Demographic and clinical characteristics of the 12 patients at baseline and of the 9 (1-9) patients included in the analysis of long-term outcome
| . | Sex, age at anakinra treatment (years) . | Age at ECD diagnosis (years) and BRAF status . | ECD localizations . | Previous treatments . | Biopsy site . | Treatment duration . | Anakinra failure, progression of disease . | Anakinra efficacy . |
|---|---|---|---|---|---|---|---|---|
| 1 | M, 64 | 52 WT | Retroperitoneal, bones, aorta, pericardium, atrial pseudomass, sinus, spine | SteroidsIFN, PEG-IFN | Pericardium, perirenal | 22 mo | Heart, pleura | Spine |
| 2 | M, 68 | 60 ND | Retroperitoneal, bones, aorta, atrial pseudomass | Steroids, IFN, PEG-IFN, imatinib | Perirenal | 31 mo | Pleura, lung, spine | |
| 3 | M, 76 | 72 V600E | Retroperitoneal, Bones, aorta, pericardium, atrial pseudomass | IFN, PEG-IFN | Perirenal | 26 mo | Aorta, heart | Pleura |
| 4 | F, 80 | 72 V600E | Retroperitoneal, bones, aorta, pericardium, atrial pseudomass, lung | Steroids, IFN | Perirenal | 7 mo | Stable | |
| 5 | F, 58 | 52 ND | Bones | IFN, PEG-IFN | Bone | 28 mo | Bones | |
| 6 | M, 75 | 72 ND | Bones | None | Bone | 11 mo | CNS | |
| 7 | F, 22 | 16 ND | DI, lung, sinus, retroperitoneal | Steroids, IFN, PEG-IFN, vinblastine | Sinus | 3 mo | Stable | |
| 8 | M, 71 | 65 V600E | Retroperitoneal, bones, lung, aorta, atrial pseudomass, CNS | IFN, PEG-IFN | Perirenal | 5 mo | Bones, pleura, CNS | |
| 9 | M, 70 | 69 V600E | Aorta, bones, retroperitoneal, atrial pseudomass, retroorbital, xanthelasma | Steroids, PEG-IFN | Perirenal | 34 mo | Heart | |
| 10 | M, 72 | 71 ND | Aorta, bones, pericardium, atrial pseudomass, hydronephrosis, retroorbital | PEG-IFN | Perirenal | 3 wk | NA | NA |
| 11 | F60 | 59 V600E | Aorta, bones, atrial pseudomass, skin, bones, retroorbital, DI | Steroids, PEG-IFN | Perirenal | 3 wk | NA | NA |
| 12 | F70 | 69 ND | Aorta, DI, bones | None | Bones | 1 wk | NA | NA |
| . | Sex, age at anakinra treatment (years) . | Age at ECD diagnosis (years) and BRAF status . | ECD localizations . | Previous treatments . | Biopsy site . | Treatment duration . | Anakinra failure, progression of disease . | Anakinra efficacy . |
|---|---|---|---|---|---|---|---|---|
| 1 | M, 64 | 52 WT | Retroperitoneal, bones, aorta, pericardium, atrial pseudomass, sinus, spine | SteroidsIFN, PEG-IFN | Pericardium, perirenal | 22 mo | Heart, pleura | Spine |
| 2 | M, 68 | 60 ND | Retroperitoneal, bones, aorta, atrial pseudomass | Steroids, IFN, PEG-IFN, imatinib | Perirenal | 31 mo | Pleura, lung, spine | |
| 3 | M, 76 | 72 V600E | Retroperitoneal, Bones, aorta, pericardium, atrial pseudomass | IFN, PEG-IFN | Perirenal | 26 mo | Aorta, heart | Pleura |
| 4 | F, 80 | 72 V600E | Retroperitoneal, bones, aorta, pericardium, atrial pseudomass, lung | Steroids, IFN | Perirenal | 7 mo | Stable | |
| 5 | F, 58 | 52 ND | Bones | IFN, PEG-IFN | Bone | 28 mo | Bones | |
| 6 | M, 75 | 72 ND | Bones | None | Bone | 11 mo | CNS | |
| 7 | F, 22 | 16 ND | DI, lung, sinus, retroperitoneal | Steroids, IFN, PEG-IFN, vinblastine | Sinus | 3 mo | Stable | |
| 8 | M, 71 | 65 V600E | Retroperitoneal, bones, lung, aorta, atrial pseudomass, CNS | IFN, PEG-IFN | Perirenal | 5 mo | Bones, pleura, CNS | |
| 9 | M, 70 | 69 V600E | Aorta, bones, retroperitoneal, atrial pseudomass, retroorbital, xanthelasma | Steroids, PEG-IFN | Perirenal | 34 mo | Heart | |
| 10 | M, 72 | 71 ND | Aorta, bones, pericardium, atrial pseudomass, hydronephrosis, retroorbital | PEG-IFN | Perirenal | 3 wk | NA | NA |
| 11 | F60 | 59 V600E | Aorta, bones, atrial pseudomass, skin, bones, retroorbital, DI | Steroids, PEG-IFN | Perirenal | 3 wk | NA | NA |
| 12 | F70 | 69 ND | Aorta, DI, bones | None | Bones | 1 wk | NA | NA |
DI, diabetes insipidus; NA, not available; ND, not determined; PEG, pegylated; V600E, presence of mutation BRAF V600E; WT, wild type.