Metastatic chest wall malignant schwannoma responding to sorafenib: Case report and literature review

Abstract

Malignant schwannomas or malignant peripheral nerve sheath tumors (MPNST) represent approximately 10% of all soft tissue sarcomas. Metastatic disease from chest wall MPNST is very rare. We present a case of a major clinical response to the tyrosine kinase inhibitor (TKI) sorafenib in a patient with metastatic MPNST. A 42-year-old woman with a prior history of neurofibromas developed MPNST, which later metastasized to the lungs and brain. She was initially placed on sorafenib with significant clinical response to lung metastases. MPNST show high levels of Ras activity and hence these tumors are promising targets for TKIs. In conclusion sorafenib appears to be active in or patient with metastatic MPNST and results from planned pase II trials are eagerly awaited. ©2008 Landes Bioscience.