Table 1.
Baseline characteristics for adults with LCH (N = 219). All data are presented as n (% of total in that column)
| Characteristic | 2001-2009 (n = 90) | ≥ 2010 (n = 129) | Total (N = 219) | P |
|---|---|---|---|---|
| Age at diagnosis, y, mean (range) | 41.5 (19-76) | 45.3 (19-88) | 43.8 (18-88) | .234 |
| Female, n (%) | 47 (52.2) | 65 (50.4) | 112 (51.1) | .789 |
| Smoking history, n = 214 (%) | 66 (76.7) | 75 (58.6) | 141 (65.9) | .006 |
| BRAFV600E, n = 88 (%) | 3 (30) | 37 (47.4) | 40 (45.5) | .297 |
| Organ involvement through follow-up, n (%) | ||||
| Lung | 62 (68.9) | 53 (41.1) | 115 (52.5) | <.001 |
| Osseous | 29 (32.2) | 62 (48.1) | 91 (41.6) | 0.019 |
| Skin | 20 (22.2) | 32 (24.8) | 52 (23.7) | .658 |
| Pituitary/hypothalamus | 14 (15.6) | 21 (16.3) | 35 (16.0) | .886 |
| Central nervous system | 6 (6.7) | 9 (7) | 15 (6.9) | .929 |
| Meningeal disease | 3(3.3) | 12 (9.3) | 15 (6.9) | .085 |
| Nodal | 10 (11.1) | 10 (7.8) | 20 (9.1) | .396 |
| Liver | 2 (2.2) | 7 (5.4) | 9 (4.1) | .240 |
| Spleen | 5 (5.6) | 4 (3.1) | 9 (4.1) | .368 |
| Gastrointestinal | 3 (3.3) | 6 (4.7) | 9 (4.1) | .629 |
| Oral | 5 (5.6) | 2 (1.6) | 7 (3.2) | .097 |
| Bone marrow∗ | 1 (1.1) | 3 (2.3) | 4 (2.7) | .509 |
| Retroperitoneal† | 1 (1.1) | 2 (1.6) | 3 (1.4) | .783 |
| Sinus | 1 (1.1) | 1 (0.8) | 2 (0.9) | .797 |
| Heart | 0 | 1 (0.8) | 1 (0.5) | .402 |
| LCH Classification at diagnosis, n (%) | ||||
| Unifocal | 19 (21.1) | 43 (33.3) | 62 (28.3) | 0.048 |
| Single-system pulmonary | 39 (43.3) | 31 (24) | 70 (32) | 0.003 |
| MS/MF, RO− | 25 (27.8) | 43 (33.3) | 68 (31.1) | 0.382 |
| RO+ | 7 (7.8) | 12 (9.3) | 19 (8.7) | 0.693 |
RO, risk organ (liver, spleen, and bone marrow); MS/MF RO−, multisystem/multifocal risk organ negative; RO+, risk organ positive.
∗
One case was tissue-proven, remaining with discrete lesions on advanced imaging.
†
Includes cases with renal, adrenal, and retroperitoneal fat involvement.