Research progress of malignant peripheral nerve sheath tumors
ZHANG Tiantian
WU Qiong
SU Fang
Abstract：Malignant peripheral nerve sheath tumors (MPNSTs) are a kind of rare and aggressive soft tissue sarcomas.Clinical and imaging findings are not specific, and diagnosis depends mainly on pathology and immunohistochemistry.To date, there are no standard treatment options for MPNSTs.Surgery is the only definitive treatment, and the role of chemotherapy and radiation therapy is undetermined.MPNSTs behave aggressively with a high rate of local recurrence and a significant propensity to metastasize.With multidisciplinary treatment, the outcome is poor and 5-year survival rates range from 35％to 50％.Due to the rare morbidity, clinicians do not know enough about them, and this article reviews clinical manifestations, diagnosis, treatment and so on of MPNSTs as to improve awareness and standardize the diagnosis and treatment of the disease.
Keywords：Malignant peripheral nerve sheath tumorsTherapyTargeted therapyClinical manifestation
Publication Date：2018-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 1140-1144 )