Classification of familial pheochromocytoma.
| Syndrome | Gene | Locus | Exons | Phaeo | Malignancy (malignancy) |
| Multiple endocrine neoplasia type 2a/b | RET | 12q 11.2 | 21 | 50 % | 3-5 % |
| Von Hippel-Lindau syndrome | VHL | 3p 25-26 | 3 | 20 % | 5 % |
| Recklinghausen’s disease (neurofibromatosis type I) | Nf1 | 17q 11.2 | 59 | 2 % | 10 % |
| Paraganglioma syndrome type 1 | SDHD | 11q 23 | 4 | 4-7 % | 23 % |
| Paraganglioma syndrome type 2 | SDHB | 1p 36 | 8 | 3-10 % | 50 % |
