Table 1 Clinical and genomic features in hereditary tumors linked with adrenal tumor
From: Current prospects of hereditary adrenal tumors: towards better clinical management
Hereditary tumor | Relevant adrenal tumor | Causative gene | Mode of inheritance | Incidence of respective syndrome | Median onset of adrenal tumor | Percentage of patients who were diagnosed with adrenal tumor among hereditary syndrome individuals | Genotype/phenotype relationship | References |
|---|---|---|---|---|---|---|---|---|
LFS | ACC | TP53 | Autosomal dominant | 1: 5,000-20,000 | 3 years | 13% (27% in children cohort; 3% in adult cohort) | Yes | |
LS | ACC | MMR genes (MLH1, MSH2, MSH6, and PMS2) | Autosomal dominant | 1: 660-2,000 | 64 years | 0.5% | Unknown | [7] |
FAP | ACC | APC | Autosomal dominant | 1: 8,000-18,000 | NA | NA | Unknown | [8] |
BWS | ACC | Defects on chromosome 11p15.5 | Not applicable | 1: 13,700 | 10 months | 0.3% | Unknown | [9] |
MEN2 | PPGL | RET | Autosomal dominant | 1: 35,000 | 39 years | 37% | Yes | [10] |
VHL | PPGL | VHL | Autosomal dominant | 1: 36,000–91,000 | 27 years (mean) | 19% | Yes | |
NF1 | PPGL | NF1 | Autosomal dominant | 1: 2,500-3,000 | 41 years; 42 years | 3% | Unknown |