New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications.

Abstract

Almost all individuals with neurofibromatosis type 1 (NF1) develop peripheral nerve sheath tumors (PNSTs), mainly benign neurofibromas, however about 10% of PNSTs will undergo transformation to malignant peripheral nerve sheath tumors (MPNSTs). Surgical treatment of PNSTs has traditionally been regarded as a standard approach. The availability of new agents that target specific molecular pathways involved in the pathogenesis of PNST has led to a number of clinical trials, which resulted in increased chances for better survival and quality of life. This review presents the latest evidence and clinical implications for new therapies of PNSTs in patients with NF1 emphasizing the potential benefit from the use of Ras/MAPK pathway inhibitors, immunotherapy, chemotherapy or radiation therapy. We present evaluation of current knowledge on available treatment modalities.

• Authors: Agata Marjanska, Przemyslaw Galazka, Mariusz Wysocki, Jan Styczynski
• Journal: Anticancer research (Anticancer Res) Vol. 40 Issue 4 Pg. 1817-1831 (Apr 2020) ISSN: 1791-7530 [Electronic] Greece
• PMID: 32234870 (Publication Type: Journal Article, Review)
• Copyright: Copyright© 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

Chemical References

• Protein Kinase Inhibitors
• ras Proteins

Topics

• Humans
• MAP Kinase Signaling System (drug effects, genetics)
• Neoplasms (complications, drug therapy, genetics, pathology)
• Nerve Sheath Neoplasms (complications, drug therapy, genetics, pathology)
• Neurofibromatosis 1 (complications, drug therapy, genetics, pathology)
• Protein Kinase Inhibitors (therapeutic use)
• Signal Transduction (drug effects)
• Tomography, X-Ray Computed
• ras Proteins (antagonists & inhibitors, genetics)