Biallelic loss-of-function mutations to the subunits of SDH (A-D) or SDH assembly factors (SDHAF1-2), can lead to neoplasms in neuroendocrine tissues and the kidney (Astuti et al., 2001; Bardella et al., 2011; Baysal et al., 2000; Burnichon et al., 2010; Hao et al., 2009; Niemann and Müller, 2000). The gene discussed is SDHB; the disease is neoplasm.