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- W2988207992 endingPage "i49" @default.
- W2988207992 startingPage "i40" @default.
- W2988207992 abstract "Abstract One of the most common malignancies affecting adults with the neurofibromatosis type 1 (NF1) cancer predisposition syndrome is the malignant peripheral nerve sheath tumor (MPNST), a highly aggressive sarcoma that typically develops from benign plexiform neurofibromas. Approximately 8–13% of individuals with NF1 will develop MPNST during young adulthood. There are few therapeutic options, and the vast majority of people with these cancers will die within 5 years of diagnosis. Despite efforts to understand the pathogenesis of these aggressive tumors, the overall prognosis remains dismal. This manuscript will review the current understanding of the cellular and molecular progression of MPNST, diagnostic workup of patients with these tumors, current treatment paradigms, and investigational treatment options. Additionally, we highlight novel areas of preclinical research, which may lead to future clinical trials. In summary, MPNST remains a diagnostic and therapeutic challenge, and future work is needed to develop novel and rational combinational therapy for these tumors." @default.
- W2988207992 created "2019-11-22" @default.
- W2988207992 creator A5036526586 @default.
- W2988207992 creator A5037690587 @default.
- W2988207992 creator A5049583085 @default.
- W2988207992 creator A5089359152 @default.
- W2988207992 date "2019-11-14" @default.
- W2988207992 modified "2023-10-02" @default.
- W2988207992 title "Diagnosis and management of malignant peripheral nerve sheath tumors: Current practice and future perspectives" @default.
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