Germline alterations in GIST-associated genes may also confer high risk for other cancers, such as paragangliomas and pheochromocytomas for SDH-complex genes and nerve sheath tumors, breast cancer, and gliomas for NF1. Therefore, their identification has critical implications for future cancer surveillance and clinical management of the patients, as well as appropriate care of their at-risk family members. The gene discussed is SDHB; the disease is gastrointestinal stromal tumor.