Treatments and outcomes of the different clinical groups of ALK-positive histiocytosis and the overall cohort
| . | Group 1A (n = 6) . | Group 1B (n = 10) . | Group 2 (n = 23) . | Overall (n = 39) . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| . | Number of cases . | Objective response . | Progression or relapse . | Number of cases . | Objective response . | Progression or relapse . | Number of cases . | Objective response . | Progression or relapse . | Number of cases . | Objective response . | Progression or relapse . |
| First-line | ||||||||||||
| Conventional systemic therapy | 3 (50%) | 1/3 (33%) | 3/3 (100%) | 6 (60%) | 3/6 (50%) | 2/6 (33%) | 4 (17%)† | 3/4 (75%) | 1/4 (25%) | 13 (33%) | 7/13 (54%) | 6/13 (46%) |
| Surgical resection | 13 (57%)‡ | 11/13 (85%) | 2/13 (15%) | 13 (33%) | 11/13 (85%) | 2/13 (15%) | ||||||
| ALK inhibition | 4 (40%)* | 4/4 (100%) | 0/4 (0%) | 1 (4%) | 1/1 (100%) | 0/1 (0%) | 5 (13%) | 5/5 (100%) | 0/5 (0%) | |||
| Observation & supportive care | 2 (33%) | 2/2 (100%) | 0/2 (0%) | 1 (4%) | 1/1 (100%) | 0/1 (0%) | 3 (8%) | 3/3 (100%) | 0/3 (0%) | |||
| Radiotherapy | 1 (4%) | 1/1 (100%) | 0/1 (0%) | 1 (3%) | 1/1 (100%) | 0/1 (0%) | ||||||
| Unknown or not treated yet | 1 (17%) | 3 (13%) | 4 (10%) | |||||||||
| Second- and further-line | ||||||||||||
| ALK inhibition | 3 (30%) | 3/3 (100%) | 0/3 (0%) | 3 (13%) | 3/3 (100%) | 0/3 (0%) | 6 (15%) | 6/6 (100%) | 0/6 (0%) | |||
| Conventional systemic therapy | 3 (50%) | 2/3 (67%) | 1/3 (33%) | 2 (9%) | 0/2 (0%) | 2/2 (100%) | 5 (13%) | 2/5 (40%) | 3/5 (60%) | |||
| . | Group 1A (n = 6) . | Group 1B (n = 10) . | Group 2 (n = 23) . | Overall (n = 39) . | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| . | Number of cases . | Objective response . | Progression or relapse . | Number of cases . | Objective response . | Progression or relapse . | Number of cases . | Objective response . | Progression or relapse . | Number of cases . | Objective response . | Progression or relapse . |
| First-line | ||||||||||||
| Conventional systemic therapy | 3 (50%) | 1/3 (33%) | 3/3 (100%) | 6 (60%) | 3/6 (50%) | 2/6 (33%) | 4 (17%)† | 3/4 (75%) | 1/4 (25%) | 13 (33%) | 7/13 (54%) | 6/13 (46%) |
| Surgical resection | 13 (57%)‡ | 11/13 (85%) | 2/13 (15%) | 13 (33%) | 11/13 (85%) | 2/13 (15%) | ||||||
| ALK inhibition | 4 (40%)* | 4/4 (100%) | 0/4 (0%) | 1 (4%) | 1/1 (100%) | 0/1 (0%) | 5 (13%) | 5/5 (100%) | 0/5 (0%) | |||
| Observation & supportive care | 2 (33%) | 2/2 (100%) | 0/2 (0%) | 1 (4%) | 1/1 (100%) | 0/1 (0%) | 3 (8%) | 3/3 (100%) | 0/3 (0%) | |||
| Radiotherapy | 1 (4%) | 1/1 (100%) | 0/1 (0%) | 1 (3%) | 1/1 (100%) | 0/1 (0%) | ||||||
| Unknown or not treated yet | 1 (17%) | 3 (13%) | 4 (10%) | |||||||||
| Second- and further-line | ||||||||||||
| ALK inhibition | 3 (30%) | 3/3 (100%) | 0/3 (0%) | 3 (13%) | 3/3 (100%) | 0/3 (0%) | 6 (15%) | 6/6 (100%) | 0/6 (0%) | |||
| Conventional systemic therapy | 3 (50%) | 2/3 (67%) | 1/3 (33%) | 2 (9%) | 0/2 (0%) | 2/2 (100%) | 5 (13%) | 2/5 (40%) | 3/5 (60%) | |||