Table 1.
Clinical Characteristics of Patients with PPGL According to the Genetic Analysis Result
| Characteristic | Total (n=57) | Genetic analysis result | P valuea | |||
|---|---|---|---|---|---|---|
|
| ||||||
| Positive (n=28) | Negative (n=29) | |||||
|
| ||||||
| All period (n=28) | Pre-NGS period (n=14) | Post-NGS period (n=14) | ||||
| Age, yrb | 48.5±13.7 | 46.2±15.0 | 48.1±15.7 | 48.5±13.7 | 50.8±12.2 | 0.211 |
|
| ||||||
| Age of onset, yrb | 42.8±14.8 | 39.5±15.9 | 42.2±18.2 | 42.8±14.8 | 46.0±13.1 | 0.096 |
|
| ||||||
| Female sex | 31 (54.4) | 16 (57.1) | 7 (50.0) | 9 (64.3) | 15 (51.7) | 0.681 |
|
| ||||||
| Family history | 9 (15.8) | 9 (32.1) | 2 (14.3) | 7 (50.0) | 0 | 0.001 |
|
| ||||||
| Type of PPGL | ||||||
| PCC | 40 (70.2) | 20 (71.4) | 7 (50.0) | 13 (92.9) | 20 (71.4) | 0.839 |
| PGL | 17 (29.8) | 8 (28.6) | 7 (50.0) | 1 (7.1) | 8 (28.6) | |
|
| ||||||
| Location | ||||||
| PCC (n=40) | ||||||
| Unilateral adrenal gland | 32 (80.0) | 14 (70.0) | 4 (57.1) | 10 (76.9) | 18 (90.0) | 0.114 |
| Right adrenal gland | 16 (40.0) | 8 (40.0) | 2 (28.6) | 6 (46.2) | 8 (40.0) | |
| Left adrenal gland | 16 (40.0) | 6 (30.0) | 2 (28.6) | 4 (30.8) | 10 (50.0) | |
| Bilateral adrenal glands | 8 (20.0) | 6 (30.0) | 3 (42.9) | 3 (23.1) | 2 (10.0) | |
| PGL (n=17) | ||||||
| Head and neck | 3 (17.7) | 1 (12.5) | 1 (14.3) | 0 | 2 (22.2) | 0.600 |
| Othersc | 14 (82.4) | 7 (87.5) | 6 (85.7) | 1 (100.0) | 7 (77.8) | |
|
| ||||||
| Tumor size, cm (n=48)d | 3.7 (2.7–4.8) | 3.5 (2.0–4.6) | 3.1 (2.0–4.4) | 4.1 (3.2–4.7) | 3.8 (3.2–5.0) | 0.261 |
|
| ||||||
| Metastasis | 15 (26.3) | 10 (35.7) | 6 (42.9) | 4 (28.6) | 5 (17.2) | 0.113 |
| Synchronous | 7 (12.3) | 4 (14.3) | 3 (21.4) | 3 (21.4) | 3 (10.3) | 0.464 |
| Metachronous | 8 (14.0) | 6 (21.4) | 3 (21.4) | 1 (7.2) | 2 (6.9) | |
|
| ||||||
| Recurrence | 11 (19.3) | 7 (25.0) | 4 (28.6) | 3 (21.4) | 4 (13.8) | 0.284 |
|
| ||||||
| Presence of other tumors | 18 (31.6) | 12 (42.9) | 3 (21.4) | 9 (64.3) | 6 (20.1) | 0.072 |
|
| ||||||
| Discovery route (n=51) | ||||||
| Incidental | 21 (41.2) | 11 (45.8) | 4 (33.3) | 7 (58.3) | 11 (37.0) | 0.224 |
| Symptomatic | 28 (54.9) | 11 (45.8) | 7 (58.3) | 4 (33.3) | 17 (63.0) | |
| Gene mutation | 2 (3.9) | 2 (8.3) | 1 (8.3) | 1 (8.3) | 0 | |
|
| ||||||
| Biochemical profile | ||||||
| Adrenergic/Noradrenergic | 9 (15.8) | 4 (14.3) | 1 (7.1) | 3 (21.4) | 5 (17.2) | 0.732 |
| Adrenergic | 1 (1.8) | 1 (3.6) | 0 | 1 (7.1) | 0 | |
| Noradrenergic | 30 (52.6) | 14 (50.0) | 9 (64.3) | 5 (35.7) | 16 (55.2) | |
| Silent | 17 (29.8) | 9 (32.1) | 4 (28.6) | 5 (35.7) | 8 (27.6) | |
Values are expressed as mean±standard deviation, number (%), or median (interquartile range).
PPGL, pheochromocytoma and paraganglioma; NGS, next-generation sequencing; PCC, pheochromocytoma; PGL, paraganglioma.
a
P value for the correlation between positive group and negative group;
b
P value was calculated by t test, as they were parametric variables (Shapiro-Wilk test P=0.680 for age; P=0.574 for age of onset);
c
Others include retroperitoneum, pancreas, para-aortic space, and bladder;
d
P value was calculated by the Mann-Whitney U test, as it was a nonparametric variable (Shapiro-Wilk test P<0.001).