Metastatic chest wall malignant schwannoma responding to sorafenib: case report and literature review.
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 female 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. Studies are ongoing and the results are eagerly awaited regarding the responses to these medications and whether they can positively impact on the natural history of this disease.
Gudena
Vinay
V
Department of Hematology-Oncology, Medical University of South Carolina, Charleston, South Carolina 29425, USA.
Verma
Nitin
N
Post
Ginell
G
Kizziah
Michael
M
Fenning
Robert
R
Montero
Alberto J
AJ
D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001557:Benzenesulfonates / Q000627:therapeutic use*; D001932:Brain Neoplasms / Q000188:drug therapy / Q000556:secondary; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000188:drug therapy / Q000556:secondary; D008279:Magnetic Resonance Imaging; D009362:Neoplasm Metastasis; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology; D009442:Neurilemmoma / Q000175:diagnosis* / Q000188:drug therapy*; D009536:Niacinamide / Q000031:analogs & derivatives; D010671:Phenylurea Compounds; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D011725:Pyridines / Q000627:therapeutic use*; D013902:Radiography, Thoracic; D000077157:Sorafenib; D035441:Thoracic Wall / Q000473:pathology*; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D001557:Benzenesulfonates; D010671:Phenylurea Compounds; D047428:Protein Kinase Inhibitors; D011725:Pyridines; D009536:Niacinamide; D000077157:Sorafenib