Table 1.
Summary of characteristics of 18 patients with X-LAG syndrome.
| Study ID |
Sex | Age at start of abnormal growth/ age at diagnosis (months) |
GH | OGTT: GH Suppression |
IGF-1 (xULN) |
Prolactin | Max tumor/ hyperplasia diameter (mm) |
Treatment order | Pathology | Excessive GH/IGF-1 controlled |
Excessive growth controlled (age at control) |
Age at last follow up (years) |
Hypopituitarism (axes) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| S1 | M | 6/56 | ↑↑↑ | No | 4.4 | ↑↑↑ | 27 | Sx, SSA, DA, PV | PA; GH+/PRL+; Ki-67: 3% | Yes | Y (7 yr) | 7 | ACTH, TSH, DI |
| S2 | F | 12/40 | ↑ | ND | 4.9 | ↑ | 15 | Sx, DA, SSA, PV, Sx, SSA | PA; GH+/PRL+; Ki-67:<1% | Yes | Y (11 yr) | 18 | TSH, DI |
| S3 | F | 18/42 | ↑ | No | 2.4 | ↑↑ | 17 | Sx, DA, SSA, RTx (γ-knife) | PA; GH+/PRL+ | No | N | 7.5 | No |
| S4 | F | 2/23 | ↑↑ | ND | 5.2 | ↑↑ | global enlargement | DA, SSA | Pending surgery | No | N | 3.5 | No |
| S5 | F | 7/17 | ↑↑↑ | ND | 3.9 | ↑↑ | 19 | DA, Sx | PA; GH+/PRL+; Ki-67:<1% | Yes | Y (19 mo) | 13 | Panhypopituitarism plus DI |
| S6 | F | 6/36 | ↑↑↑ | Paradoxical increase | 3.1 | ↑↑ | 39 | DA, 2Sx, SSA, PV | PA; GH+/PRL+; Ki-67<1% | Yes | Y (5.5 yr) | 6 | DI |
| S7 | F | 36/63 | ↑ | N/A | N/A | 10 | Sx, SSA, DA, RTx (Conv.), Sx, SSA | PA; GH+/FSH+; Ki-67<1% | Yes | Y (8 yr) | 30 | TSH | |
| S8 | F | 11/33 | ↑↑↑ | Paradoxical increase | 3.3 | ↑ | 18 | DA, Sx, RTx, SSA | Eosinophilic adenoma | No | N | 8 | No |
| S9 | F | 3/33 | ↑↑ | No | 3.4 | ↑↑ | 18; general enlargement | DA, Sx | Global hyperplasia; Ki-67<1% | Yes | Y (21 mo) | 11 | Pahypopituitarism plus DI |
| S10 | F | 6/43 | ↑↑ | No | 3.3 | ↑↑↑ | 24 | DA, Sx, SSA, RTx (Conv.), PV | PA; GH+/PRL+ | Yes | Y (4.9 yr) | 11 | No |
| S11 | M | 48/67 | ↑↑↑ | No | 3.6 | ↑↑ | 32.5 | Sx | PA; GH+/PRL+ | No | N | 10 | No |
| S12 | F | 10/48 | ↑ | No | N/A | ↑ | 25 | Sx, SSA, DA, RTx (Conv.), PV | PA; GH+/PRL+ | Yes | Y (adult) | 35 | ACTH, TSH, LH |
| S13 | F | 48/91 | ↑ | Paradoxical increase | 2.6 | ↑ | 17; general enlargement | SSA, DA, Sx | Global hyperplasia (GH+/PRL+; TSH+); Ki-67<1%) | Yes* | N | 8 | N/A* |
| F1A | F | 12/31 | ↑↑ | No | N/A | ↑↑ | >10 | Sx | Eosinophilic adenoma | Yes | Y (5 yr) | 45 | ACTH, TSH, GH |
| F1B | M | 12/18 | ↑↑ | No | 3.3 | ↑↑↑ | 15 | SSA, DA, 3Sx | PA plus hyperplasia; GH+/PRL+ | Yes | Y (4yr) | 18 | ACTH, TSH, GH |
| F1C | M | 14/14 | ↑ | Paradoxical increase | 1.9 | ↑ | symmetric enlargement | DA, Sx | Global hyperplasia plus microadenoma (GH+/PRL+) | Yes | Y (4 yr) | 13 | ACTH, TSH |
| F2A | M | 48/96 | ↑ | No | N/A | ↑ | 19 | Sx | PA; GH+/PRL+ | Yes | Y (12 yr) | 23 | DI |
| F2B | F | Child/264 | ↑ | No | N/A | ↑ | >10 | Sx | PA; GH+/PRL+ | Yes | Y (adult) | 26┼ | ACTH, TSH, LH |
OGTT: Oral glucose tolerance test,; ULN: upper limit of the normal range; SSA: somatostatin analog; DA: dopamine agonist; PV: pegvisomant; RTx: radiotherapy; Conv.: conventional; PA: pituitary adenoma; DI: diabetes insipidus. For hormonal criteria: ↑: 2–10 × ULN; ↑↑: >10–50 × ULN; ↑↑↑: >50× ULN.
┼
Patient died of sepsis aggravated by hypopituitarism;
*
insufficient follow up postoperatively (<1 month) to determine permanent pituitary hypofunction.