Vemurafenib Treatment of BRAF V600E-Mutated Malignant Peripheral Nerve Sheath Tumor
No effective systemic treatment exists for malignant peripheral nerve sheath tumors (MPNSTs). These tumors have been reported to show increased activity in the mitogen-activated protein kinase pathway from the loss of neurofibromatosis-1 regulation and occasionally from BRAF V600E mutation. A patient with sporadic metastatic MPNST and the BRAF V600E mutation was treated with standard doses of sorafenib and later vemurafenib and followed for response. The patient showed a rapid but modest and transient response to sorafenib and a very dramatic response to vemurafenib. This case represents the first report of successful systemic treatment of MPNST with an inhibitor of the BRAF V600E mutation. It will be important to define the general utility of this approach and related therapies in this disease.
This activity has been designated to meet the educational needs of physicians and nurses involved in the management of patients with cancer.
Upon completion of this activity, participants will be able to:
- Evaluate treatment options for patients with malignant peripheral nerve sheath tumors
- Discuss the potential benefits of vemurafenib in the presence of a BRAF mutation
Henry G. Kaplan, MD
Swedish Cancer Institute
- 1.00 Participation
- 1.00 Nurse
- 1.00 Physician