Treatment of succinate dehydrogenase B-associated renal cancer

Germline mutations in the succinate dehydrogenase B (SDHB) gene are associated with a rare renal cancer with a young age of onset. It is important to raise awareness of this condition as it is dominantly inherited and is associated with other neoplasms. The diagnosis should be considered in patients with renal cancers with distinctive histology. There is little guidance in the literature about how to treat metastatic SDHB renal cancers. We report a confirmed partial response to treatment with a vascular endothelial growth factor receptor tyrosine kinase inhibitor, pazopanib.