Table 3.
Patient treatment summary
| Patient | Mutation | Reason of IFN treatment | Imaging exam (baseline and follow-up) | New lesion on scintigraphy (FDG or MIBG) at baseline | Response to IFN PERCIST 1.0/RECIST 1.1 | Loco-regional treatment associated | Duration of treatment with IFN | SRE (n) |
|---|---|---|---|---|---|---|---|---|
| #1 | SDHB | Progression | MRI and FDG-PET | NA | NA/NA | None | 4 months | 0 |
| #2 | SDHB | Progression within 7 months | MRI and FDG-PET | Yes, epidural compression on MRI | SD/NE | Kiphoplasty L1 and L4 | 54 months | 2 |
| #3 | No | Progression within 18 months | FDG-PET and CT scan | Yes | SD/SD | None | 6 months, discontinuation for fever and arthralgiaa | 0 |
| #4 | SDHB | Progression within 10 months | CT scan, FDG-PET, and octreo scan | Yes | PMR/NA | Radiofrequency ablation and kiphoplasty T4, left iliac and left femur | 25 months | 0 |
| #5 | SDHB | Progression within 9 months | CT scan and octreo scan | Yes | SD/PD | None | 11 months | 0 |
| #6 | SDHB | Progression within 6 months | FDG-PET | Yes | PMR/NE | C1 to C5 radiation | 19 months | 1 |
| #7 | SDHB | Progression within 6 months | FDG-PET and CT scan | Yes | PMR/SD | C1 radiation | 14 months | 1 |
| #8 | SDHB | Progression within 7 months | FDG-PET and MRI | Yes | SD/NA | T2T3 surgery and radiation therapy | 37 months, treatment discontinuation for asthenia | 1 |
| #9 | No | Progression within 6 months | MIBG | Yes | SD/NE | Sacro-iliac joint and right femur radiation therapy | 17 months | 1 |
| #10 | SDHB | Progression within 8 months | FDG-PET | Yes | PMR/NE | Kiphoplasty L4, radiofrequency ablation T4, left rib and manubrium | 40 months | 1 |
| #11 | No | Progression within 33 months | MIBG and CT scan | Yes | SD/SD | None | 3 months, treatment until MIBG therapy | 0 |
| #12 | SDHB | Progression within 4 months | FDG-PET and scan | Yes | SD/SD | L2 radiofrequency ablation | 7 months until progressive disease with new bone lesion | 0 |
| #13 | No | Progression within 3 months | FDG-PET and MRI | Yes | NA/NA | L4 and sacrum cryotherapy | 37 months, pause | 0 |
| #14 | No | Progression within 3 months | FDG-PET | Yes | SD/NE | None | 12.6 months, discontinued for skin toxicity, stabilization remains at last follow-up | 0 |
NA not available, NE not evaluable, SD stable disease, PD progressive disease, PMR partial metabolic response
aPatient #3 was admitted to ICU 1 month after discontinuation of IFN-α for thoracic pain + hypertension + pulmonary edema + renal insufficiency which led to cardiac arrest