Pheochromocytomas commonly manifest as a part of Multiple Endocrine Neoplasia 2 A&B and Von Hippel-Lindau syndromes (secondary to alterations of the RET and VHL genes, respectively). Pheochromocytoma/paragangliomas do not typically present as a part of Multiple Endocrine Neoplasia 1 syndrome or Tuberous Sclerosis (secondary to alterations of the MEN1 and TSC1/TSC2 genes, respectively). Extra-adrenal paragangliomas commonly occur due to germline mutations of the succinate dehydrogenase (SDHA, SDHB, SDHC, SDHD, and SDHAF2) and fumarate hydratase (FH) genes.
Guo Z, Lloyd RV. Pheochromocytomas and Paragangliomas: An Update on Recent Molecular Genetic Advances and Criteria for Malignancy. . Adv Anat Pathol. 2015 Sep;22(5):283-93.