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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.
URL
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.

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Molecular Profile Treatment Approach
Gene Name Source Synonyms Protein Domains Gene Description Gene Role
Therapy Name Drugs Efficacy Evidence Clinical Trials
Drug Name Trade Name Synonyms Drug Classes Drug Description
Gene Variant Impact Protein Effect Variant Description Associated with drug Resistance
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