Main characteristics of PNSs experienced by patients selected for analysis after central review
| Results | Patients with pre-existing PNS (cohort 1), n = 16 | Patients with newly diagnosed PNS (cohort 2), n = 16 | Total patientsn = 32 |
|---|---|---|---|
| Time from diagnosis of cancer to diagnosis of the PNS, median (range), months | 0.3 (−62.8;406.2)a | 18.6 (3.9;281.5) | 11.9 (−62.8;406.2) |
| Neurologic PNS, n patients (%) | 4 (25) | 7 (44) | 11 (34) |
| - Encephalitis | 1 | 5 | 6 |
| - Neuropathy | 2 | 1 | 3 |
| - Lambert-Eaton syndrome | 1 | 1 | 2 |
| Rheumatologic PNS, n patients (%) | 3 (19) | 6 (38) | 9 (28) |
| - Hypertrophic osteoarthropathy | 2 | 4 | 6 |
| - RS3PE | 0 | 2 | 2 |
| - Rhizomelic pseudopolyarthritis | 1 | 0 | 1 |
| Connective tissue PNS, n patients (%) | 6 (37) | 2 (12) | 8 (25) |
| - Dermatomyositis | 4 | 1 | 5 |
| - Systemic sclerosis | 1 | 1 | 2 |
| - Myositis (anti-PL7 antisynthetase syndrome) | 1 | 0 | 1 |
| Other PNSs, n patients (%) | 3 (19) | 1 (6) | 4 (13) |
| - Membranous nephropathy | 1 | 0 | 1 |
| - IgA vasculitis or Henoch-Schönlein purpura | 1 | 0 | 1 |
| - Other, thrombotic microangiopathy | 1 | 0 | 1 |
| - Other, Cushing’s disease | 0 | 1 | 1 |
| Highest CTCAE grade for PNS severity, n of patients (%) | |||
| - Grade 1–2 | 5 (31) | 5 (31) | 10 (31) |
| - Grade 3–4 | 11 (69) | 7 (43) | 18 (56) |
| - Grade 5 | 0 | 4 (25) | 4 (13) |
| CTCAE grade for PNS severity at last follow-up, n of patients (%) | |||
| - Grade 0–1 | 7 (44) | 5 (31) | 12 (38) |
| - Grade ≥ 2 | 9 (56) | 11 (69) | 20 (62) |
| Causes of death, n of patients (%) | 3 (19) | 6 (38) | 9 (28) |
| - PNS | 0 | 4 | 4 (13) |
| - tumor progression | 2 | 2 | 4 (13) |
| - comorbidity | 1 | 0 | 1 (3) |
CTCAE Common Terminology Criteria for Adverse Events, PNS paraneoplastic syndrome, RS3PE remitting seronegative symmetrical synovitis with pitting edema
aSome patients presented with a PNS before the cancer diagnosis, which explains the negative lower boundary. Some patients presented with PNS at cancer relapse, which explains why the time between cancer diagnosis and PNS exacerbation was sometimes greater than 60 months