Table 2. Outcome Data for All Registrants.
| Outcome | No./Total No. (%) | P Value | ||
|---|---|---|---|---|
| Total (n = 625) | Steroid Dependent (n = 377) | Steroid Independent (n = 248) | ||
| Follow-up, median (IQR), y | 8 (3-25) | 10 (4-22) | 7 (3-13) | <.001 |
| Relapse in ipsilateral adrenal | 35/625 (5.6) | 2/377 (0.5) | 33/248 (13.3) | <.001 |
| Adrenal crises at least once | 67/625 (10.7) | 67/377 (17.7) | NA | NA |
| No. of adrenal crises | 177/625 (28.3) | 177/377 (46.9) | NA | NA |
| No. of patients with metastases of any primary | 47/625 (7.5) | 37/377 (9.8) | 10/248 (4.0) | .001 |
| No. of deaths | 63/625 (10.1) | 47/377 (12.4) | 16/248 (6.4) | <.001 |
| Symptoms of steroid overdosing | 50/625 (8.0) | 50/377 (13.2) | NA | NA |
| Causes of adrenal crises | ||||
| Infection | 45/67 (67.2) | 45/67 (67.2) | NA | NA |
| Stress | 14/67 (20.9) | 14/67 (20.9) | NA | NA |
| Trauma | 2/67 (3.0) | 2/67 (3.0) | NA | NA |
| Withdrawal or noncompliance | 6/67 (9.0) | 6/67 (9.0) | NA | NA |
| Metastatic tumors in living patients | ||||
| Pheochromocytoma or paraganglioma | 8/37 (21.6) | 5/29 (17.2) | 3/8 (37.5) | .90 |
| Multiple endocrine neoplasia type 2 with medullary thyroid carcinoma | 18/37 (48.7) | 18/29 (62.1) | 0/8 | .001 |
| VHL with renal cell carcinoma | 2/37 (5.4) | 2/29 (6.9) | 0/8 | .50 |
| VHL with pancreatic neuroendocrine tumor | 7/37 (18.9) | 3/29 (10.3) | 4/8 (50) | .40 |
| VHL with other | 0/37 | 0/29 | 0/8 | >.99 |
| Nonsyndromic cancer | 2/37 (5.4) | 1/29 (3.5) | 1/8 (12.5) | .80 |
| Causes of deaths | ||||
| Pheochromocytoma or paraganglioma | 3/63 (4.8) | 2/47 (4.3) | 1/16 (6.25) | >.99 |
| Adrenal crises | 2/63 (3.2) | 2/47 (4.3) | NA | NA |
| Multiple endocrine neoplasia type 2 with medullary thyroid carcinoma | 31/63 (49.2) | 25/47 (53.2) | 6/16 (37.5) | .001 |
| VHL with renal cell carcinoma | 1/63 (1.6) | 1/47 (2.1) | 0/16 | >.99 |
| VHL with pancreatic neuroendocrine tumor | 3/63 (4.8) | 1/47 (2.1) | 2/16 (12.5) | .50 |
| VHL with other | 3/63 (4.8) | 2/47 (4.3) | 1/16 (6.3) | >.99 |
| Nonsyndromic cancer | 8/63 (12.7) | 6/47 (12.8) | 2/16 (12.5) | >.99 |
| Other cause or unknown | 12/63 (19.1) | 8/47 (17.0) | 4/16 (25.0) | >.99 |
Abbreviations: IQR, interquartile range; NA, not applicable; VHL, von Hippel-Lindau disease.