Table 1.
Clinical Characteristics and Outcomes
| Case No | Age* | Gender | Mutation | Largest lesion size (cm) | PA Operationsa | Recurrence site/time | Path | Follow up (yrs) | Steroids |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 17 | M | 167: Arg>Gln | 3.0 | lap-RPA | None | Pheo | 15.63 | No |
| 2 | 13 | M | 167: Arg>Gln | 2.7 | lap-LPA, lap-BPA | None | Pheo | 15.77 | No† |
| 3 | 13 | M | 128: Leu>Phe | 2.5 | open-LPA | None | Pheo | 12.74 | No |
| 4 | 13 | M | 167: Leu>Gln | 4.1 | lap-LPA, lap-RPA | None | Composite | 9.41 | No |
| 5 | 15 | F | 161: Leu>Gln | 1.4 | lap-LPA, lap-RPA | None | Pheo | 2.56 | No |
| 6 | 6 | M | 124: Thr>Ile | 3.5 | lap-LPA, lap-RPA | Right/5mos | Composite | 4.58 | No |
| 7 | 17 | F | 149: Ala>Ser | 2.0 | open-BPA | None | Pheo | ** | ** |
| 8 | 13 | M | 167: Leu>Gln | 1.5 | lap-RPA | None | Pheo | 5.00 | No |
| 9 | 16 | M | 167: Arg>Gln | 6.5 | open-RPA, lap-RPA | Right/1yr | Pheo | 4.06 | No |
| 10 | 15 | M | 129: Leu>Pro | 5.5 | robo-LPA, robo-RPA | None | Pheo | 4.42 | Yes x2 years post-op |
*
Age at first operation
**
Post-operative death
a
Operations are in chronological order and do not include total adrenalectomies.
†
Patient was steroid independent for 10 years post operatively. He has recently begun steroid supplementation after removal of an ACTH secreting ectopic tumor in the lung.
lap - laparoscopic
robo - robotic
LPA – left partial adrenalectomy
RPA – right partial adrenalectomy
BPA – bilateral partial adrenalectomy