Table 2.
Presence of various tumors in hereditary syndromes associated with pheochromocytomas or paragangliomas
| Paraganglioma syndromes (SDH) | |
| SDHD | Head and neck paraganglioma |
| Intra-adrenal pheochromocytoma | |
| Extra-adrenal pheochromocytoma# | |
| SDHB | Head and neck paraganglioma |
| Intra-adrenal pheochromocytoma | |
| Extra-adrenal pheochromocytoma# | |
| Renal carcinoma | |
| Multiple Endocrine Neoplasia type 2 | |
| Type 2a. | Medullary thyroid carcinoma |
| Pheochromocytoma | |
| Hyperparathyroidism | |
| Cutaneous lichen amyloidosis | |
| Type 2b. | Medullary thyroid carcinoma |
| Pheochromocytoma | |
| Multiple neuromas | |
| Marfanoid habitus | |
| FMTC | Familial medullary thyroid carcinoma |
| Von Hippel-Lindau syndrome type 2 | |
| Type 2a. | Retinal and central nervous system haemangioblastomas |
| Intra-adrenal pheochromocytoma | |
| Endolymphatic sac tumors | |
| Epididymal cystadenomas | |
| Type 2b. | Renal-cell cysts and carcinomas |
| Retinal and central nervous system haemangioblastomas | |
| Pancreatic neoplasms and cysts | |
| Intra-adrenal pheochromocytoma | |
| Endolymphatic sac tumors | |
| Epididymal cystadenomas | |
| Type 2c. | Intra-adrenal pheochromocytoma |
| Neurofibromatosis type 1 | |
| Neurofibromas (multiple) | |
| Café-au-lait spots | |
| Intra-adrenal pheochromocytoma | |
Adapted from Lenders et al.1, officially extra-adrenal paraganglioma, more frequent in SDHB (~80%).