Table 4.
Characteristics and outcomes of patients with PNS, by types
| Patient characteristics | Paraneoplastic hypertrophic osteoarthropathy (n = 6 pts) | Paraneoplastic encephalitis (n = 6 pts) |
Paraneoplastic dermatomyositis (n = 5 pts) |
|---|---|---|---|
| Patients with a pre-existing PNS | 2 | 1 | 4 |
| Patients with a newly diagnosed PNS | 4 | 5 | 1 |
| Clinical type | • Hypertrophic osteoarthropathy (n = 6 pts) |
• Anti-Ma2 autoantibody encephalitis (n = 4 pts) • Anti-neuron antibody encephalitis (n = 1 pt) • Cortical myoclonus encephalitis (n = 1 pt) |
• Seronegative dermatomyositis (n = 4 pts) • Anti-TIF1-associated dermatomyositis (n = 1 pt) |
| Cancer type | |||
| - NSCLC | 4 | 3 | 2 |
| - Pulmonary sarcomatoid carcinoma | 1 | 0 | 0 |
| - Renal carcinoma | 1 | 1 | 0 |
| - Mesothelioma | 0 | 1 | 0 |
| - Melanoma | 0 | 1 | 2 |
| - Neuroendocrine carcinoma | 0 | 0 | 1 |
| Bone metastasis | 1 | 0 | 1 |
| General outcome for the PNS following anti-PD1 or PD-L1 immunotherapy | |||
| - No worsening | 1 | 0 | 1 |
| - Worsening | 1 | 1 | 3 |
| - Newly diagnosis of a PNS | 4 | 5 | 1 |
| Highest CTCAE grade of PNS severity | |||
| - Grade 1–2 | 4 | 0 | 0 |
| - Grade ≥ 3 | 2 | 6 | 5 |
| Time interval between initiation of immunotherapy to worsening or new diagnosis of the PNS, median (range), months | 1.4 (0.5–5) | 2.6 (0.5–5.5) | 0.7 (0.5–0.9) |
| Antitumor response at the time of worsening or newly diagnosis of a PNS | |||
| - CR | 0 | 0 | 1 |
| - PR | 0 | 4 | 1 |
| - SD | 5 | 2 | 0 |
| - PD | 1 | 0 | 0 |
| - Not evaluated | 0 | 0 | 2 |
| Impact of paraneoplastic syndrome on immunotherapy, n | |||
| - temporary discontinuation | 3 | 0 | 1 |
| - permanent discontinuation | 0 | 6 | 3 |
| - no discontinuation | 3 | 0 | 1 |
| Paraneoplastic syndrome treatment | |||
| First-line treatment |
All 5 patients received first-line treatment: - Steroids (n = 3): complete resolution in one patient, partial resolution in two patients - NSAIDs (n = 2): complete resolution in one patient, partial resolution in one patient |
All 6 patients received first-line treatment: - Steroids (n = 5): partial resolution in four patients, no resolution in one patient. - Steroids plus immunoglobulins (n = 1): partial resolution in one patient three patients |
All 4 patients received first-line treatment: - Steroids (n = 2): partial resolution in two patients - Steroids plus immunoglobulins (n = 2): partial resolution in one patient, no resolution in one patient. |
| Second-line treatments (if required) |
Second-line treatment was required in one patient: - Methotrexate: partial resolution in one patient. |
Second-line treatment was required in five patients: - Immunoglobulins (n = 2) partial response in two patients - Cyclophosphamide (n = 1): partial resolution in one patient - Rituximab (n = 1): partial resolution in one patient - Plasma exchange (n = 1): partial resolution in one patient |
Second-line treatment was required in four patients: - Methotrexate (n = 3): partial resolution in three patients - Plasma exchange plus methotrexate (n = 1): partial resolution in one patient. |
| Persistent of PNS symptoms with a CTCAE grade > 1 at last follow-up, n (%) | 1 (20) | 5 (83) | 3 (75) |
| PNS related death, n (%) | 0 | 3 (50) | 0 |
CR Complete response, CTCAE Common Terminology Criteria for Adverse Events, NA Not available, NSAID Non-steroidal anti-inflammatory drug, NSCLC Non-small-cell lung carcinoma, PD Progressive disease, PNS Paraneoplastic syndrome, PR Partial response, Pt patient, SD Stable disease