Table 1.
Analysis of the 24-h urine steroid metabolome in the patient at initial presentation with a large adrenal tumor at gestational week 24.
| Urinary steroid metabolites | 24-h Urinary steroid metabolite excretion in the PUMAH patient (μg/24 h) | Female Reference Range (μg/24 h) |
Range observed in 45 patients with adrenocortical carcinoma |
|---|---|---|---|
| Androsterone (An) | 767 | 859 (176-2257) | 1130 (515-2445) |
| Etiocholanolone (Et) | 4384 | 974 (255-2564) | 3671 (1171-7372) |
| 11-Hydroxyandrosterone (11OHAn) | 4357 | 328 (119-774) | 623 (346-1921) |
| Dehydroepiandrosterone (DHEA) | 24 432 | 172 (11-1972) | 612 (98-18273) |
| 16α-Hydroxy-dehydroepiandrosterone (16aDHEA) | 5126 | 289 (29-1573) | 653 (169-3168) |
| Pregnenetriol (5PT) | 2206 | 114 (30-390) | 1901 (554-7865) |
| Pregnenediol (5PD) | 8916 | 140 (44-536) | 3128 (848-14308) |
| Tetrahydro-11-dehydrocorticosterone (THA) | 213 | 76 (34-183) | 112 (56-195) |
| Tetrahydrocorticosterone (THB) | 312 | 84 (31-172) | 147 (61-380) |
| 5α-Tetrahydrocorticosterone (5aTHB) | 379 | 164 (41-335) | 155 (77-356) |
| Tetrahydro-11-deoxycorticosterone (THDOC) | 592 | 13 (5-111) | 103 (36-222) |
| 3α,5β-Tetrahydroaldosterone (3a5bTHAldo) | 129 | 30 (13-57) | 24 (11-48) |
| Pregnanediol (PD) | 16 999 | 160 (62-1286) | 839 (364-2657) |
| 3α,5α-17-Hydroxypregnanolone (3a5a17HP) | 33 | 5 (1-36) | 19 (11-44) |
| 17-Hydroxypregnanolone (17HP) | 1730 | 47 (18-331) | 511 (299-1021) |
| Pregnanetriol (PT) | 3568 | 293 (104-853) | 511 (299-1021) |
| Pregnanetriolone (PTONE) | 148 | 7 (3-63) | 32 (10-99) |
| Tetrahydro-11-deoxycortisol (THS) | 1197 | 56 (22-149) | 2151 (334-4703) |
| Cortisol (F) | 2256 | 44 (24-78) | 245 (95-701) |
| 6β-Hydroxycortisol (6bOHF) | 4633 | 103 (47-222) | 356 (187-1530) |
| Tetrahydrocortisol (THF) | 10 426 | 1035 (455-1648) | 2836 (1373-5234) |
| 5α-Tetrahydrocortisol (5aTHF) | 556 | 748 (178-1597) | 852 (450-1711) |
| α-Cortol (a-cortol) | 2286 | 201 (101-384) | 557 (279-1589) |
| β-Cortol (b-cortol) | 1393 | 301 (135-708) | 740 (316-1341) |
| 11β-Hydroxyetiocholanolone (11OHEt) | 1688 | 235 (40-606) | 366 (149-1668) |
| Cortisone (E) | 645 | 72 (37-130) | 164 (83-364) |
| Tetrahydrocortisone (THE) | 13 908 | 2100 (1035-3658) | 3701 (1909-6852) |
| α-Cortolone (a-cortolone) | 3671 | 848 (472-1459) | 1840 (868-2853) |
| β-Cortolone (b-cortolone) | 1605 | 424 (187-904) | 677 (458-1312) |
| 11-Oxoetiocholanolone (11oxoEt) | 1305 | 271 (59-626) | 484 (232-1903) |
The 7 steroids typically increased in patients with ACC14 are followed in bold and were all increased above the reference range derived from 73 healthy, nonpregnant women16 and similar to the range previously observed in 45 patients with ACC.14 Multisteroid profiling in 24-h urine was carried out by GC–MS.