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.

journal_name

Anticancer Res

journal_title

Anticancer research

authors

Marjanska A,Galazka P,Wysocki M,Styczynski J

doi

10.21873/anticanres.14136

subject

Has Abstract

pub_date

2020-04-01 00:00:00

pages

1817-1831

issue

4

eissn

0250-7005

issn

1791-7530

pii

40/4/1817

journal_volume

40

pub_type

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