Metabolism and pheochromocytoma/paraganglioma

Massimo Mannelli, Elena Rapizzi, Rossella Fucci, Letizia Canu, Tonino Ercolino, Michaela Luconi, William Francis Young

Research output: Contribution to journalArticle

6 Scopus citations


The discovery of SDHD as a pheochromocytoma/paraganglioma susceptibility gene was the prismatic event that led to all of the subsequent work highlighting the key roles played by mitochondria in the pathogenesis of these tumors and other solid cancers. Alterations in the function of tricarboxylic acid cycle enzymes can cause accumulation of intermediate substrates and subsequent changes in cell metabolism, activation of the angiogenic pathway, increased reactive oxygen species production, DNA hypermethylation, and modification of the tumor microenvironment favoring tumor growth and aggressiveness. The elucidation of these tumorigenic mechanisms should lead to novel therapeutic targets for the treatment of the most aggressive forms of pheochromocytoma/paraganglioma.

Original languageEnglish (US)
Pages (from-to)T83-T90
JournalEndocrine-Related Cancer
Issue number4
StatePublished - Aug 1 2015


  • Angiogenesis
  • Mitochondria
  • Oncometabolites
  • Pheochromocytoma/paraganglioma
  • Succinatedehydrogenase

ASJC Scopus subject areas

  • Endocrinology
  • Oncology
  • Cancer Research
  • Endocrinology, Diabetes and Metabolism

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  • Cite this

    Mannelli, M., Rapizzi, E., Fucci, R., Canu, L., Ercolino, T., Luconi, M., & Young, W. F. (2015). Metabolism and pheochromocytoma/paraganglioma. Endocrine-Related Cancer, 22(4), T83-T90.