PPGL are much rarer neoplasms and annual incidence is estimated to be 0.8 per 100,000 person years.[7] Current data suggest that a hereditary syndrome is found in about 30% to 40% of the cases, which raises the critical importance of genetic testing in these patients.[3,8] Mutation of the SDH subunit genes A, B, C, or D is a known cause of hereditary PGL with an autosomal dominant inheritance pattern. This evidence concerns the gene SDHB and neoplasm.