Table 2.
Clinical features of four patients with occult Cushing's syndrome
| Characteristic | Occult Cushing's syndrome |
|||
|---|---|---|---|---|
| 1 | 2 | 3 | 4 | |
| Age at diagnosis (yr) | 26 | 44 | 50 | 62 |
| Sex | Male | Female | Male | Female |
| ACTH (pg/ml) (normal, <46) | 639 | 74.8 | 167 | 123 |
| 24-h UFC (μg/24 h) (Normal: 3.5–45) | 11,651 | 565 | 507 | 112 |
| CRH stimulation test results suggest | Ectopic | Done twice: discordant results first, then ectopic | Pituitary | Ectopic |
| HD DST results suggest | Ectopic | Pituitary | Ectopic | Ectopic |
| MRI Pituitary | 4-mm pituitary adenoma | 4-mm possible right pituitary adenoma | 4-mm possible right pituitary adenoma | No evidence of adenoma |
| IPSS venogram | Normal | Bilateral small petrosal veins, difficult to cannulate | Small left petrosal vein | Small left petrosal vein, large right petrosal vein |
| IPSS | ||||
| Peak ACTH IPS/Pa | 1.3 (685/527) | 2.3 (188/82) | 1.3 (285/235) | 1.4 (185/132) |
| Baseline PRL IPS/Pa | 13.2 (104/7.9) | 4.2 (71.6/17.1) | 1.2 (10.8/9.3) | 1.6 (34/21.8) |
| ACTH/PRL IPS/P | 0.1 | 0.6 | 1.1 | 0.9 |
| Other Imaging | ||||
| CT C/A/P | Negative | Negative | 8-mm lung nodule | Left lung nodules |
| MRI C/A/P, cardiac | Possible pericardiac lesion | Negative | Negative | Negative |
| Octreotide scan | Negative | Negative | Negative | Negative |
| Surgery | Underwent TSS at outside facility, pathology with no evidence of adenoma, persistent postoperative hypercortisolism | Underwent TSS at NIH, pathology with no evidence of adenoma, persistent postoperative hypercortisolism | Underwent TSS at NIH, pathology with no evidence of adenoma, persistent postoperative hypercortisolism | Left adnexal mass, status post bilateral salpingo-oopherectomy at NIH, pathology showed fibrothecoma, persistent postoperative hypercortisolism |
| Current status | Medical treatment with ketoconazole and metyrapone, being followed for tumor localization | Medical treatment with Mifepristone, being followed for tumor localization | Underwent bilateral adrenalectomy, being followed for tumor localization | Died |
C/A/P, chest, abdomen, and pelvis; CT, computerized tomography; PRL, prolactin; TSS, transsphenoidal surgery. To convert ACTH level to picomoles per liter, multiply by 0.2202. To convert 24-h UFC level to nanomoles per day, multiply by 2.759.
a
Actual ACTH or prolactin values are shown in parentheses.