Horm Metab Res 2019; 51(07): 414-418
DOI: 10.1055/a-0586-3710
© Georg Thieme Verlag KG Stuttgart · New York

Diagnostic Investigation of Lesions Associated with Succinate Dehydrogenase Defects

David Taïeb
1  Department of Nuclear Medicine, La Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France
Henri Timmers
2  Department of Internal Medicine, Division of Endocrinology, Radboud University Medical Centre, Nijmegen, The Netherlands
Karel Pacak
3  Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA
› Author Affiliations
Further Information

Publication History

received 05 November 2017

accepted 28 February 2018

Publication Date:
04 April 2018 (online)


The mitochondrial enzyme succinate dehydrogenase (SDH) acts as a tumor suppressor. Biallelic inactivation of one of the genes encoding for SDH subunits (collectively named SDHx) leads to complete loss of the protein function and the development of diverse group of tumors. Pheochromocytomas-paragangliomas are the prime example of hereditary tumors caused by SDH deficiency. In this review, we discuss the roles of imaging examinations, and illustrate new insights into genotype-imaging phenotype relationships.