the biochemical markers characterizing metastatic <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>s\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content>s along with evolving molecular markers such as mi<jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content>s and <jats:styled-content style=\"fixed-case\">SNAIL<\/jats:styled-content>. Conventional imaging is used for tumour localization, whereas <jats:sup>18<\/jats:sup>F\u2010<jats:styled-content style=\"fixed-case\">FDG<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">PET<\/jats:styled-content> for staging of metastatic <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>s\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content>s especially those related to <jats:italic><jats:styled-content style=\"fixed-case\">SDHB<\/jats:styled-content><\/jats:italic> gene mutations. In addition, <jats:sup>68\u00a0<\/jats:sup>Ga\u2010<jats:styled-content style=\"fixed-case\">DOTATATE PET<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">CT<\/jats:styled-content> is emerging as a highly sensitive alternative. Surgery remains the gold standard treatment in reducing tumour bulk and\/or controlling the clinical syndrome. Treatment with <jats:sup>131<\/jats:sup>I\u2010<jats:styled-content style=\"fixed-case\">MIBG<\/jats:styled-content> or radiolabelled somatostatin analogues is considered for unresectable disease. Conventional chemotherapy is reserved for more advanced and refractory to other therapies disease although new schemes are currently evolving. Recent genetic studies have highlighted a number of pathways involved in <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>s\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content>s pathogenesis directing towards the use of targeted therapies which have still to be validated in clinical practice.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Metastatic <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>s\/<jats:styled-content style=\"fixed-case\">PGL<\/jats:styled-content>s remain an orphan disease that is only curable by surgery. 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