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| Ref Type | Journal Article | ||||||||||||
| PMID | (31383651) | ||||||||||||
| Authors | Davis LE, Nicholls LA, Babiker HM, Liau J, Mahadevan D | ||||||||||||
| Title | PD-1 Inhibition Achieves a Complete Metabolic Response in a Patient with Malignant Peripheral Nerve Sheath Tumor. | ||||||||||||
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| Abstract Text | High-grade malignant peripheral nerve sheath tumors (MPNST) have a poor prognosis with limited responsiveness to systemic therapy. We document a case of a complete metabolic response to pembrolizumab monotherapy in metastatic disease. Tumor molecular profiling identified programmed-death ligand-1 (PD-L1) positivity. This characteristic provided a rationale for immune-checkpoint therapy. Treatment with pembrolizumab resulted in a complete metabolic response after four cycles of therapy. Patients with PD-L1-positive, metastatic MPNST may be candidates for immune-checkpoint therapy, which may produce a durable complete remission. Future study of anti-PD-1/PD-L1 therapy is warranted. | ||||||||||||
| Molecular Profile | Treatment Approach |
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| Gene Name | Source | Synonyms | Protein Domains | Gene Description | Gene Role |
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| Therapy Name | Drugs | Efficacy Evidence | Clinical Trials |
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| Drug Name | Trade Name | Synonyms | Drug Classes | Drug Description |
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| Gene | Variant | Impact | Protein Effect | Variant Description | Associated with drug Resistance |
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| Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
|---|---|---|---|---|---|---|---|
| CD274 positive | malignant peripheral nerve sheath tumor | predicted - sensitive | Pembrolizumab | Case Reports/Case Series | Actionable | In a clinical case study, Keytruda (pembrolizumab) treatment resulted in complete metabolic response after 4 cycles of treatment in a patient with CD274 (PD-L1)-positive malignant peripheral nerve sheath tumor, and the patient stayed on treatment for 21 cycles (PMID: 31383651). | 31383651 |