Clinical Presentations, Biochemical Phenotypes, and Genotype-Phenotype Correlations in Patients with<i>Succinate Dehydrogenase Subunit B</i>-Associated Pheochromocytomas and Paragangliomas

Abstract

SDHB-related PGL often presents as apparently sporadic PGL with symptoms related to tumor mass effect rather than to catecholamine excess. The predominant biochemical phenotype consists of hypersecretion of norepinephrine and/or dopamine, whereas 10% of tumors are biochemically silent. The clinical expression of these tumors cannot be predicted by the genotype.