Characteristics of 53 patients with ECD
| Variable of interest . | Value . |
|---|---|
| Epidemiologic data | |
| Male sex, no. (%) | 39 (74) |
| Median age at onset, y (range) | 53 (6-76) |
| Median age at diagnosis, y (range) | 57 (16-80) |
| Median age at compilation, y (range) | 62 (22-81) |
| Median follow-up from disease onset, mo (range) | 89 (4-430) |
| Median follow-up from diagnosis, mo (range) | 38 (1-189) |
| Global mortality, no. (%) | 14 (26) |
| 1-year survival, % | 96 |
| 5-year survival, % | 68 |
| Skeletal involvement, no. (%) | 51 (96) |
| Extraskeletal involvement, no. (%) | 52 (98) |
| Cardiac involvement, no. (%) | 34 (64) |
| Coronary involvement | 22 (42) |
| Pericardial involvement | 22 (42) |
| Right atrial pseudotumor | 21 (40) |
| Valvulopathy | 9 (17) |
| Large-vessel involvement, no. (%) | 35 (66) |
| CNS involvement, no. (%) | 27 (51) |
| Diencephalic involvement | 23 (43) |
| Meningeal involvement | 9 (17) |
| Cerebellar involvement | 11 (21) |
| Other manifestations of the disease, no. (%) | |
| Paranasal sinus involvement | 33 (62) |
| Maxillary involvement | 19 (36) |
| Cutaneous involvement (xanthelasma) | 15 (28) |
| Orbital involvement | 13 (25) |
| Hypophyseal involvement | 12 (23) |
| Pulmonary involvement | 23 (43) |
| Retroperitoneal infiltration | 36 (68) |
| Adrenal gland infiltration | 12 (23) |
| Treatments, no. (%) | |
| Corticosteroids | 30 (57) |
| Interferon-α | 46 (87) |
| PEGylated-interferon-α | 18 (34) |
| Mesylate imatinib | 7 (13) |
| Sunitinib malate | 6 (11) |
| Vinblastine | 2 (4) |
| 2CDA (cladribine) | 2 (4) |
| Infliximab | 1 (2) |
| Etoposide | 1 (2) |
| Anakinra | 1 (2) |
| Methotrexate | 1 (2) |
| Cyclophosphamide | 1 (2) |
| Variable of interest . | Value . |
|---|---|
| Epidemiologic data | |
| Male sex, no. (%) | 39 (74) |
| Median age at onset, y (range) | 53 (6-76) |
| Median age at diagnosis, y (range) | 57 (16-80) |
| Median age at compilation, y (range) | 62 (22-81) |
| Median follow-up from disease onset, mo (range) | 89 (4-430) |
| Median follow-up from diagnosis, mo (range) | 38 (1-189) |
| Global mortality, no. (%) | 14 (26) |
| 1-year survival, % | 96 |
| 5-year survival, % | 68 |
| Skeletal involvement, no. (%) | 51 (96) |
| Extraskeletal involvement, no. (%) | 52 (98) |
| Cardiac involvement, no. (%) | 34 (64) |
| Coronary involvement | 22 (42) |
| Pericardial involvement | 22 (42) |
| Right atrial pseudotumor | 21 (40) |
| Valvulopathy | 9 (17) |
| Large-vessel involvement, no. (%) | 35 (66) |
| CNS involvement, no. (%) | 27 (51) |
| Diencephalic involvement | 23 (43) |
| Meningeal involvement | 9 (17) |
| Cerebellar involvement | 11 (21) |
| Other manifestations of the disease, no. (%) | |
| Paranasal sinus involvement | 33 (62) |
| Maxillary involvement | 19 (36) |
| Cutaneous involvement (xanthelasma) | 15 (28) |
| Orbital involvement | 13 (25) |
| Hypophyseal involvement | 12 (23) |
| Pulmonary involvement | 23 (43) |
| Retroperitoneal infiltration | 36 (68) |
| Adrenal gland infiltration | 12 (23) |
| Treatments, no. (%) | |
| Corticosteroids | 30 (57) |
| Interferon-α | 46 (87) |
| PEGylated-interferon-α | 18 (34) |
| Mesylate imatinib | 7 (13) |
| Sunitinib malate | 6 (11) |
| Vinblastine | 2 (4) |
| 2CDA (cladribine) | 2 (4) |
| Infliximab | 1 (2) |
| Etoposide | 1 (2) |
| Anakinra | 1 (2) |
| Methotrexate | 1 (2) |
| Cyclophosphamide | 1 (2) |