Table 1.
Clinical and biochemical presentation of patients with Cushing syndrome at time of diagnosis and after remission
| Variable | Total N = 174 |
Pituitary CS N = 106 |
Ectopic CS N = 25 |
Adrenal CS N = 43 |
P |
|---|---|---|---|---|---|
| Age at diagnosis, y, median (range) | 51 (16-82) | 49 (16-82) | 51 (34-77) | 55 (35-78) | < .001 |
| Age at time of curative procedure, y, median (range) | 52.5 (19-82) | 50 (19-82) | 51 (34-78) | 56 (37-78) | .001 |
| Women, n (%) | 127 (73.0%) | 86 (81.1%) | 12 (48%) | 29 (67.4%) | .002 |
|
BMI, median (range) Available for n = 170 |
34.84 (21.03-66.4) | 36.97 (22.4-66.4) | 29.53 (21-41.6) | 32.7 (22-48.4) | < .001 |
| CS biochemical severity score, n (%) | < .001 | ||||
| Mild | 25 (14.5%) | 8 (7.6%) | 4(16%) | 13 (30.2%) | |
| Moderate | 57 (32.9%) | 35 (33.3%) | 2 (8%) | 20 (46.5%) | |
| Severe | 91 (52.6%) | 62 (59.1%) | 19 (76%) | 10 (23.3%) | |
| Available for n = 173 | |||||
| CS clinical severity score, n (%) | < .001 | ||||
| Mild | 16 (9.2%) | 3 (2.8%) | 2 (8%) | 11 (25.6%) | |
| Moderate | 56 (32.2%) | 27 (25.5%) | 8 (32%) | 21 (48.8%) | |
| Severe | 102 (58.6%) | 76 (71.7%) | 15 (60%) | 11 (25.6%) | |
| Duration of hypercortisolism before curative procedure, mo, median (range) | 24 (0-240) | 36 (0-240) | 12 (0.5-108) | 18 (0-228) | < .001 |
| Biochemical assessment | |||||
|
24-h urine cortisol, mcg/24 h Median (range) Available for n = 158 Normal range: < 45 mcg/24 h |
180.5 (5.7-6459) | 178.5 (7.7-2638) | 990 (133-6459) | 73 (5.7-1643) | < .001 |
|
1-mg dexamethasone suppression test, mcg/dL Median (range) Available for n = 103 Normal range: < 1.8 mcg/dL |
13.7 (2.2-85.4) | 17 (3.8-78.5) | 51.35 (8-85.4) | 6.35 (2.2-60) | < .001 |
|
ACTH, pg/mL, median (range) Available for n = 169 Normal range: 6-63 pg/mL |
73.5 (2-531) | 81 (21-387) | 185 (29-531) | 5.7 (2-40) | < .001 |
|
Salivary cortisol, ng/dL, median (range) Available for n = 55 Normal range: < 50 ng/dL |
263 (41-8300) | 265.5 (50-1600) | 2550 (645-8300) | 109 (41-1270) | .002 |
| Curative procedure for CS | |||||
| Pituitary surgery, n (%) | 77 (44.3%) | 77 (72.6%) | – | – | |
| Pituitary radiationa, n (%) | 2 (1.1%) | 2 (1.9%) | – | – | |
| Resection of the neuroendocrine tumor, n (%) | 10 (5.7%) | – | 10 (40%) | – | |
| Unilateral adrenalectomy, n (%) | 36 (20.7%) | – | – | 36 (83.7%) | |
| Bilateral adrenalectomy, n (%) | 49 (28.2%) | 27 (25.5%) | 15 (60%) | 7 (16.3%) | |
| Postoperative adrenal insufficiency and glucocorticoid replacement therapy after curative procedure | |||||
| Patients taking glucocorticoids at end of follow-up, n (%) | 72 (41.4%) | 47 (44.3%) | 10 (40%) | 15 (34.9%) | .08 |
| Duration of supraphysiological glucocorticoid replacement therapy, mo, median (range)b | 6.5 (0-45) | 8 (0-23) | 2 (0-30) | 7 (1-24) | |
| Recovery of adrenal function at end of follow-up, n (%) | 102 (58.6%) | 59 (55.7%) | 15 (60%) | 28 (65.1%) | .38 |
| Duration of glucocorticoid replacement therapy, mo, median (range)c | 13 (0-139) | 13 (1-139) | 10 (0-65) | 14.5 (0-55) |
Abbreviations: ACTH, adrenocorticotropin; BMI, body mass index; CS, Cushing syndrome.
a Previously had pituitary surgery. Biochemical recurrence was noted, leading patients to treatment before symptom recurrence (no medical therapy required). Evidence of improvement after radiation was documented biochemically.
b Defined as patients who were still on glucocorticoid replacement or patients with bilateral adrenalectomy on more than 30-mg hydrocortisone equivalent.
c Two patients with adrenal CS were on glucocorticoids for less than 1 month (mild CS or rapid improvement of hypothalamic-pituitary-adrenal axis), and one patient with ectopic CS was on ketoconazole preoperatively, which contributed to short duration of steroids.