Table 1.
Tumor and clinical characteristics |
Sporadic | SDHB | MEN, VHL, NF1 |
---|---|---|---|
Malignancy rate | 10 – 36% | High but to be determined | < 5% |
Most common location of primary tumor | Extra-adrenal | Extra-adrenal | Adrenal |
Biochemical phenotype | NE | NE, DA | NE, EPI |
Most common sites of metastatic lesions | Bones, LNs, liver, lungs | Bones, | Bones, LNs |
Unfavorable prognostic factors | Younger age, large tumor, low NE levels | Younger age, large tumor, low NE and high DA levels | Large tumor |
Abbreviations: NE: norepinephrine; EPI: epinephrine; DA: dopamine; LNs: lymphatic nodes.